Apendicectomía en pacientes con situs inversus: Serie de tres casos con manejo laparoscópico / Appendectomy in patients with situs inversus: Three cases series with laparoscopic approach

Introducción. La transposición de los órganos debido al situs inversus (SI) es una rara afección que dificulta el diagnóstico de la apendicitis aguda. Esta condición hace que la sintomatología del paciente y los hallazgos al examen físico puedan ser atípicos, lo que demanda el uso de imágenes para la confirmación diagnóstica en la mayoría de los casos. Métodos. Se describieron tres casos de apendicitis en pacientes con diagnóstico de situs inversus. Dos de ellos tenían el antecedente conocido, mientras el tercer caso fue diagnosticado de forma intraoperatoria. Resultados. En dos pacientes se decidió llevar a cirugía vía laparoscópica sin imágenes diagnósticas adicionales. Los pacientes evolucionaron de manera satisfactoria. Conclusión. Siempre se debe considerar la apendicitis dentro de los diagnósticos diferenciales en los pacientes con dolor en fosa ilíaca izquierda. Es fundamental diagnosticar y tratar la apendicitis de manera efectiva para minimizar las complicaciones asociadas. La importancia de la anamnesis y la sospecha clínica del examinador son vitales en estos casos, que se pueden confirmar con las imágenes diagnósticas. Pueden existir casos en donde la condición clínica del paciente no permita la realización de estudios diagnósticos por imágenes; esto apoya cada vez más el uso del abordaje laparoscópico. Se recomienda considerar el abordaje laparoscópico en primera instancia, ya que nos permite la confirmación diagnóstica de situs inversus totalis en caso de que el antecedente sea desconocido y facilita el manejo oportuno de la urgencia.

Introduction. Organ transposition due to situs inversus (SI) is a rare condition that makes the diagnosis of acute appendicitis difficult. This condition entails that the patient' symptoms and physical examination findings may be atypical, which requires the use of images for diagnostic confirmation in most cases. Clinical cases. Three cases of appendicitis in patients diagnosed with situs inversus are described. Two of them had a known medical history, while the third case was diagnosed intraoperatively. Results. In two patients it was decided to undergo laparoscopic surgery without additional diagnostic images. The patients progressed satisfactorily. Conclusion. Appendicitis should always be considered in the differential diagnoses in patients with pain in the left iliac fossa. It is essential to diagnose and treat appendicitis effectively to minimize associated complications. The importance of the anamnesis and the examiner's clinical suspicion are vital in these cases, which can be confirmed with diagnostic images. There may be cases where the patient's clinical condition does not allow diagnostic imaging studies to be performed, increasingly supports the use of the laparoscopic approach. It is recommended to consider the laparoscopic approach in the first instance, since it allows us to confirm the diagnosis of situs inversus totalisin case the history is unknown and facilitates timely management of the emergency.

Síndrome de Rapunzel: atadura sicológica y simulación orgánica / Rapunzel syndrome: psychological bondage and organic simulation

Introducción. El síndrome de Rapunzel es una entidad infrecuente, que se presenta como un tricobezoar a causa de una aglomeración de cabello acumulado dentro del tracto gastrointestinal, por lo que simula otras patologías quirúrgicas. Caso clínico. Paciente femenina de 10 años de edad, con tricotilomanía y tricofagia, dolor abdominal y síntomas inespecíficos de obstrucción intestinal de ocho meses de evolución. Al examen físico se encontró abdomen con distensión y masa palpable en epigastrio y mesogastrio. La ecografía permitió hacer el diagnóstico de tricobezoar gástrico extendido hasta el intestino delgado, por lo que se llevó a cirugía para gastrotomía y se extrajo el tricobezoar, con evolución satisfactoria de la paciente. El abordaje integral permitió conocer la atadura sicológica por posible maltrato infantil. Resultado. La paciente tuvo una evolución satisfactoria y se dio egreso al quinto día de hospitalización. Actualmente se encuentra en seguimiento por sicología, siquiatría infantil y pediatría. Discusión. El caso clínico denota la importancia en reconocer situaciones de presentación infrecuente en pediatría, que puedan estar asociadas a alteraciones sicológicas o presunción de maltrato infantil y que se presenten como una condición orgánica recurrente que simule otras patologías abdominales frecuentes en la infancia. El retraso diagnóstico puede conducir a un desenlace no deseado con complicaciones. Conclusión. Se hace mandatorio el manejo integral del paciente pediátrico y aumentar la sensibilidad para reconocer situaciones de presunción de maltrato infantil, sobre todo en pacientes con una condición orgánica quirúrgica recurrente.

Introduction. Rapunzel syndrome is an uncommon condition that manifests as trichobezoars, which are hair bundles in the stomach or small intestine that can mimics other surgical illnesses. Multiple complications can arise from delayed diagnosis and treatment. Clinical case. A 10-year-old female patient with trichotillomania and trichophagia, with abdominal pain and nonspecific symptoms of intestinal obstruction of eight months of evolution. Physical examination revealed epigastric tenderness and a solid mass was palpable in the mesogastric and epigastric region. An abdominal ultrasound showed gastric trichobezoar that extended into the small intestine. A gastrotomy was performed and the trichobezoar was extracted with satisfactory evolution of the patient. The comprehensive approach allowed knowing the psychological bond due to possible child abuse. Results. The patient had a satisfactory evolution and was discharged on the fifth day of hospitalization. He is currently being monitored by psychology, child psychiatry and pediatrics. Discussion. This clinical case highlights the importance of recognizing situations that seldom present in pediatrics, which may have a psychological aspect due to the presumption of child abuse, and which present as a recurrent organic condition simulating other frequent abdominal pathologies in childhood; all of which may lead to an unwanted outcome due to diagnostic delay. Conclusion. The comprehensive management of the pediatric patient is mandatory to recognize situations of presumed child abuse, in the face of a recurrent surgical conditions.

Carcinoma basocelular de vulva: relato de caso / Basal cell carcinoma of the vulva: case report

Introdução: O carcinoma basocelular (CBC) de vulva é uma condição rara que corresponde a menos de 0,4% dos casos de CBC e de 2% a 4% das neoplasias de vulva. O CBC de vulva é mais comum entre mulheres brancas, multíparas e na pósmenopausa, especialmente na sétima década de vida. O objetivo é relatar um caso de CBC de vulva no qual discutiram-se os aspectos do diagnóstico e tratamento. Relato de Caso: Mulher de 63 anos de idade, G1P1A0, chega ao consultório em janeiro de 2022 para tratamento de lesão persistente em vulva. Realizou-se biópsia incisional que mostrou tratar-se de provável carcinoma basocelular nodular com invasão da derme. A paciente submeteu-se a uma ressecção do tumor com margens macroscópicas livres e sutura primária. A cirurgia não teve complicações no pré-operatório e no pós-operatório. O histopatológico da peça cirúrgica mostrou tratar-se de carcinoma basocelular nodular com área irregular, plana, branco, medindo 0,7x0,4cm, com as margens laterais distando 7,0 e 5,0mm e profundas, 5,9mm; todas livres. Conclusão: O caso relatado é raro, tendo sido o tratamento de ressecção cirúrgica do CBC de vulva com margens bem-sucedido. Catorze meses após a cirurgia, a paciente encontra-se sem evidências de recidiva local ou regional.

Introduction: Basal cell carcinoma (BCC) of the vulva is a rare condition that accounts for less than 0.4% of BCC cases and 2% to 4% of vulvar neoplasms. BCC of the vulva is more common among white, multiparous and postmenopausal women, especially in the seventh decade of life. The aim is to report a case of BCC of the vulva in which aspects of diagnosis and treatment were discussed. Case report: A 63-year-old woman, G1P1A0, arrives at the office in January 2022 for treatment of a persistent lesion on her vulva. An incisional biopsy was performed and showed that it was likely nodular basal cell carcinoma with invasion of the dermis. The patient underwent tumor resection with free macroscopic margins and primary suture. The surgery had no complications preoperatively or postoperatively. The histopathology of the surgical specimen showed that it was a nodular basal cell carcinoma with an irregular, flat, white area, measuring 0.7x0.4cm, with the lateral margins 7.0 and 5.0mm apart and 5.9mm deep; all free. Conclusion: The reported case is rare, with surgical resection of BCC of the vulva with margins being successful. Fourteen months after surgery, the patient has no evidence of local or regional recurrence.

Tumor metastásico en tejido blando a nivel del sacro secundario a carcinoma de células claras de riñón. Reporte de caso / Metastatic tumor in soft tissue at the level of the sacrum secondary toclear cell carcinoma of the kidney. A case report

Introducción. El cáncer de riñón es la undécima neoplasia maligna más común en los Estados Unidos Mexicanos. El carcinoma de células claras de riñón (CCR) es considerado la estirpe más frecuente y representa el 2-3 % de todos los cánceres a nivel mundial. En el contexto de la enfermedad metastásica, por lo general se identifica un tumor renal primario y las metástasis se localizan en pulmón, hueso, hígado, cerebro y, raramente, en tejidos blandos. Los pacientes con metástasis a tejidos blandos no tienen síntomas en las etapas iniciales y generalmente se identifican sólo cuando las lesiones aumentan de tamaño o durante el estudio de la pieza de resección quirúrgica. Caso clínico. Se presenta el caso de una paciente en la séptima década de la vida, con una metástasis en tejidos blandos de la región sacra, de 10 años de evolución posterior a una nefrectomía secundario a CCR. Resultados. Hallazgos clínicos e imagenológicos de un tumor bien delimitado. Se realizó resección quirúrgica de la lesión, bajo anestesia regional, con extirpación completa. Conclusión. Se recomienda que los pacientes con un sitio metastásico resecable y solitario sean llevados a resección quirúrgica con márgenes libres, como fue el caso de nuestra paciente, por su fácil acceso y ser una lesión única. En el CCR, además de su tratamiento quirúrgico inicial, es indispensable una estrecha vigilancia con examen físico e imágenes transversales, para detectar la presencia de metástasis y con ello evitar tratamientos tardíos.

Introduction. Kidney cancer is the eleventh most common malignancy in the United States of Mexico. Carcinoma renal cell (CRC) is considered the most frequent type and represents 2-3% of all cancers worldwide. In the setting of metastatic disease, a primary renal tumor is usually identified, and metastases are located in the lung, bone, liver, brain, and rarely in soft tissue. Patients with soft tissue metastases do not have symptoms in the initial stages and are generally found only when the lesions increase in size or during the study of the surgical resection piece. Clinical case. In this case, we report a female patient in the seventh decade of life with a soft tissue metastasis located in the sacral region, 10 years after a nephrectomy secondary to CRC. Results. Clinical and radiological findings of a well-defined tumor. Surgical resection of the lesion is performed under regional anesthesia with complete excision. Conclusions. It is recommended that patients with a resectable and solitary metastatic site be candidates for surgical resection with free margins, as was the case with our patient due to its easy access and single lesion. In CRC, in addition to its initial surgical treatment, close surveillance with physical examination and cross-sectional images is essential to monitor the presence of metastases and thus avoid late treatments.

Relationship between periapical lesions and sinus changes on multi-slice computed tomography sean / Relagao entre lesoes periapicais e alteragóes sinusais na tomografia computadorizada multislice

ABSTRACT Differentiating orofacial odontogenic pain/disorders from pain/disorders associated with maxillary sinusitis is important to avoid unnecessary dentalprocedures and to properly refer patients to colleagues/dentists and vice versa. Aim To analyze the association between apical lesions and sinus changes and to evaluate the agreement between the diagnoses of an endodontist, a radiologist, an oral and maxillofacial surgeon, and an otolaryngologist. Materials and Method 385 axial, coronal, and sagittal MSCT scans were selected using an image archiving andcommunication system (PACS). The examinations had been performed between 2018 and 2022. Results Apical lesions were observed in 36.10% of sinusitis cases, 73.8% of unilateralsinusitis cases, 48.7% of sinus floor discontinuity cases, and 67.2% of cases in which endodontic treatment had been performed. Agreement between the diagnoses made by the endodontist and those made by the other investigators was high for most study variables (k > 0.60). The exceptions were mucosal thickening, for which agreement between the endodontist and the other investigators was intermediate (k=0.397), and the presence of periapicallesions (k=0.010), previous endodontic treatment (k=0.013), and mucosal thickness (k=0.024), for which agreement between endodontists and radiologists was low. Conclusions: There was an association between sinus changes and apical lesions.

RESUMO Diferenciar a dor/desordens odontogénicas orofaciais da dor/desordens as sociadas á sinusite maxilar é importante para evitar procedimentos odontológicos desnecessários e para encaminhar adequadamente os pacientes aos colegas/dentistas e vice-versa. Objetivo Analisar a associagdo entre lesoes apicais e alteragóes sinusais e avaliar a concordáncia entre os diagnósticos de um endodontista, um radiologista, um cirurgido bucomaxilofacial e um otorrinolaringologista. Material e Método foram avaliadas 385 imagens. Resultados Aslesoes apicais foram observadas em 36,10% dos casos de sinusite, em 73,8% dos casos de sinusite unilateral, em 48,7% dos casos de descontinuidade do assoalho do seio e em 67,2%odos casos em que o tratamento endodontico havia sido realizado. A concordancia entre os diagnósticos feitos pelo endodontista e os feitos pelos outros pesquisadores foi alta para a maioria das variáveis do estudo (k > 0,60). As excegoes foram o espessamento da mucosa, para o qual a concordáncia entre o endodontista e os outros pesquisadores foi intermediária (k=0,397) e a presenga delesoes periapicais (k=0,010), tratamento endodontico prévio (k=0,013) e espessura da mucosa (k=0,024), para os quais a concordáncia entre endodontistas e radiologistas foi baixa. Conclusoes Houve uma associagdo entre as alteragóes sinusais e aslesoes apicais.

Enfermedad de Rosai-Dorfman con afección ganglionar y extraganglionar / Rosai-Dorfman disease with nodal and extranodal involvement

Introducción: la enfermedad de Rosai-Dorfman, conocida como histiocitosis sinusal con linfadenopatía masiva, es una variedad muy rara de las histiocitosis reactivas de origen desconocido. Presentación del caso: paciente femenina de 32 años de edad quien consultó por dificultad respiratoria, voz ronca, estridor y múltiples zonas inflamadas en cuello y tronco desde hace 5 años. Al examen físico se detectaron ganglios linfáticos grandes, firmes y móviles submandibulares y múltiples nódulos en tronco. El examen histológico mostró características típicas de enfermedad de Rosai-Dorfman con afección ganglionar y extraganglionar. Discusión: es un proceso patológico que afecta los ganglios linfáticos cervicales, aunque puede haber lesiones en varias regiones, órganos y sistemas, lo cual puede confundirse con otras neoplasias. Debe considerarse en el diagnóstico diferencial de lesiones histiocíticas de tejidos blandos y otros trastornos linfoproliferativos. La mortalidad ocurre por la alteración funcional de órganos y sistemas más que por la propia enfermedad. Conclusión: la enfermedad de Rosai-Dorfman con afección ganglionar y extraganglionar es un trastorno histiocítico proliferativo benigno muy raro. Es importante considerarla como diagnóstico diferencial de otras enfermedades histiocíticas proliferativas. Su pronóstico es variable pero relativamente benigno.

Introduction: Rosai-Dorfman disease, known as sinus histiocytosis with massive lymphadenopathy, is a very rare variety of reactive histiocytosis of unknown origin. Case presentation: a 32-year-old female patient who consulted for a five-year history of respiratory distress, dysphonia, stridor and multiple swollen areas in the neck and trunk. Physical examination revealed large, firm, and mobile submandibular lymph nodes and numerous nodules on the trunk. Histological examination showed the characteristic features of Rosai-Dorfman disease with nodal and extranodal involvement. Discussion: Rosai-Dorfman disease is a pathological process which affects cervical lymph nodes, although lesions may develop in several sites, organs, and systems, and may be confused with other neoplasms. It should be considered in the differential diagnosis of soft tissue histiocytic lesions and other lymphoproliferative disorders. Death is related to organs and systems dysfunction rather than to the disease itself. Conclusion: Rosai-Dorfman disease including nodal and extranodal involvement, is a very rare benign proliferative histiocytic disorder. It should always be included in the differential diagnosis of other histiocytic proliferative disorders. It carries a variable relatively benign prognosis.

The role of trimethylamine-N-oxide level in the diagnosis of diabetic retinopathy and the differential diagnosis of diabetic and nondiabetic retinopathy / Papel do nível de N-óxido de trimetilamina no diagnóstico da retinopatia diabética e no diagnóstico diferencial da retinopatia diabética e não-diabética

ABSTRACT Purpose: Trimethylamine N-oxide serum levels have been associated with type 2 diabetes mellitus and its complications. The current study aimed to find out if plasma trimethylamine N-oxide level may be a novel marker in the diagnosis of diabetic retinopathy and if it can be used in the differential diagnosis of diabetic and nondiabetic retinopathy. Methods: The study included 30 patients with diabetic retinopathy, 30 patients with nondiabetic retinopathy, 30 patients with type 2 diabetes mellitus without retinopathy, and 30 healthy control participants. Biochemical parameters, serum IL-6, TNF-α, and trimethylamine N-oxide levels were measured in all participants. Results: Trimethylamine N-oxide level was significantly higher in diabetic retinopathy than in the other groups (p<0.001). There was no significant difference in trimethylamine N-oxide levels between nondiabetic retinopathy and control or type 2 diabetes mellitus Groups. There was a significant positive correlation between trimethylamine N-oxide level and elevated FPG, BMI, HOMA-IR score, BUN, IL-6, and TNF-α levels. Conclusion: The current study showed that the trimethylamine N-oxide level is elevated in diabetic retinopathy. These findings suggest that serum trimethylamine N-oxide level might be a novel marker for diabetic retinopathy, and it might be used in the differential diagnosis of diabetic and nondiabetic retinopathy.

RESUMO Objetivo: Os níveis séricos de N-óxido de trimetilamina têm sido associados ao diabetes mellitus tipo 2 e suas complicações. O presente estudo tem como objetivo responder a duas questões, entre elas: O nível plasmático de N-óxido de trimetilamina poderia ser um novo marcador no diagnóstico de retinopatia diabética? e Ele poderia ser utilizado no diagnóstico diferencial de retinopatia diabética e não diabética? Métodos: Trinta pacientes com retinopatia diabética, 30 pacientes com retinopatia não diabética, 30 pacientes com diabetes mellitus tipo 2 sem retinopatia e 30 participantes saudáveis do grupo controle foram incluídos no estudo. Parâmetros bioquímicos, níveis séricos de IL-6, de TNF-α e de N-óxido de trimetilamina foram medidos em todos os participantes. Resultados: O nível de N-óxido de trimetilamina foi significativamente maior na retinopatia diabética do que nos outros grupos (p<0,001). Não houve diferença significativa no nível de N-óxido de trimetilamina entre o grupo de retinopatia não diabética, do grupo controle ou do grupo de diabetes mellitus tipo 2. Houve uma correlação positiva significativa entre o nível de N-óxido de trimetilamina e os níveis elevados de FPG, IMC, HOMA-IR, BUN, IL-6 e TNF-α. Conclusão: O estudo atual mostrou que o nível de N-óxido de trimetilamina encontra-se elevado na retinopatia diabética. Esses achados sugerem que o nível sérico de N-óxido de trimetilamina pode ser um novo marcador na retinopatia diabética, podendo ser usado no diagnóstico diferencial de retinopatia diabética e não diabética.

The dark side of T2: central nervous system lesions with low signal intensity on T2-weighted imaging / The dark side of T2: lesões do sistema nervoso central com baixo sinal em ponderações T2

Abstract The majority of central nervous system diseases show high signal intensity on T2-weighted magnetic resonance imaging. Diseases of the central nervous system with low signal intensity are less common, which makes it a finding that helps narrow the differential diagnosis. This was a retrospective analysis of brain and spine magnetic resonance imaging examinations in which that finding was helpful in the diagnostic investigation. We selected the cases of patients examined between 2015 and 2022. All diagnoses were confirmed on the basis of the clinical-radiological correlation or the histopathological findings. We obtained images of 14 patients with the following central nervous system diseases: arteriovenous malformation; cavernous malformation; metastasis from lymphoma; medulloblastoma; embryonal tumor; metastasis from melanoma; Rathke's cleft cyst; Erdheim-Chester disease; aspergillosis; paracoccidioidomycosis; tuberculosis; syphilis; immunoglobulin G4-related disease; and metastasis from a pulmonary neuroendocrine tumor. We described lesions of different etiologies in which the T2-weighted imaging profile helped narrow the differential diagnosis and facilitated the definitive diagnosis.

Resumo A grande maioria das doenças do sistema nervoso central apresenta alto sinal em ponderações T2 na ressonância magnética. As alterações com baixo sinal são menos comuns, de forma que essa característica permite estreitar o diagnóstico diferencial. Analisamos, retrospectivamente, pacientes com imagens de ressonância magnética de crânio e/ou coluna em que este achado foi útil na investigação diagnóstica. Os pacientes foram selecionados no período entre 2015 e 2022 e todos tiveram seus diagnósticos confirmados por estudo clinicorradiológico ou por estudo histopatológico. Obtivemos imagens de 14 pacientes com as seguintes afecções: malformação arteriovenosa, cavernoma, metástase de linfoma, meduloblastoma, tumor embrionário, metástase de melanoma, cisto da bolsa de Rathke, doença de Erdheim-Chester, aspergilose, paracoccidioidomicose, tuberculose, sífilis, doença relacionada à IgG4 e metástase de tumor neuroendócrino de pulmão. Descrevemos lesões de diversas origens etiológicas que, a partir de suas características nas imagens ponderadas em T2, foi possível reduzir o quadro de diagnósticos diferenciais e chegar mais facilmente à hipótese final.

Immunohistochemical and histopathological analyses of cutaneous innervation to improve the diagnostic efficacy in hansen disease skin lesion / Análises imuno-histoquímica e histopatológica da inervação cutânea para aumento da eficácia diagnóstica nas lesões de pele da hanseníase

Abstract Background The diagnosis of Hansen disease (HD) can be difficult when acid-fast bacilli are not detected in the patient's skin sample. Objective To demonstrate that detailed morphological analysis of nonspecific inflammatory and/or noninflammatory alterations in dermal nerves as well as skin adnexa in leprosy-suspected biopsy samples could improve the efficacy of histopathological diagnosis. Methods Patients with one to five skin lesions were enrolled in the study and classified into three groups by skin histopathology findings: Hansen disease (HD, n = 13), other diseases (OD, n = 11), and inconclusive cases (INC, n = 11). We quantified dermal nerve damage via the nerve lesion index (NLI) and PGP9.5-immunoreactive axon quantitative index in dermal nerves (AQI). We also measured inflammatory involvement of adnexa in cutaneous samples as indirect evidence of HD. Results We observed a higher median endoneurial inflammatory infiltrate NLI (HD = 0.5; INC = 0; OD = 0; p < 0.001) and more frequent inflammatory involvement of skin adnexa in samples of the HD group compared with those of the INC and OD groups (HD = 7; INC = 1; OD = 0). However, samples from the INC and OD groups also showed inflammatory and noninflammatory damage of dermal nerves, with 2 or more kinds of alterations in nerves in the same sample (respectively: INC = in 1 and 2 samples; OD = in 3 and 5 respectively). The quantification of PGP9.5-immunoreactive axons in dermal nerves revealed no difference between the groups. Conclusion A detailed morphological analysis of cutaneous nerves in lesions with a suspicion of HD enabled us to select patients with nonspecific inflammatory or non-inflammatory lesions in the dermal nerves in the INC and OD groups, so they may be clinically monitored aiming at a possible future diagnosis of the disease. These INC and OD patients cannot have the HD diagnosis definitely excluded, and HD may coexist with another disease as a comorbidity.

Resumo Antecedentes A hanseníase pode ter o seu diagnóstico histopatológico dificultado quando bacilos álcool-ácido resistentes não são encontrados nas amostras de pele dos pacientes. Objetivo Demonstrar que uma análise morfológica detalhada de alterações histopatológicas dos nervos dérmicos pode aumentar a eficácia diagnóstica. Métodos Foram selecionadas amostras de pele de pacientes com uma a cinco lesões suspeitas de hanseníase. Os casos selecionados foram classificados conforme achados histopatológicos: hanseníase (HD, n = 13), casos inconclusivos (INC, n = 11), e outras doenças (OD, n = 11). Quantificamos as lesões dos nervos cutâneos por meio do índice de lesão de nervos (nerve lesion index, NLI, em inglês) e do índice quantitativo de axônios (axon quantitative index, AQI, em inglês) imunorreativos a PGP9.5 nos nervos cutâneos. Também medimos o envolvimento inflamatório dos anexos em amostras de pele como evidência indireta de hanseníase. Resultados Foram observadas no grupo HD medianas mais altas do NLI com relação a infiltrados inflamatórios endoneurais (HD = 0,5; INC = 0; OD = 0; p < 0,001) e mais alta frequência de acometimento inflamatório de anexos cutâneos (HD = 7; INC = 1; OD = 0). Entretanto, as amostras dos grupos INC e OD também mostraram comprometimento inflamatório e não inflamatório dos nervos cutâneos, com 2 ou mais tipos de alterações de nervos na mesma amostra (respectivamente: INC = 1 e 2; OD = 3 e 5). Não houve diferença significativa na quantidade de axônios endoneurais imunorreativos a PGP9.5 entre os grupos. Conclusão A análise morfológica detalhada dos nervos cutâneos em lesões suspeitas de hanseníase permitiu selecionar pacientes com lesões inespecíficas inflamatórias ou não inflamatórias nos nervos dérmicos nos grupos INC e OD, para que sejam monitorados clinicamente visando um possível diagnóstico futuro da doença. Esses pacientes INC e OD não podem ter o diagnóstico de HD definitivamente excluído, e a hanseníase pode coexistir com outra doença como uma comorbidade.

The decision-making skills of Brazilian physical therapists for patients with red flags / Capacidade da tomada de decisão dos fisioterapeutas brasileiros para pacientes com bandeiras vermelhas

ABSTRACT BACKGROUND AND OBJECTIVES: The red flags screening purpose is to ensure that signs and symptoms that raise suspicion of serious diseases are being considered during the assessment, assisting physical therapists in their clinical decision process. Brazilian physical therapists are autonomous and can act as first contact professionals in the management of musculoskeletal disorders, therefore, they need to know how to recognize, screen and refer patients with red flags for better therapeutic management. The objectives of this study were to verify whether Brazilian physical therapists can recognize and manage patients who presented red flags, compare professionals' skills regarding different academic degree levels and clinical experience and identify which factors can influence the results. METHODS: A cross-sectional and quantitative research was conducted, collected from an online questionnaire. The target audience consisted of Brazilian physical therapists who have clinical experience in the management of patients with musculoskeletal disorders. Participants filled demographic data and made clinical decisions based on six clinical cases created by the authors, based on the literature, and reviewed by three experts. Data were analyzed using descriptive statistics, the Chi-square test of independence and logistic regression. RESULTS: The study analyzed 384 answers from Brazilian physical therapists with clinical experience in musculoskeletal conditions. Brazilian physical therapists, in general, have not shown to be able to properly recognize and manage the clinical cases involving red flags, with 23.2% of the sample performing appropriate management for medical conditions, 53.9% for emergency conditions and 61.8% for medical conditions with associated musculoskeletal dysfunction. More years of clinical experience and post-professional education did not positively influence the outcomes. Higher academic degrees (Doctorate) can influence positively on the management of non-emergency medical conditions. CONCLUSION: Brazilian physical therapists who work with patients with musculoskeletal disorders perform poorly in identifying red flags in hypothetical clinical cases.

RESUMO JUSTIFICATIVA E OBJETIVOS: O objetivo da triagem de bandeiras vermelhas é garantir que sinais e sintomas que levantam suspeitas de doenças graves sejam considerados durante a avaliação, auxiliando os fisioterapeutas no seu processo de decisão clínica. Os fisioterapeutas brasileiros são autônomos e podem atuar como profissionais de primeiro contato no manejo de distúrbios musculoesqueléticos, portanto, precisam saber reconhecer, rastrear e encaminhar pacientes com bandeiras vermelhas para melhor manejo terapêutico. Os objetivos deste estudo foram verificar se os fisioterapeutas brasileiros conseguem reconhecer e tratar pacientes que apresentavam bandeiras vermelhas, comparar as habilidades dos profissionais com diferentes níveis de formação acadêmica e experiência clínica e identificar quais fatores podem influenciar os resultados. METHODS: Uma pesquisa transversal e quantitativa foi realizada, coletada através de um questionário online. O público-alvo consistiu em fisioterapeutas brasileiros com experiência clínica no manejo de pacientes com disfunções musculoesqueléticas. Os participantes preencheram dados demográficos e tomaram decisões clínicas com base em seis casos clínicos criados pelos autores, com base na literatura, e revisados por três especialistas. Os dados foram analisados por estatísticas descritivas, pelo teste qui-quadrado de independência e por regressão logística. RESULTADOS: Foram analisadas 384 respostas de fisioterapeutas brasileiros com experiência clínica em disfunções musculoesqueléticas. Os fisioterapeutas brasileiros, em geral, não demonstraram ser capazes de reconhecer e manejar adequadamente os casos clínicos envolvendo bandeiras vermelhas, com 23,2% da amostra realizando manejo adequado para condições médicas, 53,9% para condições de emergência e 61,8% para condições médicas com disfunção musculoesquelética associada. Mais anos de experiência clínica e educação pós-profissional não influenciaram positivamente os resultados. Graus acadêmicos mais elevados (Doutorado) podem influenciar positivamente no manejo de condições médicas não emergenciais. RESULTADOS: Foram analisadas 384 respostas de fisioterapeutas brasileiros com experiência clínica em disfunções musculoesqueléticas. Os fisioterapeutas brasileiros, em geral, não demonstraram ser capazes de reconhecer e manejar adequadamente os casos clínicos envolvendo bandeiras vermelhas, com 23,2% da amostra realizando manejo adequado para condições médicas, 53,9% para condições de emergência e 61,8% para condições médicas com disfunção musculoesquelética associada. Mais anos de experiência clínica e educação pós-profissional não influenciaram positivamente os resultados. Graus acadêmicos mais elevados (Doutorado) podem influenciar positivamente no manejo de condições médicas não emergenciais. CONCLUSÃO: Fisioterapeutas brasileiros que atuam com pacientes com disfunções musculoesqueléticas apresentam um mau desempenho na identificação de bandeiras vermelhas em casos clínicos hipotéticos.

Aproximación diagnóstica a las Cefaleas después de los 65 años

Con el objetivo de facilitar el diagnóstico diferencial de la queja de cefalea después de los 65 años de edad, se llevó a cabo una revisión de la literatura con respecto a las cefaleas primarias y secundarias de acuerdo a la Clasificación de la Sociedad Internacional de Cefaleas, seleccionándose las variedades más frecuentes en las personas mayores, describiéndose su condición aguda o crónica,  su frecuencia, características y particularidades, a manera de facilitar un diagnóstico presuntivo que oriente una intervención específica. Las cefaleas primarias más frecuentes en el adulto mayor son la cefalea tensional y la migraña. Entre las cefaleas secundarias más frecuentes se encuentran la cefalea cervicogénica, la cefalea por abuso de analgésicos, dolor craneal y las diferentes causas de cefaleas metabólicas. Se mencionan los estudios básicos y paraclínicos, así como los criterios para estudio por neuroimagen y las señales de alerta para cefaleas secundarias. (provisto por Infomedic International)

A literature review was conducted to facilitate the differential diagnosis of headaches in individuals over the age of 65. The review focused on primary and secondary headaches according to the International Headache Society Classification, highlighting the most common types among older individuals. The review described the acute or chronic nature, frequency, characteristics, and specific features of these headaches to aid in making a presumptive diagnosis and guide appropriate interventions. The most common primary headaches in older adults are tension-type headache and migraine. Among the secondary headaches, the most frequent ones include cervicogenic headache, medication-overuse headache, cranial pain, and various metabolic causes of headaches. The review also mentioned basic and paraclinical studies, criteria for neuroimaging, and warning signs for secondary headaches. (provided by Infomedic International)

Cisto gigante de segundo arco branquial em adulto: Relato de caso e revisão de literatura / Giant cyst of the second branchial arch in an adult: case report and literature review

Introdução: Os arcos branquiais são os precursores embriológicos da face, pescoço e faringe. As anomalias dos arcos branquiais são a segunda lesão congênita mais comum de cabeça e pescoço em crianças. Entre essas anomalias, estão os cistos de arcos branquiais (BCC), que surgem devido a uma incorreta obliteração das fendas branquiais, ainda no período embrionário. Os BCC podem ser assintomáticos, apenas percebidos incidentalmente, e não se manifestar até a idade adulta. Resultados: Anomalias do segundo arco branquial devem ser consideradas como um dos possíveis diagnósticos diferenciais de massas cervicais, especialmente as que se manifestam como um abaulamento em região lateral do pescoço. Os BCC são formações de revestimento epitelial, sem aberturas externas. Após seu diagnóstico, o tratamento é cirúrgico, usualmente por meio de uma incisão cervical transversa e cuidadosa dissecação das estruturas, com o objetivo de extirpar toda a lesão. Conclusão: O método descrito, de excisão da lesão, por meio de incisão transversa em região cervical, dissecção tecidual por planos e ressecção de massa cística, é uma opção para o tratamento dessa deformidade, com adequado resultado estético e boa reprodutibilidade.

Introduction: The branchial arches are the embryological precursors of the face, neck, and pharynx. Branchial arch anomalies are the second most common congenital head and neck lesions in children. Among these anomalies are branchial arch cysts (BCC), which arise due to incorrect obliteration of the branchial slits, still in the embryonic period. BCCs may be asymptomatic, only noticed incidentally, and not manifest until adulthood. Results: Anomalies of the second branchial arch should be considered as one of the possible differential diagnoses of neck masses, especially those that manifest as a bulge in the lateral region of the neck. BCCs are epithelial lining formations without external openings. After diagnosis, treatment is surgical, usually through a transverse cervical incision and careful dissection of the structures, with the aim of extirpating the entire lesion. Conclusion: The method described of excision of the lesion through a transverse incision in the cervical region, tissue dissection in planes, and resection of the cystic mass is an option for the treatment of this deformity, with adequate aesthetic results and good reproducibility.

Aspergilosis pulmonar crónica en asociación con tuberculosis activa: reporte de un caso

La aspergilosis es una infección fúngica causada por el microorganismo Aspergillus spp. Las manifestaciones clínicas dependen del estado inmunológico del paciente y de las alteraciones estructurales del parénquima pulmonar. Pese a su baja incidencia, siempre se debe considerar como diagnóstico diferencial en el contexto de pacientes con enfermedades pulmonares de base. Se presenta un caso de hombre de 66 años, agricultor, con antecedente de tuberculosis pulmonar y enfermedad pulmonar obstructiva crónica, quien consultó por cuadro de 5 meses de disnea progresiva, fiebre, tos, hemoptisis y pérdida de peso. Los hallazgos imagenológicos fueron sugestivos de tuberculosis pulmonar asociado a aspergiloma, lo que fue confirmado por tinción de hidróxido de potasio (KOH) y cultivo de hongos positivo para Aspergillus fumigatus. El Gene Xpert fue positivo para Mycobacterium tuberculosis demostrando coinfección activa.

Aspergillosis is a fungal infection caused by the microorganism Aspergillus spp. Clinical manifestations depend on the patient's immune status and structural alterations of the lung parenchyma. Despite its low incidence, it should always be considered as a differential diagnosis in the context of patients with underlying lung diseases. We present the case of a 66-year-old male farmer, with a history of pulmonary tuberculosis and chronic obstructive pulmonary disease, who presented with progressive dyspnea months, fever, cough, hemoptysis and weight loss for 5 months. Imaging findings were suggestive of aspergiloma- associated pulmonary tuberculosis, which was confirmed by potassium hydroxide (KOH) staining and positive fungal culture for Aspergillus fumigatus. Gene Xpert was positive for Mycobacterium tuberculosis showing active co-infection.

Dificultad diagnóstica en masa retroperitoneal. Reporte de un caso / Diagnostic difficulty in retroperitoneal mass: A case report

El Schwannoma se origina de la vaina perineural de Schwannoma, se detecta con frecuencia incidentalmente en estudios imagenológicos siendo el principal método diagnóstico la Tomografía Computada. El tratamiento es la resección quirúrgica con márgenes libres. Se presenta una paciente femenina de 49 años, en control por oncología por enfermedad de base, cáncer de mama izquierda, se identifica por TAC y PECT/TC imagen voluminosa en retroperitoneo situación lateroaórtica izquierda de configuración no quística e hipermetabólica, solicita biopsia percutánea, ante la falta de ventana, se decide exeresis completa de masa. Diagnóstico definitivo patológico Schwannoma. Sin indicación de tratamiento adyuvante, cursa buena evolución postoperatoria sin recidiva.

Schwannoma, a benign tumor that arises from Schwann cells of the perineural nerve sheath, is often incidentally detected in imaging tests and mainly diagnosed by CT scan. Treatment consists of surgical resection with clear margins. We present the case of a 49-year-old female patient subject to Oncology Department follow-up due to an underlying disease, left breast cancer. A large, hypermetabolic, noncystic mass in the retroperitoneal region is identified by CT and PECT/CT scan in the left lateral aortic area. A percutaneous biopsy is requested. Due to the limited acoustic window, complete resection of the mass is decided. Final histopathology diagnosis of Schwannoma. No adjuvant treatment indication; undergoing favorable postoperative progress, without recurrence

Adolescence Pseudolymphoma / Pseudolinfoma na adolescência

Objective: report an uncommon case of cutaneous pseudolymphoma in teenage years, undiagnosed for approximately 8 years old. Methodology: data were taken from medical records, patient interviews, photographic records of the injuries, diagnostic methods, and literature review. The paper was subjected and approved by the Research Ethics Committee (REC), under the number 4.952.193, authorized by the patient and their legal sponsor. Final Conclusions: the related case shows the importance of reliable and differential diagnoses since the patient carried the injury through approximately eight years without getting any diagnosis and/or treatment. Furthermore, the unusual age and the location of the injuries make the information presented here fundamental to helping other professionals and contributing to the Public Health System (AU).

Objetivo: Relatar um caso incomum de Pseudolinfoma Cutâneo na adolescência, não diagnosticado por aproximadamente oito anos. Método: As informações foram obtidas pela revisão do prontuário, entrevista com o paciente, registro fotográfico das lesões e dos métodos diagnósticos e revisão de literatura. O trabalho foi submetido e aprovado pelo Comitê de Ética em Pesquisa (CEP), número de aprovação 4.952.193, com autorização do paciente e seus responsáveis legais. Considerações Finais: o caso relatado evidencia a importância de um diagnóstico fidedigno e dos diagnósticos diferenciais, uma vez que a paciente apresentou a lesão por aproximadamente oito anos, sem receber nenhum diagnóstico e/ou tratamento. Além disso, não só a faixa etária é incomum, mas também a localização da lesão e por esta razão, as informações são fundamentais para auxiliar outros profissionais, com benefício deste estudo para o Sistema de Saúde (AU).

Presentación clínica de patologías no malignas que simulan leucemia cutis: a propósito de dos casos / Clinical presentation of non-malignant diseases mimicking leukemia cutis: A report of two cases

La infiltración cutánea por células leucémicas conocida como leucemia cutis es una presentación infrecuente de esta patología y constituye un desafío diagnóstico. Los diagnósticos como infecciones, otras patologías neoplásicas con afectación cutánea y los trastornos histiocíticos, entre otros, constituyen los principales diagnósticos diferenciales, ya que configuran un escenario pronóstico y terapéutico diferente. Se presentan dos pacientes que fueron diagnosticados inicialmente como leucemia cutis, cuyo diagnóstico final fue de patologías no malignas.

The infiltration of leukemia cells into the skin, known as leukemia cutis, is a rare presentation of this disease and accounts for a diagnostic challenge. The main differential diagnoses include infections, other neoplastic diseases with skin involvement and histiocytic disorders, among others, as they entail different prognostic and therapeutic approaches. Here we describe two patients who were initially diagnosed with leukemia cutis, whose final diagnosis was of non-malignant diseases.

Differential Diagnosis of Oral Metastases / Diagnóstico Diferencial de las Metástasis Orales

Metastatic lesions in the mouth can resemble common inflammatory lesions. Therefore, we set out to investigate oral metastases whose clinical and imaging characteristics mimicked those of harmless lesions, confusing and delaying the diagnosis. For this, a systematic review was carried out from case reports, case series, and cross-sectional studies in the PubMed/Medline, Scopus, Embase-via Elsevier, Virtual Health Library, Web of Science, and gray literature, using PICO strategy without period restriction. We assessed the quality of studies using the Joanna Briggs Institute assessment tool. A narrative synthesis of the data was carried out. Association analyses using chi-square and Fisher's exact tests were performed, with statistical significance at p<0.05. Most of the lesions came from the lung, breast, kidneys, liver, and thyroid. They affected mainly the mandibles of men, between the fifth and seventh decades of life, causing osteolysis. In soft tissue, there were firm swellings, associated with bleeding. Limitations regarding the heterogeneity of the included studies and the absence of clinic pathological descriptions of the tumors substantially reduced the chance of statistical analysis of the data. Knowing the different possibilities of clinical presentation of oral and maxillofacial metastases is important for the diagnost ic suspicion to occur and diagnostic errors to be avoided. Thus, treatment is instituted and survival can be extended. Protocol registration: PROSPERO CRD42020200696.

Las lesiones metastásicas en la cavidad oral pueden parecer similares a lesiones inflamatorias comunes. Por ello, nos propusimos investigar metástasis orales cuyas características clínicas e imagenológicas simularan las de lesiones inofensivas, confundiendo y retrasando el diagnóstico. Para ello, se realizó una revisión sistemática a partir de reportes de casos, series de casos y estudios transversales en PubMed/Medline, Scopus, Embase-vía Elsevier, Virtual Health Library, Web of Science y literatura gris, utilizando la estrategia PICO sin restricción de periodo. La calidad de los estudios se evaluó mediante la herramienta de evaluación del Instituto Joanna Briggs. Se realizó una síntesis narrativa de los datos. Se realizaron análisis de asociación mediante chi-cuadrado y prueba exacta de Fisher, con significancia estadística en p<0,05. La mayoría de las lesiones procedían de pulmón, mama, riñones, hígado y tiroides. Afectan principalmente a las mandíbulas de los hombres, entre la quinta y la séptima década de la vida, provocando osteólisis. En los tejidos blandos, había hinchazones firmes, asociadas con sangrado. Las limitaciones con respecto a la heterogeneidad de los estudios incluidos y la ausencia de descripciones clinicopatológicas de los tumores redujeron sustancialmente la posibilidad de realizar un análisis estadístico de los datos. Conocer las diferentes posibilidades de presentación clínica de las metástasis orales y maxilofaciales es importante para que se produzca la sospecha diagnóstica y se eviten errores diagnósticos. Por lo tanto, se instituye el tratamiento y se puede prolongar la supervivencia. Registro de protocolo: PROSPERO CRD42020200696.

Diagnóstico diferencial: ¿Qué es, cómo se hace, dónde lo enseñan? / Differential diagnosis: what is it, how is it done and where is it taught?

El diagnóstico es la piedra angular de la medicina individual, por tanto, dominarlo y conocerlo es esencial para todo médico al indagar en el estado de salud y patológico de los pacientes. Los profesionales de la salud deben dedicar todos sus esfuerzos a su realización, siempre que disponga de los elementos y medios necesarios, tanto teóricos como prácticos, para la correcta utilización del método clínico, elemento esencial del diagnóstico diferencial. En este artículo se abordan los aspectos más relevantes que intervienen en la realización del diagnóstico de los pacientes; se enfatiza en los pasos necesarios para efectuar un verdadero diagnóstico diferencial que posibilite la decantación de las posibilidades etiológicas del cuadro clínico del enfermo. A través de la correcta aplicación del método clínico es posible la aproximación al diagnóstico clínico definitivo del paciente.

Diagnosis is the cornerstone of individual medicine, therefore, mastering it and knowing it is essential for every doctor when inquiring into the health and pathological status of patients. Health professionals must dedicate all their efforts to its realization as long as they have the necessary elements and means, both theoretical and practical, for the correct use of the clinical method, which is an essential element of differential diagnosis. This article addresses the most relevant aspects involved in carrying out patient's diagnosis; emphasis is placed on the necessary steps to carry out a true differential diagnosis that makes it possible to decant the etiological possibilities of the patient's clinical manifestations. It is possible to approach the definitive clinical diagnosis of the patient through the correct application of the clinical method.

Crohn's Disease in a Patient with Tuberculosis: A Diagnostic and Therapeutic Challenge

Introduction: Crohn's disease (CD) is unusual in Colombia. Tuberculosis (TB) occurs more frequently, but intestinal involvement is rare. Differentiating these two entities and treating the cases in which they coexist is a challenge. Clinical case: A 28-year-old man with three months of constitutional, respiratory, and GI symptoms was initially diagnosed with pulmonary tuberculosis, and all the clinical manifestations were attributed to this entity. Given the absence of improvement with treatment and the sum of radiological, endoscopic, and pathological findings, CD was confirmed. Treatment was complex due to the coexistence of the two entities, although he finally went into remission with the use of biologicals. Discussion: Diagnosing CD requires the sum of clinical and paraclinical findings. A therapeutic test may be necessary to differentiate it from intestinal TB. The treatment of CD in a patient with TB has some limitations; steroids are not contraindicated, and biologicals must be initiated cautiously. Conclusions: Differentiating CD from intestinal TB is a diagnostic challenge. Therapeutic management when these two entities coexist requires an interdisciplinary approach.

Introducción: la enfermedad de Crohn (EC) es inusual en Colombia. La tuberculosis (TB) se presenta con mayor frecuencia, pero el compromiso intestinal es raro. Diferenciar estas dos entidades y el tratamiento de los casos en los que coexisten es un reto. Caso clínico: hombre de 28 años con 3 meses de síntomas constitucionales, respiratorios y gastrointestinales al que en un inicio se le confirmó el diagnóstico de tuberculosis pulmonar y se le atribuyó a esta entidad todas las manifestaciones clínicas. Ante la ausencia de mejoría con el tratamiento y la suma de hallazgos radiológicos, endoscópicos y patológicos, se confirmó EC. El tratamiento fue difícil debido a la coexistencia de las dos entidades, aunque finalmente presentó remisión con el uso de biológicos. Discusión: diagnosticar la EC requiere de la sumatoria de hallazgos clínicos y paraclínicos. Para diferenciarla de TB intestinal puede llegar a ser necesaria una prueba terapéutica. El tratamiento de EC en un paciente con TB tiene algunas limitaciones, los esteroides no se contraindican de manera absoluta y el inicio de biológicos debe hacerse con precaución. Conclusiones: diferenciar la EC de la TB intestinal es un reto diagnóstico. El enfoque terapéutico cuando coexisten estas dos entidades requiere un abordaje interdisciplinario.

Características clínicas de neoplasia de células dendríticas plasmocitoides blásticas en Chile: reporte de 10 casos

Introducción: La neoplasia de células dendríticas plasmocitoides blásticas (NCDPB) es una hemopatía maligna poco frecuente y de mal pronóstico, con reportes de casos aislados en la realidad nacional. Produce compromiso cutáneo y de médula ósea y frecuentemente es confundida con otras patologías al diagnóstico. El presente trabajo tiene como objetivo describir las características clínicas de 10 pacientes diagnosticados en centros asistenciales chilenos. Material y Métodos: Se obtuvo en forma retrospectiva información clínica e inmunofenotípica de pacientes diagnosticados de NCDPB en los centros participantes en el periodo 2013-2021. Resultados: Se identificaron 10 pacientes, el 80% de sexo masculino, con una mediana de edad de 66 años (15-81). Los diagnósticos iniciales de derivación más frecuentes fueron linfoma T (4/10) y leucemia aguda mieloblástica (3/10). La mayoría presentó afección cutánea (7/10) y compromiso de médula (7/10) y en menor frecuencia adenopatías, esplenomegalia y hepatomegalia. En el hemograma se observó anemia y leucopenia, con blastos en frotis en 5/10. Se indicó CHOP en 8/10 casos con remisión en 5/8 y en un caso HyperCVAD seguido de trasplante alogénico de médula ósea. La mediana de sobrevida fue de 10 meses (IC 95% 4,2-15,8 meses) con 9/10 fallecidos. Se documentó recaída en sistema nervioso central en 2 casos. Conclusiones: La NCDPB es una patología poco frecuente que se presenta en la realidad nacional de forma similar a lo descrito en la literatura. Es susceptible de responder a quimioterapia inicial asociada a terapia intratecal.

Background: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant tumor with a dismal prognosis, with isolated case reports in Chile. The BPDCN can present skin and bone marrow compromise, and its diagnosis is frequently confused with other pathologies. This study aimed to evaluate the clinical and immunophenotypical features of BPDCN in the Chilean population. Methods: We performed a retrospective study from 2013 to 2021 in clinical records of 2 public Chilean referral hospitals, including ten patients, 80% male, with a median age of 66 years (15-81). Results: The most frequent initial referral diagnoses were T-cell lymphoma (4/10) and acute myeloblastic leukemia (3/10). Seven patients presented skin and bone marrow involvement; we found a lower frequency of adenopathies (5/10), splenomegaly (2/10), and hepatomegaly (2/10). The complete blood count revealed anemia and leukopenia, with blasts in 5/10. Nine patients received induction therapy. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) was indicated in 8/10 cases with remission in 5/8, and 1 patient received HyerCVAD (cyclophosphamide, vincristine, doxorubicin and dexamethasone, methotrexate, cytarabine) and an allogeneic bone marrow transplant. The median survival was 10 months (95% CI 4.2-15.8 months) with 9/10 deaths. Relapse in the central nervous system was documented in 2 cases. Conclusions: Our study found that BPDCN, a rare pathology in the Chilean population, shows a similar clinical presentation compared to previous studies. It is susceptible to respond to initial systemic and intrathecal chemotherapy.