Malaria cerebral con pancitopenia por plasmodium vivax en la amazonía peruana: reporte de caso / Plasmodium vivax cerebral malaria with pancytopenia in the peruvian amazon: case report

RESUMEN Plasmodium vivax es la especie más común en la Amazonía peruana y ocasiona el 81% del total de casos de malaria. Presentamos el caso de un paciente adulto varón con malaria cerebral por Plasmodium vivax, que inicia con malestar general y fiebre, luego presenta convulsiones más de dos veces al día con pérdida de consciencia y limitación funcional motora. Se le realiza gota gruesa donde se observa trofozoítos de Plasmodium vivax y depresión de las tres series sanguíneas. Se inicia tratamiento con artesunato y clindamicina por cinco días, se le transfunde un paquete globular, y continua con primaquina por siete días. El paciente muestra mejoría clínica con secuela neurológica en extremidad inferior izquierda.

ABSTRACT Plasmodium vivax causes 81% of all malaria cases and is the most common species in the Peruvian Amazon. We present the case of a male patient with cerebral malaria caused by Plasmodium vivax, who had general malaise and fever, and then presented seizures more than twice a day with loss of consciousness and motor functional limitation. Plasmodium vivax trophozoites were detected by thick blood smear, besides, we also observed low counts of all three blood cell types. Treatment began with artesunate and clindamycin for five days, then one unit of packed red blood cells was transfused; treatment continued with primaquine for seven days. The patient showed clinical improvement with neurological sequelae in one lower limb.

Aetiology of pancytopenia: Experience of a South African tertiary academic centre

Background: Pancytopenia is a manifestation of numerous disease entities. The causes of pancytopenia differ with geographic region, socio-economic factors and HIV prevalence. Awareness of the common causes of pancytopenia may aid timely diagnosis. Objective: This study aimed to determine the aetiology of pancytopenia in a South African population.Methods: A retrospective observational study of adult patients presenting with pancytopenia at Tygerberg Academic Hospital, South Africa, from January 2016 to December 2017 was performed. Data on pancytopenia cases were obtained from the laboratory information system and utilised to determine the causes of pancytopenia. Results: A total of 673 cases of pancytopenia were identified. The most common causes of pancytopenia were chemoradiation therapy (25%), sepsis (18%), haematological malignancy (9%), advanced HIV (7%), and megaloblastic anaemia (6%). The diagnostic yield of bone marrow examinations (BME) was 57% (n = 52/91). The aetiology of pancytopenia differed according to age, with malignancy being a more common cause of pancytopenia among the elderly. Conclusion: Several easily recognisable and treatable conditions can manifest as pancytopenia. Prompt management of such conditions, notably sepsis and megaloblastic anaemia, can result in the resolution of the cytopenias and negate the need for a BME. However, haematological malignancy and unexplained pancytopenia strongly rely on a BME to establish a diagnosis. Pancytopenia investigations, when guided by appropriate clinic-laboratory findings, can promptly identify the underlying aetiology, while also identifying cases where an expedited BME is required. This is valuable in resource-conscious medicine

Hipoplasia de medula óssea: manifestação atípica do hipertireoidismo / Bone marrow hypoplasia: atypical manifestation of hyperthyroidism / Hipoplasia de la médula ósea: manifestación atípica del hipertiroidismo

Alterações hematológicas, como anemia ou pancitopenia, podem ser manifestações atípicas do hipertireoidismo. Embora a maioria dos casos de pancitopenia na doença de Graves seja decorrente do uso de drogas antitireoidianas ­ tionamidas ­, ela também pode estar associada ao quadro de hipertireoidismo não tratado e, assim, se manifestar como uma complicação grave. Este estudo tem como objetivo relatar o caso de uma paciente portadora de hipertireoidismo que evoluiu durante o internamento por pancitopenia secundária à doença primária. As informações foram obtidas por meio da revisão do prontuário, entrevista com a paciente e revisão da literatura. Trata-se de uma paciente do sexo feminino, de 39 anos, internada em um hospital de Salvador por apresentar um quadro de edema em membros inferiores que evoluiu progressivamente para anasarca por 45 dias. Associados ao quadro, a paciente apresentava perda ponderal, dispneia paroxística noturna, taquicardia, sudorese e calafrios, além dos exames laboratoriais terem apresentado pancitopenia. O caso relatado demonstra uma manifestação atípica e rara do hipertireoidismo, que melhorou após o início do tratamento e do estado de eutireoidismo.

Hematological changes such as anemia or pancytopenia can occur as atypical manifestations of hyperthyroidism. Although most cases of pancytopenia in Graves' disease are due to the use of antithyroid drugs (thionamides), it can also be associated with untreated hyperthyroidism, thus manifesting as a serious complication. This study aims to report the case of a 39-year-old hyperthyroid female patient who developed pancytopenia during hospitalization. Data were collected by means of the medical record, an interview conducted with the patient, and literature review. The patient was admitted to a hospital in Salvador presenting lower extremity edema that progressively evolved to anasarca within 45 days prior to the interview. She also presented weight loss, paroxysmal nocturnal dyspnea, tachycardia, sweating, chills, and laboratory test results for pancytopenia. The case reported here demonstrates an atypical and rare manifestation of hyperthyroidism that improved after the beginning of the treatment, reaching the euthyroid state.

Los cambios hematológicos como anemia o pancitopenia pueden ocurrir como manifestaciones atípicas de hipertiroidismo. Aunque la mayoría de los casos de pancitopenia en la enfermedad de Graves se deben al uso de fármacos antitiroideos (tionamidas), también se puede asociar a um hipertiroidismo no tratado y, por tanto, puede manifestarse como una complicación grave. Este estudio tiene como objetivo reportar el caso de una paciente con hipertiroidismo que desarrolló durante la hospitalización pancitopenia secundaria a la enfermedad primaria. La información se obtuvo revisando la historia clínica, entrevistando a la paciente y revisando la literatura. Se trata de una paciente de 39 años ingresada en un hospital de Salvador por presentar edema en miembros inferiores, evolucionando progresivamente a anasarca durante 45 días. Asociado a la afección, presentaba pérdida de peso, disnea paroxística nocturna, taquicardia, sudoración, escalofríos y análisis de laboratorio que mostraban pancitopenia. El caso aquí reportado demuestra una manifestación atípica y rara de hipertiroidismo, que evolucionó luego del inicio del tratamiento y del estado de eutiroidismo.

Quality of life and changes in the social dimension of hematopoietic stem cell transplants recipients / Calidad de vida y cambios en el dominio social de los receptores de trasplantes de células madre hematopoyéticas / Qualidade de vida e alterações no domínio social de transplantados de células-tronco hematopoéticas

ABSTRACT Objectives: to assess/correlate health-related quality of life with the social dimension of hematopoietic, autologous and allogeneic stem cell transplant patients in the three years post-transplant. Methods: longitudinal, observational study with 55 patients, in a reference hospital in Latin America, from September 2013 to February 2019, using the Quality of Life Questionnaire-Core and Functional Assessment Cancer Therapy Bone Marrow Transplantation. Results: A total of 71% underwent allogeneic transplantation. The social dimension had low averages since the baseline stage (55, 21) and low scores (56) for quality of life in pancytopenia. There was a significant positive correlation between social dimension, quality of life in pancytopenia (p<0.01) and follow-up after hospital discharge (p<0.00). There is a significant difference (p<0.00) throughout the stages, however, not in terms of the type of transplant (p>0.36/0.86). Conclusions: patients with better assessments in the social dimension have a better quality of life. Interventions focusing on the multidimensionality of the quality of life construct are necessary.

RESUMEN Objetivo: evaluar/correlacionar la calidad de vida relacionada a la salud con el ámbito social de pacientes trasplantados de células madre hematopoyéticas, autólogas y alogénicas, tres años post trasplante. Métodos: se trata de un estudio longitudinal, observacional, llevado a cabo en un hospital referencial de América Latina con 55 pacientes entre septiembre de 2013 y febrero de 2019, utilizando la Escala de Calidad de Vida-Núcleo y la Evaluación funcional de la terapia del cáncer: trasplante de médula ósea. Resultados: el 71% realizó un trasplante alogénico; el ámbito social presentó promedios bajos desde la etapa inicial (55, 21) y puntuaciones bajas (56) en cuanto a la calidad de vida en la pancitopenia. Se observó una correlación positiva significativa entre el ámbito social, la calidad de vida en la pancitopenia (p<0,01) y el seguimiento después del alta hospitalaria (p<0,00). Hubo una diferencia significativa (p<0,00) a lo largo de los estadios, pero no en lo que respecta a la modalidad del trasplante (p>0,36/0,86). Conclusiones: aquellos pacientes con mejor evaluación en el ámbito social presentan mejor calidad de vida. Es necesario realizar intervenciones que se centren en la multidimensionalidad de la construcción de la calidad de vida.

RESUMO Objetivo: avaliar/correlacionar a qualidade de vida relacionada à saúde com o domínio social de pacientes transplantados de células-tronco hematopoéticas, autólogo e alogênico, nos três anos pós-transplante. Métodos: estudo longitudinal, observacional, com 55 pacientes, num hospital referência da América Latina, de setembro de 2013 a fevereiro de 2019, com o Quality of Life Questionnarie-Core e Functional Assessment Cancer Therapy Bone Marrow Transplantation. Resultados: 71% realizaram transplante alogênico. O domínio social apresentou médias baixas desde a etapa basal (55, 21) e escores baixos (56) para a qualidade de vida na pancitopenia. Houve correlação positiva significativa entre domínio social, qualidade de vida na pancitopenia (p<0,01) e acompanhamento após alta hospitalar (p<0,00). Observa-se diferença significativa (p<0,00) ao longo das etapas, porém, não quanto à modalidade de transplante (p>0,36/0,86). Conclusões: pacientes com melhor avaliação no domínio social apresentam melhor qualidade de vida. Intervenções com foco na multidimensionalidade do constructo qualidade de vida são necessárias.

Hemophagocytic lymphohistiocytosis as a presentation of inflammatory bowel disease / Linfohistiocitosis hemofagocítica como presentación de la enfermedad inflamatoria intestinal

Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is considered a medical emergency that should be recognized in patients with fever, splenomegaly, and progressive deterioration of the general condition. Laboratory findings include cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. For HLH diagnosis, it is essential, although not mandatory, to perform a bone marrow biopsy. Given its nature, secondary causes of HLH should be sought, mainly infections, hemato-oncological disorders, autoimmune diseases, and auto-inflammatory conditions. Case report: We present the case of a female adolescent who presented with fever and lower gastrointestinal bleeding. Upon admission, acute liver failure and pancytopenia were documented. A bone marrow aspirate was performed, which revealed hemophagocytosis; other tests confirmed HLH diagnosis. During the diagnostic approach, inflammatory bowel disease was diagnosed. The patient received first-line treatment with an adequate response. Conclusions: Inflammatory bowel disease can be considered a cause of secondary HLH, particularly in patients with suggestive symptoms, such as digestive bleeding in the absence of other secondary causes of HLH.

Resumen Introducción: La linfohistiocitosis hemofagocítica (LHH) es considerada una urgencia médica que debe reconocerse en pacientes con deterioro progresivo del estado general, fiebre, pancitopenia y esplenomegalia. Los hallazgos de laboratorio incluyen citopenia, hipertrigliceridemia, hipofibrinogenemia e hiperferritinemia. Para su diagnóstico es importante, aunque no obligatoria, la realización de aspirado de médula ósea. Dada su naturaleza, se deben buscar causas secundarias de LHH, principalmente enfermedades infecciosas, hematooncológicas, autoinmunitarias y autoinflamatorias. Caso clínico: Se presenta el caso de una adolescente que inició con fiebre y sangrado digestivo bajo. A su ingreso, se documentó falla hepática aguda y pancitopenia. Se realizó aspirado de médula ósea y se encontró hemofagocitocis; el resto de los exámenes concluyeron LHH. Durante su abordaje se diagnosticó enfermedad inflamatoria intestinal. La paciente recibió tratamiento de primera línea con adecuada respuesta. Conclusiones: La enfermedad inflamatoria intestinal puede considerarse como una causa secundaria de LHH, en particular en pacientes con clínica sugestiva, como es el sangrado digestivo, en ausencia de otras causas secundarias de LHH.

Acidemia propiónica: una causa sumamente rara de linfohistiocitosis hemofagocítica en un lactante / Propionic acidemia: an extremely rare cause of hemophagocytic lymphohistiocytosis in an infant

La linfohistiocitosis hemofagocítica (LHH) puede ser primaria (hereditaria) o secundaria a infecciones, tumores malignos, trastornos reumatológicos, síndromes de inmunodeficiencia y metabolopatías. Se informaron casos de intolerancia a la proteína lisinúrica, deficiencia de múltiples sulfatasas, galactosemia, enfermedad de Gaucher, síndrome de Pearson y galactosialidosis. No se sabe cómo se desencadena la LHH en las metabolopatías. Se diagnosticó LHH en un lactante de 2 meses con letargo, palidez, alimentación deficiente, hepatoesplenomegalia, fiebre y pancitopenia, y se instauró el protocolo HLH-2004. Se realizaron, en conjunto, análisis para detectar mutaciones genéticas y pruebas metabólicas; los resultados fueron negativos para las mutaciones genéticas de LHH primaria, pero se detectaron hiperamoniemia y concentración elevada de metilcitrato. Se diagnosticó acidemia propiónica. Aquí informamos sobre un caso de LHH secundaria a acidemia propiónica. Es posible la realización simultánea de pruebas de detección de trastornos metabólicos y de mutaciones genéticas para el diagnóstico temprano en los lactantes con LHH

Hemophagocytic lymphohystiocytosis (HLH) may be primary (inherited/familial) or secondary to infections, malignancies, rheumatologic disorders, immune deficiency syndromes and metabolic diseases. Cases including lysinuric protein intolerance, multiple sulfatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis have previously been reported. It is unclear how the metabolites trigger HLH in metabolic diseases. A 2-month-old infant with lethargy, pallor, poor feeding, hepatosplenomegaly, fever and pancytopenia, was diagnosed with HLH and the HLH-2004 treatment protocol was initiated. Analysis for primary HLH gene mutations and metabolic screening tests were performed together; primary HLH gene mutations were negative, but hyperammonemia and elevated methyl citrate were detected. Propionic acidemia was diagnosed with tandem mass spectrometry in neonatal dried blood spot. We report this case of HLH secondary to propionic acidemia. Both metabolic disorder screening tests and gene mutation analysis may be performed simultaneously especially for early diagnosis in infants presenting with HLH.

Parvovirus B19: un nuevo patógeno emergente en pacientes con SIDA. Presentación de 3 casos / PARVOVIRUS B19: HUMAN PARVOVIRUS B19: A NEW EMERGING DISEASE IN PATIENTS WITH AIDS. REPORT OF THREE PATIENTS

El Parvovirus humano B19 puede presentarse con una amplia variedad de manifestaciones clínicas, con distinto compromiso y evolución según el huésped afectado. En pacientes inmunocomprometidos se asocia con cuadros hematológicos prolongados y graves. Se describen 3 casos de pacientes con antecedentes de infección por virus de la inmunodeficiencia humana (VIH) que desarrollaron infecciones agudas por Parvovirus B19 que se presentaron con síndrome febril, citopenias (anemia, plaquetopenia y disminución de reticulocitos) y esplenomegalia. En todos los casos el diagnóstico se confirmó por la serología específica. Todos recibieron tratamiento con inmunoglobulina humana (Ig) intravenosa (IV); 2 pacientes tuvieron buena respuesta clínica y mejoría de citopenias mientras que el restante falleció. La infección por Parvovirus B19 debe incluirse en el diagnóstico diferencial de los pacientes VIH positivos con fiebre y citopenias, principalmente anemia persistente y compromiso linfoganglionar con esplenomegalia

Human Parvovirus B 19 is presented as a variety of diseases with different compromise and evolution according to the affected host. In immunocompromised patients the acute infection due to Parvovirus B19 is associated with severe and prolonged hematological clinical pictures. Three cases of patients with a history of infection with human immunodeficiency virus (HIV) co-infected with Human Parvovirus B19 are presented. All of they presented with febrile syndrome, cytopenias (anemia, platelet count and reticulocyte reduction) and lymphadenopathy and splenomegaly. In all cases the diagnosis was confirmed by serology. All were treated with intravenous human immunoglobulin (IVI G; 2 patients had good clinical response and better cytopenias while the other died. We consider thinking about Parvovirus B19 infection in HIV immunocompromised hosts with haematological involvement, mainly persistent anemia and lymph node involvement with splenomegaly

Deficiência de cianocobalamina como causa de pancitopenia: relato de um caso e levantamento de literatura / Cyanocobalamin deficiency as a cause of pancytopenia: case report and survey of literature

Contexto: A pancitopenia é uma condição clínica grave caracterizada pela diminuição simultânea dos níveis séricos dos glóbulos vermelhos, brancos e plaquetários. A sua resolução só ocorre quando tratado o processo etiológico. Descrição do caso: Nesse estudo, relatou-se o caso de um paciente pediátrico, procedente de região carente, que foi trazido ao serviço de referência da região pela mãe com quadro progressivo de queda do estado geral, inapetência, diarreia, náuseas e vômitos. Esses sintomas haviam se iniciado cinco dias antes. No recordatório alimentar, a responsável relatou dieta pobre em alimentos de origem animal e vegetal, tendo, ainda, histórico de internação recente. Ao exame físico, apresentou-se hipocorado, com palidez cutânea, sinais vitais normais e sem demais alterações dignas de nota. Inicialmente, solicitaram-se exames complementares que mostraram pancitopenia associada a leucocitúria. Instituídos sintomáticos e antibioticoterapia de amplo espectro para determinado foco. No sexto dia de internação, o paciente evoluiu com quadro séptico, necessitando de transferência e terapia intensiva. Após estabilização do quadro infeccioso, o paciente retornou à enfermaria do serviço para continuidade do tratamento e investigação etiológica da pancitopenia. Por meio de exames complementares, foi possível identificar que a anemia normocítica e normocrômica se tratava de deficiência de vitamina B12 de apresentação atípica. Discussão: Diante de casos com apresentação atípica associada à pancitopenia, é necessário considerar a possibilidade de deficiência de micronutrientes, por se tratar de uma condição frequente em nosso país. Conclusão: O presente relato de caso pode ser utilizado como alerta a profissionais de saúde, para que considerem a deficiência de cianocobalamina como causa de pancitopenia, principalmente em regiões mais vulneráveis, visto que essa condição pode estar associada a quadros graves e que ameaçam a vida.

Zigomicosis y síndrome hemofagocítico en un paciente inmunocompetente: informe de caso / Zygomycosis and hemophagocytic lymphohistiocytosis in an immunocompetent patient: a case report

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)

Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)

Infecção por parvovírus B19 em receptores de transplante renal: série de caso / Parvovirus B19 infection in kidney transplant recipients: a case series

Os receptores de transplante renal são mais suscetíveis a infecções, entre elas o parvovírus B19, que pode ser transmitido por via respiratória, adquirido por meio do enxerto ou por reativação de infecção latente. A anemia normocítica normocrômica, com diminuição dos reticulócitos e resistência ao tratamento com eritropoietina, é a principal forma de apresentação da infecção por parvovírus B19 em transplante renal. O diagnóstico requer alto índice de suspeição clínica e realização de testes diagnósticos selecionados. Tratamento com imunoglobulina e suspensão dos imunossupressores durante a infecção mostraram-se eficazes. Os autores relatam sua experiência com cinco casos de infecção por parvovírus B19 em receptores de transplante renal de um hospital universitário. Os aspectos clínicos, diagnósticos e terapêuticos são revistos.

Kidney transplant recipients are more susceptible to infections, including by parvovirus B19, spread through the respiratory tract, acquired through the graft or reactivation of latent infection. Normocytic normochromic anemia, with decreased reticulocytes and resistance to erythropoietin treatment, is the most common presentation of Parvovirus B19 infection in renal transplant. Diagnosis requires a higher clinical suspicion and the performance of selected diagnostic tests. Treatment with immunoglobulin and suspension of immunosuppressive therapy during the infection may be effective. The authors report five cases of PB19 infection in kidney transplant patients at a hospital. The clinical, diagnostic, and treatment features are reviewed.

Successful multiple-exchange peritoneal dialysis in a patient with severe hematological toxicity by methotrexate: case report and literature review / Diálise peritoneal com múltiplas trocas bem-sucedida em paciente com grave toxicidade hematológica por metotrexato: relato de caso e revisão de literatura

Abstract Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.

Resumo Apesar de sua toxicidade, o metotrexato é um medicamento eficaz no controle de várias doenças. A mielossupressão, um de seus principais efeitos adversos, aumenta em gravidade e frequência nos pacientes com insuficiência renal. Apresentamos o caso de um homem de 68 anos de idade com doença renal terminal relacionada à vasculite associada ao ANCA em diálise peritoneal, que recebeu a medicação em dose baixa em função da atividade da doença e que teve como complicação pancitopenia grave com mucosite, tratada com medidas de suporte e diálise peritoneal com múltiplas trocas. Revisamos 20 casos publicados até o presente momento sobre pancitopenia associada a metotrexato em pacientes em diálise. Foi identificada alta morbidade e mortalidade, razão pela qual seu uso nesse tipo de paciente não é recomendado. No entanto, quando esta complicação ocorre, uma opção terapêutica pode ser o uso de diálise peritoneal com múltiplas trocas, além da terapia de suporte para toxicidade medicamentosa. Maiores estudos são necessários para demonstrar o papel da diálise peritoneal com múltiplas trocas na remoção desse medicamento.

Infección por Rhodotorula rubra en el lactante con leucemia linfoblástica aguda. Reporte de un caso / Rhodotorula rubra infection in infants with acute lymphoblastic leukemia. Case report

Rhodotorula especies (spp.) son levaduras comensales de la familia Sporidiobolaceae. Rhodotorula rubra es un patógeno oportunista y emergente que tiene la habilidad de colonizar e infectar a los pacientes susceptibles como son los que padecen de enfermedades hematológicas malignas, y está asociado con el uso de catéteres venosos centrales. Hay pocos reportes de casos en niños inmunocomprometidos. Presentamos el caso de una paciente lactante con diagnóstico de leucemia linfoblástica aguda (LLA) B común, que presentó infección fúngica por Rhodotorula rubra.

Rhodotorula species (spp.) are commensal yeasts of the family Sporidiobolaceae. Rhodotorula rubra is an opportunistic and emerging pathogen with the ability to colonize and infect susceptible patients like patients with malignant haematological diseases and it is associated with the use of central venous catheters. Only a few reports have linked found in immunocompromised children. In this article we present the case report of a lactating patient with a diagnosis of common acute lymphoblastic leukemia (B), who presented a fungal infection by Rhodotorula rubra.

Metastasis of Rhabdomyosarcoma to the Male Breast: a Case Report with Magnetic Resonance Imaging Findings

Metastasis of rhabdomysarcoma to the breast is a very rare manifestation in adult males. Herein, we report a case of metastasis from embryonal rhabdomyosarcoma in the left hypothenar muscle that presented as a breast mass in a 38-year-old man, who four months later expired because of multiple bone metastases related to pancytopenia. We describe the various imaging findings, including mammograms, ultrasonography, computerized tomography (CT), positron emission tomography-computed tomography (PET-CT), and magnetic resonance imaging (MRI) of this rare disease. The various imaging findings of this lesion could be helpful for future diagnosis of male breast lesions.

Therapeutic Plasma Exchange in a Patient with Hemophagocytic Lymphohistiocytosis

A 22-year old female patient with systemic lupus erythematosus presenting microangiopathic hemolytic anemia was treated with therapeutic plasma exchange 23 times. The patient's condition and laboratory findings (aspartate aminotransferase, alanine aminotransferase, ferritin, total bilirubin, and lactate dehydrogenase) did not improve despite the initial 18 therapeutic plasma exchange treatments. Thrombotic thrombocytopenic purpura was ruled out due to normal ADAMTS-13 activity test result; hemophagocytic lymphohistiocytosis was diagnosed based on fever, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow aspiration. The patient's condition improved rapidly upon treatment with a combination of immunosuppressants and cytotoxic agents, and more therapeutic plasma exchanges were performed five consecutive times with prolonged intervals in between. We observed that therapeutic plasma exchange treatment alone was not effective enough to treat hemophagocytic lymphohistiocytosis, unlike thrombotic thrombocytopenic purpura. Therefore, it is necessary to determine and start drug administration promptly in the treatment of hemophagocytic lymphohistiocytosis with thrombotic microangiopathy.

A Report of Bone Marrow Metastasis of Colon Cancer as a Primary Diagnosis, Supported by Cytokeratin Immunohistochemical Staining

Bone marrow metastasis of colon cancer is rare. Here, we report a 56-year-old female patient who presented with pancytopenia. She was diagnosed with colon cancer accompanied by lung and axial skeleton metastasis. The bone marrow study showed metastatic carcinoma. Immunohistochemical (IHC) staining with anti-cytokeratin 7 (CK7) and anti-cytokeratin 20 (CK20) antibodies showed that the bone marrow samples were negative for CK7 and positive for CK20, consistent with metastatic colon cancer. To the best of our knowledge, there has been only one other reported case of bone marrow metastasis of colon cancer as the primary diagnosis in an adult patient in Korea. Bone and bone marrow metastases of colon cancer are regarded as uncommon. However, for proper management, bone marrows should be promptly examined in patients with solid tumors when unexplained cytopenia is noted, even if the origin of the tumor is known to be rarely metastatic to bone marrow. In addition, the use of cytokeratin IHC staining is helpful for determining the origin of metastatic carcinoma.

Evaluation of hematologic profile may be needed for patients treated with oxcarbazepine / 소아과

PURPOSE: The major side effects of treatment with oxcarbazepine (OXC) are skin rash and hyponatremia. Hematologic side effects are reported rarely. The aim of this study was to investigate the rate and types of the hematologic side effects of OXC. METHODS: The medical records of 184 patients diagnosed with epilepsy or movement disorder and on OXC monotherapy, at the Department of Pediatrics of Inje University Sanggye Paik Hospital from July 2001 to July 2018, were retrospectively reviewed. RESULTS: Of the 184 patients, 10 (5.4%) developed leukopenia in addition to pancytopenia and 2 (1.0%) developed pancytopenia. Leukopenia developed in 11 days to 14 years after OXC administration and was more frequent in males than in females (male vs. female, 9 vs. 1; Fisher exact test, P0.05, t-test). CONCLUSION: OXC-induced leukopenia is not rare and may result in pancytopenia. Patients being treated with OXC should be regularly monitored for abnormal complete blood count profiles.

Pancitopenia y aplasia medular asociada a abatacept en una paciente con Artritis Reumatoide / Pancytopenia and medular aplasia associated to abatacept in a patient with rheumatoid arthritis

El tratamiento de pacientes con artritis reumatoide (AR) debe ser temprano y agresivo para prevenir el daño articular y la discapacidad. Los fármacos no biológicos modificadoes de enfermedad, como el metotrexato, han sido utilizados par controlar la actividad de la enfermedad y para prevenir el daño de las articulaciones. Existen pacientes con AR resistentes al tratamiento con fármacos modificadores de la enfermedad y otros que no responden adecuadamente a la terapia con inhibidores de factores de necrosis tumoral. Nosotros describimos el caso de una paciente de sexo femenino de 77 años que se presentó al servicio de emergencias con fiebre, mucositis y mal estado general luego de recibir una dosis de abatacept. A su ingreso el laboratorio demostró: glóbulos blancos 500 cel/mm3, neutrófilos 150 cel/mm3, plaquetas 21000 cel/mm3, hematocrito 29%, VCM 81, LDH 314 UI/L, función renal y hepatograma normales. En el examen clínico se objetivaron ulceras y lesiones ampollares en mucosa yugal. El medulograma evidenció hipocelularidad, con disminución de las tres series. El informe anatomopatológico fue de médula ósea hipoplásica. Recibió tratamiento con factor estimulante de colonias de neutrófilos, ácido fólico y metilprednisona, con resolución del cuadro a los 3 días de instituido el tratamiento. Hasta nuestro conocimiento esta es la primera comunicación de pancitopenia asociada a abatacept es una paciente con artritis AR intolerante a metotrexato

Treatment of patient with rheumatoid arthritis (RA) should be early and aggressive to prevent joint injury and disability. Disease-modifying antirheumatic drugs (DMARDs) like methotrexate has been used as initial treatment toward the disease activity and to prevento joint damage. Some patients with RA are resistant to initial therapy with nonbiiologic DMARDs or TNF inhibitiors. We described a 77 years old women who presented to the emergency room with fever andoral lesions after been treated with abatacept. On examination patient appeared ill. She had oral ulcers. laboratory testing showed white cells count 500 cells per mm3, hematocrit 29 %, platelets count 21000 cells per mm3, LDH 314 U/l. Renal and liver functions were normal. Bone marrow showed decreased in the three cells lineages. Patient was treated with granulocyte colony-stimulating factor, folic acid, and prednisone. Patient improved her physical and laboratory features three days after admission. This case showed the rare association between pancytopenia and abatacept in a patient with RA

Premières données nigériennes à propos des étiologies de la Pancytopénie : une étude sur 285 patients au service d'Onco-Hématologie de l'Hôpital National de Niamey

Introduction : La pancytopénie est définie comme étant une baisse associée des lignées érythrocytaire, granulocytaire et plaquettaire. L'origine est centrale ou périphérique. Cliniquement elle est caractérisée par une pâleur, de la fièvre et un syndrome hémorragique. C'est la première étude spécifique consacrée à ce sujet. Matériels et Méthodes: Il s'agit d'une étude portant sur 285 dossiers de patients sur 14 années. La pancytopénie était définie en présence des critères suivants : un taux d'Hb < 11 g/dl ; un taux de globules blancs (GB) < 4000/mmᶾ avec un taux de PNN < 1500 /mmᶾ ; un taux de plaquettes < 150 000/mmᶾ.Les paramètres étudiés avaient porté sur l'âge, le sexe, les signes cliniques (Pâleur, asthénie, fièvre, syndrome hémorragique), les résultats des examens paracliniques : hémogramme, myélogramme, biopsie ostéo-médullaire, goutte épaisse, sérologie VIH, hémocultures et échographie. Les critères de non inclusion étaient les cas de bicytopénie et les pancytopénie chimio-induite. Résultats : La fréquence des pancytopénies était de 20,5 cas/an; le sex-ratio: 0,8. La population était très jeune avec 70% de moins de 45 ans. Les principales manifestations cliniques étaient: la pâleur (271 cas : 95,1%), asthénie (253 cas: 88,8%), le syndrome tumoral (143 cas: 50,1%), la fièvre (133 cas: 46,7%), le syndrome hémorragique (48 cas: 17%). Les étiologies étaient dominées par les carences vitaminiques (87 cas : 30,5%), l'hypersplénisme (67 cas : 23,5%), les infections (45 cas : 15,8%), les hémopathies malignes (19 cas : 6,7%). Conclusion: Les étiologies des pancytopénies sont dominées par les carences vitaminiques et l'hypersplénisme. L'enquête étiologique doit être essentiellement axée sur ces 2 causes après avoir éliminé un syndrome infectieux et une leucémie aigüe qui sont souvent des urgences