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1.
Arch. argent. pediatr ; 119(3): e242-e246, Junio 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1248200

ABSTRACT

La leucemia linfoblástica aguda (LLA) es la patología oncológica más frecuente en pediatría, y corresponde al 23% de las neoplasias en menores de 15 años. Alrededor del 20% de los pacientes con LLA presentan recaídas, en la mayoría de los casos, en la médula ósea. Las recaídas extramedulares son inusuales y las dos localizaciones más frecuentes son el sistema nervioso central (SNC) y los testículos. Cuando las recaídas ocurren en el SNC, suelen manifestarse con un síndrome meníngeo. El síndrome hipotalámico se define como la presencia de hiperfagia, obesidad y/o cambios en el estado de ánimo, y es una forma de presentación clínica inusual de las recaídas en el SNC y debe alertar al pediatra para mantener un alto índice de sospecha.Se describen cuatro casos que se presentaron con síndrome hipotalámico al momento de desarrollar una recaída de LLA en el SNC


Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood, corresponding to 23% of cancer in children younger than 15 years old. About 20% of ALL cases will relapse, commonly in the bone marrow. Extramedullar relapses are unusual, and the two most frequent locations are CNS and testicles. ALL relapses, when diagnosed in the CNS, frequently present with clinical features of a meningeal syndrome. The hypothalamic syndrome, consisting of hyperphagia, obesity and / or behavior disturbances, corresponds to an unusual presentation of relapses in this location and should alert pediatricians to suspect it.We describe 4 ALL cases of hypothalamic syndrome at the time of CNS relapse


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Lymphoma, B-Cell , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Recurrence , Fatal Outcome , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Hypothalamic Diseases/diagnosis
2.
Rev. colomb. gastroenterol ; 36(supl.1): 30-36, abr. 2021. tab, graf
Article in Spanish | LILACS | ID: biblio-1251543

ABSTRACT

Resumen La tuberculosis es una enfermedad de importancia en la salud pública a nivel mundial, con una alta incidencia a nivel del territorio colombiano. Principalmente, afecta el parénquima pulmonar; sin embargo, en un porcentaje elevado de casos se diagnostica en su forma extrapulmonar y el tracto gastrointestinal es uno de los sitios más frecuentes. Así mismo, la región ileocecal y el íleon terminal son las regiones con más predilección por la bacteria Mycobaterium tuberculosis. Las manifestaciones en esta rara presentación de la enfermedad están dadas por dolor abdominal y sensación de masa principalmente, lesiones ulcerosas en la mucosa intestinal y hallazgos histológicos correspondientes a granulomas caseificantes de gran tamaño y de morfología confluente, que se diferencian de otras entidades como la enfermedad de Crohn. La búsqueda de la enfermedad dentro del tracto gastrointestinal se realiza con ayuda de métodos invasivos como la colonoscopia y de ayudas diagnósticas de laboratorio como cultivos, tinciones o reacción en cadena de la polimerasa (PCR). Dada la complejidad en el diagnóstico de esta forma de tuberculosis, el conocimiento y la manera en que se aborda un paciente con un cuadro sugestivo de esta enfermedad son factores importantes para establecer el manejo terapéutico oportuno. Se comparte un caso inusual de tuberculosis ileocecal como manifestación de síndrome febril prolongado con desenlace fatal.


Abstract Tuberculosis is a disease of public health importance worldwide with a high incidence in Colombia. It mainly affects the lung parenchyma. However, in a large number of cases, it is diagnosed in its extrapulmonary form, with the gastrointestinal tract being one of the most frequent sites. Mycobacterium tuberculosis has a strong predilection for the ileocecal region and the terminal ileum. Manifestations of this rare form of the disease are abdominal pain and mass sensation mainly, as well as ulcerative lesions in the intestinal mucosa and histological findings corresponding to large caseating granulomas of confluent morphology, which distinguish it from other entities such as Crohn's disease. Invasive procedures, such as colonoscopy, and diagnostic laboratory aids, such as cultures, stains, and PCR, are used to find the disease in the gastrointestinal tract. Given the difficulty of diagnosing this type of tuberculosis, knowledge and how a patient with symptoms suggestive of the disease is approached are critical factors for establishing timely treatment. The following is an unusual case of ileocecal TB as a manifestation of prolonged febrile illness with a fatal outcome.


Subject(s)
Humans , Male , Aged , Tuberculosis, Gastrointestinal , Mycobacterium tuberculosis , Bacteria , Colonoscopy , Fatal Outcome , Laboratories
3.
Braz. j. med. biol. res ; 54(2): e10462, 2021. tab, graf
Article in English | LILACS | ID: biblio-1153510

ABSTRACT

Infections caused by uncommon and resistant pathogens in unusual sites have been increasingly reported in medical literature. We describe four cases of rare cytological findings and clinical impact for patients. In the first case, Aspergillus sp and Pneumocystis jirovecii were observed in the bronchoalveolar lavage of a patient with severe systemic lupus. In the second and third cases, we describe the presence of Trichomonas sp and Strongyloides sp larvae in samples of pleural and peritoneal fluid, respectively. The fourth report is about a patient with a wrist subcutaneous nodule whose synovial aspiration and cytology revealed the presence of brown septate hyphae. The early identification of the infectious agent in the cytological examination was essential for the introduction and/or re-adaptation of therapy in the four cases described. Patients in this report were immunocompromised with severe comorbidities, conditions often associated with unfavorable clinical outcomes.


Subject(s)
Humans , Animals , Male , Female , Middle Aged , Communicable Diseases/diagnosis , Cytodiagnosis/methods , Pleural Effusion/parasitology , Aspergillus/isolation & purification , Strongyloides/isolation & purification , Strongyloidiasis/diagnosis , Trichomonas/isolation & purification , Trichomonas Infections/diagnosis , Ascitic Fluid/parasitology , Bronchoalveolar Lavage Fluid/microbiology , Fatal Outcome , Pneumocystis carinii/isolation & purification
4.
Autops. Case Rep ; 11: e2020226, 2021. graf
Article in English | LILACS | ID: biblio-1142402

ABSTRACT

Uterine rupture during pregnancy is a known complication of placenta accreta. This paper presents a case of sudden maternal death in the 27th week of gestation due to a ruptured uterine scar at the site of placenta accreta with a short inter-pregnancy period of 6 months with previous two C-sections. Autopsy findings revealed a massive hemoperitoneum and a thinned out anterolateral uterine wall. Internal examination revealed clotted and fluid blood in the peritoneal cavity with rupture of the anterior uterine wall at the site of the placenta accreta in a healed cesarean section scar. Placenta accreta is a rare complication of pregnancy. However, it is becoming more frequent and a significant risk factor with the increasing rate of C-section.


Subject(s)
Humans , Female , Pregnancy , Adult , Placenta Accreta , Uterine Rupture , Cesarean Section , Maternal Death , Pregnancy Complications , Autopsy , Cicatrix , Fatal Outcome , Death, Sudden
6.
Autops. Case Rep ; 11: e2021269, 2021. graf
Article in English | LILACS | ID: biblio-1249004

ABSTRACT

Rheumatic heart disease is still common in developing countries and requires prompt intervention to prevent chronic complications. Vegetations in rheumatic heart disease might be due to acute episodes of rheumatic fever itself or due to either infective endocarditis (IE) or Non-infectious thrombotic endocarditis (NITE). Each form of vegetations has specific pathological characteristics on gross and microscopic examination. However, clinically IE and NITE may have overlapping signs and symptoms. A chance of misdiagnosis of NITE as culture-negative infective endocarditis is higher if the former present with infective symptoms like fever. NITE of valves can be due to underlying associated malignant neoplasm, particularly mucinous adenocarcinoma, pneumonia, cirrhosis, autoimmune disorders, and hypercoagulable state. The coexistence of tuberculosis, non-infectious thrombotic endocarditis and rheumatic valvular heart disease was rarely documented in medical literature. We describe a case of chronic rheumatic heart disease with vegetations in the posterior mitral valve leaflet, treated as culture-negative infective endocarditis, which, at autopsy, reveals the presence of Nonbacterial thrombotic endocarditis vegetation over calcified, fibrosed mitral valve leaflets and associated disseminated tuberculosis along with classic pathological sequela findings of chronic rheumatic mitral valvular heart disease in lungs and liver.


Subject(s)
Humans , Male , Adult , Rheumatic Heart Disease , Tuberculosis , Endocarditis, Non-Infective/pathology , Autopsy , Fatal Outcome , Diagnosis, Differential
7.
Autops. Case Rep ; 11: e2021256, 2021. tab, graf
Article in English | LILACS | ID: biblio-1153179

ABSTRACT

Introduction Acute liver failure (ALF) due to diffuse infiltrating solid malignancy without any focal lesions on radiographic imaging is rare. Case report A 70-year-old man was admitted due to mental confusion, abdominal pain, and ALF. Three years before, he had undergone a left nephrectomy for urothelial carcinoma followed by adjuvant chemotherapy. The abdominal computed tomography (CT) showed hepatomegaly and ascites. Ascitic fluid had transudate characteristics, with no malignant cells. Percutaneous liver biopsy (LB) showed diffuse liver infiltration of metastatic urothelial carcinoma. The patient rapidly deteriorated and died in a week due to ALF. Discussion History of solid cancer and hepatomegaly and/or liver failure without other obvious explanation should encourage to perform LB. Conclusion LB is warranted to avoid misdiagnosis, prolonged hospital stays, and delay in palliative care.


Subject(s)
Humans , Male , Aged , Urinary Bladder Neoplasms/pathology , Carcinoma , Liver Failure, Acute/pathology , Ascites , Autopsy , Biopsy , Fatal Outcome , Diagnostic Errors , Hepatomegaly
8.
Autops. Case Rep ; 11: e2020231, 2021. tab, graf
Article in English | LILACS | ID: biblio-1153176

ABSTRACT

Cardiac lymphoma is a rare entity. In this setting, the secondary involvement of the heart is far more frequent than the primary cardiac lymphoma. Herein, we present an autopsy case of a disseminated anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma with a dominant mediastinal involvement. Extensive cardiac infiltration with the near replacement of the myocardial wall by the neoplastic cells was observed. A total of nine isolated case reports of anaplastic large cell lymphoma with cardiac involvement were found in the English-language literature, and a widespread cardiac and thymic infiltration by the systemic ALK-positive anaplastic large cell lymphoma has not been documented. An incidental regenerative nodule was also identified in the liver. The patient died of pulmonary thromboembolism and cardiac arrest.


Subject(s)
Humans , Female , Adult , Lymphoma, Large-Cell, Anaplastic/pathology , Heart Neoplasms , Autopsy , Thromboembolism , Thymus Gland/pathology , Fatal Outcome , Anaplastic Lymphoma Kinase , Heart Arrest
9.
Autops. Case Rep ; 11: e2021258, 2021. graf
Article in English | LILACS | ID: biblio-1249031

ABSTRACT

Congenital medulloblastoma is a rare brain tumor that appears in less than 1% of pediatric patients. Congenital medulloblastoma has a poor prognosis and should be suspected in patients with clinical manifestations of hyporeactivity, slow suction reflexes, and the presence of hydrocephalus. Herein we present the case of a 12-day-old female newborn who developed non-communicative hydrocephalus, hyporeactivity, and hyporeflexia. Magnetic resonance imaging of her brain showed a heterogeneous and cystic mass on the posterior cranial fossa. A suboccipital craniotomy was performed. The histopathologic analysis reported a congenital medulloblastoma. She remained in hospital until her death at 112 days old. This is one of the first case reports with clinical-radiological and pathological documentation. Awareness of this diagnosis can allow prenatal intervention, rendering a better prognosis. This case report exemplifies the importance of good prenatal follow-up.


Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Cerebellar Neoplasms/congenital , Medulloblastoma/congenital , Prenatal Diagnosis , Fatal Outcome
11.
Autops. Case Rep ; 11: e2020201, 2021. tab, graf
Article in English | LILACS | ID: biblio-1142397

ABSTRACT

Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells (UCOGCs) is an extremely rare morphologically and clinically distinct variant of pancreatic ductal adenocarcinoma (PDAC), exhibiting a characteristic component of reactive osteoclast-like giant cells admixed with neoplastic mononuclear cells. Sommers and Meissner first described it in 1954 as an "unusual carcinoma of the pancreas". Later it acquired many different names. In 2010, the WHO classified these tumors as a variant of PDAC under the heading of "undifferentiated carcinoma with osteoclast-like giant cells". Here we describe the first case of pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) composed of UCOGC and pancreatic neuroendocrine tumor (NET), which occurred in a 78-year-old man with biliary colic and pancreatitis. The mass did not respond to the chemotherapy, and he soon developed liver metastasis from the NET component, and unfortunately, the patient passed away 10 months later. Since UCOGC is extremely rare, and its association with NET has not been reported yet, our case expands the knowledge regarding its unusual presentation and poor prognosis.


Subject(s)
Humans , Male , Aged , Pancreatic Neoplasms/pathology , Neuroendocrine Tumors , Carcinoma, Pancreatic Ductal/pathology , Giant Cells , Fatal Outcome
12.
Autops. Case Rep ; 11: e2021255, 2021. graf
Article in English | LILACS | ID: biblio-1153189

ABSTRACT

Undifferentiated or anaplastic thyroid carcinoma (ATC) is rare and one of the most aggressive human malignancies. The tumor is usually voluminous and fast-growing and mostly affects older women. The most common sites of distant metastases are the lungs, brain, and bones. Herein, we describe the case of a 66-year-old woman with a history of bilateral breast carcinoma and ATC, who presented with an acute abdomen and subsequently died. At autopsy, an isolated metastasis of ATC in the small intestine leading to bowel perforation was found. Moreover, there was adenocarcinoma in the descending colon. The review of extra-abdominal malignancies metastasizing to bowel and coincidence of breast and thyroid carcinoma is included.


Subject(s)
Humans , Female , Aged , Breast Neoplasms , Thyroid Carcinoma, Anaplastic , Intestinal Perforation/etiology , Neoplasm Metastasis , Autopsy , Fatal Outcome , Intestine, Small/injuries
13.
Autops. Case Rep ; 11: e2021246, 2021. graf
Article in English | LILACS | ID: biblio-1153187

ABSTRACT

Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin's lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.


Subject(s)
Humans , Male , Adult , Nose Neoplasms/pathology , Lymphoma, Extranodal NK-T-Cell/pathology , Nasal Cavity/pathology , Autopsy , Lymphoma, T-Cell , Fatal Outcome , Herpesvirus 4, Human , Disease Progression , Heart , Lung/pathology
14.
Rev. bras. neurol ; 56(3): 21-24, jul.-set. 2020. ilus
Article in Portuguese | LILACS | ID: biblio-1120507

ABSTRACT

O infarto da artéria de Percheron é uma apresentação rara de acidente vascular cerebral, caracterizado principalmente por isquemia talâmica bilateral. A apresentação clinica desse infarto se apresenta de maneira inesperada e variável. Relata-se um caso de um paciente masculino, idoso, acometido por diversas comorbidades, admitido na emergência em coma e hemiplégico a direita, demonstrando acometimento neurológico. A condição clínica do paciente variou durante a hospitalização, apresentando melhora do quadro neurológico focal e midríase fixa à direita, levando a um diagnóstico tardio. Paciente evoluiu ao óbito devido a causas não neurológicas.


The artery of Percheron infarct is a rare presentation of stroke, featured mainly by thalamic bilateral ischemia. The clinical presentation of this infarct is unexpected and variable. It's reported a case of a male patient, elderly, affected with several comorbidities, admitted to the emergency in comatose state and right hemiplegic, proving neurological involvement. The patient's clinical condition has fluctuated throughout the hospitalization presenting improvement of the focal neurologic implication and right mydriasis, culminating in a lagged diagnosis. Patient's death due to non neurologic causes.


Subject(s)
Humans , Male , Aged , Thalamus/blood supply , Cerebral Infarction/complications , Cerebral Infarction/diagnostic imaging , Posterior Cerebral Artery/diagnostic imaging , Thalamus/diagnostic imaging , Tomography, X-Ray Computed , Comorbidity , Risk Factors , Fatal Outcome
15.
Rev. chil. pediatr ; 91(4): 584-590, ago. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1138675

ABSTRACT

INTRODUCCIÓN: El síndrome IPEX (inmunodesregulación, poliendocrinopatía y enteropatía autoinmune ligada a X) causado por mutaciones en el gen FOXP3, se caracteriza por diarrea prolongada, alteraciones endocrinológicas y dermatitis. El tratamiento consiste en la administración de medicamentos inmunosupresores, siendo el trasplante de médula ósea la única cura potencial. OBJETIVO: Describir una nueva mutación del gen FOXP3, así como los hallazgos y evolución de un paciente con síndrome IPEX. CASO CLÍNICO: Lactante menor masculino que debutó al mes de vida con diarrea cró nica, falla intestinal e infecciones recurrentes. Exámenes de laboratorio y biopsia intestinal sugerentes de enteropatía autoinmune. Durante el seguimiento, el paciente presentó refractariedad al manejo inmunosupresor con esteroides, ciclosporina y tacrolimus, falleciendo a los 7 meses de edad por complicaciones vasculares. Antecedente familiar por línea materna de múltiples muertes en hombres menores de 1 año. Ante la sospecha de síndrome IPEX se realizó exoma en trío que reportó una mutación probablemente patogénica en el gen FOXP3. CONCLUSIÓN: Se documentó una nueva mutación del gen FOXP3 en paciente con síndrome IPEX. A pesar de la baja prevalencia de esta enfermedad, es importante el reconocimiento de síntomas no específicos pero sugerentes del diagnóstico.


INTRODUCTION: The IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) syn drome is caused by the mutations of the FOXP3 gene, characterized by persistent diarrhea, endo crine disorders, and dermatitis. The treatment is the administration of immunosuppressive drugs, where hematopoietic stem cell transplantation is the only potential cure. OBJECTIVE: To describe a new FOXP3 gene mutation, as well as the findings and evolution of a patient with IPEX syndrome. CLINICAL CASE: Male infant presenting at one month of age with chronic diarrhea, intestinal failure, and recurrent infections. Lab tests and intestinal biopsy suggested autoimmune enteropathy. During follow-up, the patient presented resistance to immunosuppressive treatment with corticosteroids, cyclosporine, and tacrolimus, dying at 7 months of age due to vascular complications. He had a ma ternal family history of multiple deaths of men under 1 year of age. IPEX syndrome was suspected therefore a trio whole-exome sequencing was performed that showed a probably pathogenic FOXP3 gene mutation. CONCLUSION: A new FOXP3 gene mutation is reported in a patient with IPEX syndro me. Despite the low prevalence of this disease, it is important to recognize non-specific but suggestive symptoms for its diagnosis.


Subject(s)
Humans , Male , Infant , Genetic Diseases, X-Linked/diagnosis , Diabetes Mellitus, Type 1/congenital , Diarrhea/diagnosis , Forkhead Transcription Factors/genetics , Immune System Diseases/congenital , Pedigree , Genetic Markers , Chronic Disease , Fatal Outcome , Genetic Diseases, X-Linked/genetics , Diabetes Mellitus, Type 1/diagnosis , Diabetes Mellitus, Type 1/genetics , Diarrhea/genetics , Immune System Diseases/diagnosis , Immune System Diseases/genetics , Mutation
16.
Int. j. morphol ; 38(4): 1060-1064, Aug. 2020. graf
Article in Spanish | LILACS | ID: biblio-1124897

ABSTRACT

La linfangiomatosis es una patología benigna, de etiología desconocida, que afecta especialmente a población pediátrica, caracterizándose por desarrollo de masas subcutáneas que involucran uno o más órganos. Cuando se asocia con coagulopatía y derrames quilosos, recibe el nombre de linfangiomatosis kaposiforme (LK), cuya evidencia disponible es escasa. El objetivo de este manuscrito es reportar un caso de LK pediátrico atendido en el Instituto del Cáncer SOLCA, Cuenca, Ecuador. Paciente femenina de 1 año 7 meses, con hernia inguinal bilaterales y alteración de los tiempos de coagulación. En los estudios de imagen se evidenció una masa mediastínica asociada a presencia de líquido en espacio pleural y cavidad abdomino-pélvica. Además, se evidenció la existencia de múltiples imágenes hipogénicas en el bazo. Se practicó timectomía mínima, toma de biopsia de linfonodos mediastínicos para establecer el diagnóstico, y se instaló un dren en el tórax para tratar el derrame pleural antes señalado. Se descartaron patologías similares (linfomas, lupus, inmunológicas, etc.). Dado el continúo deterioro respiratorio, coagulopatía, lesiones hipogénicas esplénicas y drenaje abundante del quilotórax por el tubo torácico, se planteó el diagnóstico de LK. Se inició apoyo nutricional vía parenteral (sin lípidos); se indicó vincristina, y se realizó una pleurodesis con bleomicina sin buena respuesta. La paciente falleció a los 16 días de su ingreso. Se trata de un caso de mal pronóstico, con evolución rápidamente progresiva hacia un desenlace fatal.


Lymphangiomatosis is a benign pathology, of unknown etiology. Affects especially to pediatric population and is characterized by development of subcutaneous masses that involve one or more organs. When it presents with coagulopathy and chylous effusions, it is called kaposiform lymphangiomatosis (KL). The available evidence of KL is scarce. The objective of this manuscript was to report a case of pediatric KL treated at SOLCA Cancer Institute, Cuenca, Ecuador. Female patient of 1 year and 7 months age. She had a bilateral inguinal hernia and altered coagulation times. Imaging studies revealed a mediastinal mass associated with fluid in the pleural space and in the abdominalpelvic cavity. Furthermore, the existence of multiple hypogenic images in the spleen was evidenced. Minimal thymectomy, biopsy of mediastinal lymph nodes was performed to establish the diagnosis, and a drain was installed in the chest to treat the aforementioned pleural effusion. Other pathologies like lymphomas, lupus, etc. were ruled out. Given the continuous respiratory deterioration, coagulopathy, splenic hypogenic lesions and abundant drainage of the chylothorax through the chest tube, the diagnosis of KL was raised. Parenteral nutrition (lipid-free) was started; Vincristine was indicated, and a pleurodesis with bleomycin was performed without a good response. The patient died 16 days after her admission. It is a case of very bad prognosis, with a rapidly progressive evolution towards a fatal outcome.


Subject(s)
Humans , Infant , Sarcoma, Kaposi/diagnostic imaging , Kasabach-Merritt Syndrome/diagnostic imaging , Hemangioendothelioma/diagnostic imaging , Radiography, Thoracic , Tomography, X-Ray Computed , Ultrasonography , Fatal Outcome
17.
Autops. Case Rep ; 10(2): e2020170, Apr.-June 2020. tab, graf
Article in English | LILACS | ID: biblio-1131812

ABSTRACT

Histiocytic sarcoma (HS) is a rare hematolymphoid malignant neoplasm with an aggressive clinical course. It can arise de novo or from low-grade B-cell lymphoma. We describe the case of a 16-year-old boy referred to our hospital with generalized lymphadenopathy, weight loss, and decreased appetite for one month. The patient died undiagnosed on the 7th day of hospitalization. Lymph node and bone marrow biopsies were performed one day before the patient died. The lymph node biopsy revealed an architectural effacement with a diffuse proliferation of large pleomorphic neoplastic cells containing large, multilobulated nuclei, coarse vesicular chromatin, prominent nucleoli, and a moderate amount of eosinophilic cytoplasm. The bone marrow aspiration smears and biopsy also showed evidence of infiltration by these above-mentioned cells. Based on the morphology, along with the exclusion of many differential diagnoses by an extensive panel of immunohistochemical markers, a diagnosis of HS was made. This case report aims at evaluating all the clinical and immunophenotypic features of a case of HS with multifocal presentation and an aggressive clinical course in order to give a correct and definite diagnosis at the proper time.


Subject(s)
Humans , Male , Adolescent , Histiocytic Sarcoma/pathology , Autopsy , Immunophenotyping , Lymphoma, B-Cell , Fatal Outcome , Diagnosis, Differential , Lymphadenopathy
18.
Autops. Case Rep ; 10(2): e2020155, Apr.-June 2020. graf
Article in English | LILACS | ID: biblio-1131815

ABSTRACT

Diaphragmatic eventration (DE) associated with intestinal malrotation and renal agenesis is a rare entity. The authors report a case of a 69-year-old man who had symptoms of heart failure. He had a previous imaging diagnosis of right diaphragmatic eventration and dilated cardiomyopathy. He died on the second day after the hospital admission and had a post mortem examination that confirmed complete right diaphragmatic eventration, intestinal malrotation, left renal agenesis, dilated cardiomyopathy, and anteriorly rotated right kidney and had findings suggestive of a thoracoabdominal compartment syndrome. Thoracoabdominal compartment syndrome is described as transmission of abdominal pressure through a defective diaphragm causing compression of the hemithorax viscera and mediastinal shift with a hemodynamic alteration. The association of these anomalies is rare, and the possibility of this finding in a patient with eventration should always be considered.


Subject(s)
Humans , Male , Aged , Compartment Syndromes/pathology , Diaphragmatic Eventration/pathology , Kidney/abnormalities , Autopsy , Cardiomyopathy, Dilated , Fatal Outcome
19.
Autops. Case Rep ; 10(2): e2020164, Apr.-June 2020. graf
Article in English | LILACS | ID: biblio-1131820

ABSTRACT

Emphysematous gastritis (EG) is a rare and potentially lethal process caused by invasive, gas-producing bacteria leading to inflammation and gas dissection of the stomach. The most common etiologic agents are Clostridium infections, but other organisms, including enterobacteria, staphylococcus, and fungi have also been identified. We report the first case of EG due to Sarcina ventriculi in a solid organ transplant recipient, who presented with epigastric pain and vomiting. The patient had a history of type 1 diabetes mellitus (DM) with recurrent episodes of ketoacidosis and systemic diabetic complications, including severe gastroparesis. CT scan studies demonstrated EG with venous air, and endoscopy showed severe gastritis and ulcerations. In the gastric biopsies, abundant Sarcina ventriculi were noted in areas of mucosal/submucosal necrosis. Antibiotic treatment was instituted at admission, and subsequent endoscopy demonstrated the disappearance of Sarcina, with some improvement of the gastric inflammation; however, the patient developed septic shock with multiorgan failure and expired. This case highlights the need to consider other infectious etiologies in transplant patients, in addition to the well-known opportunistic infections.


Subject(s)
Humans , Adult , Diabetes Complications , Transplant Recipients , Infections/etiology , Autopsy , Opportunistic Infections/etiology , Cholestasis , Clostridium Infections , Liver Failure , Fatal Outcome , Gastroparesis/complications , Renal Insufficiency/complications , Graft Rejection
20.
Autops. Case Rep ; 10(2): e2020175, Apr.-June 2020. graf
Article in English | LILACS | ID: biblio-1131805

ABSTRACT

Peripheral neuropathy is common, but rarely due to vasculitis. This report is the case of a 74-year-old woman with systemic vasculitis who presented with progressive arm and leg weakness associated with numbness. Autopsy revealed a colon cancer, which may have triggered the vasculitis. This case illustrates the association between vasculitis and malignancy. The best treatment of vasculitis in patients with cancer-associated vasculitis is usually treatment of the cancer, which often yields remission of the vasculitis. This case also illustrates the difficulty of suspecting vasculitis since the symptoms and signs are nonspecific and protean. It is important not to miss a diagnosis of vasculitis. It is often life-threatening. It is treatable. The critical step in the diagnosis of vasculitis is to think of it.


Subject(s)
Humans , Female , Aged , Colonic Neoplasms , Paraneoplastic Polyneuropathy , Systemic Vasculitis , Signs and Symptoms , Autopsy , Fatal Outcome , Diagnosis, Differential , Muscular Diseases
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