RESUMO
The hypereosinophilic syndrome (HES) is a rare yet frequently fatal disorder of unknown etiology characterized by markedly elevated eosinophil counts and subsequent multiple organ failure due presumably to eosinophil-derived protein toxicity. We describe the laboratory and anatomic findings in a 15-year-old female with extraordinarily high circulating levels of eosinophil major basic protein (MBP) who sustained a precipitous cardiac death secondary to a massive myocardial infarction. Postmortem examination showed marked cardiomegaly with extensive recent left ventricular infarction. Occlusive thrombosis of small blood vessels was evident in the myocardium, spleen, lungs, and kidneys. Immunofluorescent staining showed massive MBP deposition in multiple organ parenchyma including the heart, renal glomeruli, adrenal cortex, bronchioles, and other visceral organs, suggesting a causal relationship. We hypothesize on the mechanisms of eosinophil toxicity in HES.
Assuntos
Síndrome Hipereosinofílica/complicações , Síndrome Hipereosinofílica/patologia , Infarto do Miocárdio/complicações , Ribonucleases , Adolescente , Proteínas Sanguíneas/metabolismo , Encéfalo/patologia , Proteínas Granulares de Eosinófilos , Evolução Fatal , Feminino , Humanos , Síndrome Hipereosinofílica/sangue , Fígado/patologia , Pulmão/patologia , Imageamento por Ressonância Magnética , Insuficiência de Múltiplos Órgãos/complicações , Insuficiência de Múltiplos Órgãos/patologia , Infarto do Miocárdio/patologia , Miocárdio/patologia , Baço/patologiaAssuntos
4-Hidroxicumarinas/intoxicação , Rodenticidas/intoxicação , 4-Hidroxicumarinas/sangue , Dor Abdominal/etiologia , Adulto , Equimose/etiologia , Epistaxe/etiologia , Feminino , Hematemese/etiologia , Hematoma/etiologia , Hematúria/etiologia , Humanos , Masculino , Saúde Pública , Problemas SociaisRESUMO
Numerous toxic exposures have been implicated in causing aplastic anemia. Thirteen cases of aplastic anemia and 5 cases of other blood dyscrasias, eg, red blood cell aplasia and thrombocytopenia, associated with lindane, have been reported in the literature. However, aplastic anemia secondary to the scabicidal product (lindane [Kwell]) has not been documented, to our knowledge. We present the case of a 21-year-old man with a diagnosis of aplastic anemia, known prolonged exposure to lindane, and documented elevated serum lindane levels. His clinical course is described as well as various defects are explored for the aplasia.
Assuntos
Anemia Aplástica/induzido quimicamente , Hexaclorocicloexano/efeitos adversos , Escabiose/tratamento farmacológico , Administração Tópica , Adulto , Hematopoese/efeitos dos fármacos , Hexaclorocicloexano/administração & dosagem , Hexaclorocicloexano/uso terapêutico , Humanos , Masculino , Subpopulações de Linfócitos T/efeitos dos fármacosRESUMO
4 men with an unusual variant of T-cell chronic lymphocytic leukaemia are reported. The clinical features differed from the virus-associated T-cell disease reported from Japan, the Caribbean, and the southeastern United States. Cytology was pleiomorphic: cells with cerebriform nuclei resembling Sézary cells and lymphocytes with cytoplasmic granules resembling T-suppressor cells occurred in the same patients. Immunofluorescence studies with monoclonal antibodies suggested that the leukaemia cells expressed determinants of both helper- (OKT4+) and suppressor-(OKT8+) related antigens. The cells were TdT-negative. In all patients the disease was very refractory to conventional cytotoxic agents, but there was prompt and extensive response to the adenosine deaminase inhibitor pentostatin (2'-deoxycoformycin). This agent merits further study in the treatment of T-cell chronic lymphocytic leukaemia.
Assuntos
Antineoplásicos/uso terapêutico , Coformicina/uso terapêutico , Leucemia Linfoide/imunologia , Ribonucleosídeos/uso terapêutico , Linfócitos T/imunologia , Adulto , Anticorpos Monoclonais , Coformicina/análogos & derivados , Epitopos/imunologia , Humanos , Leucemia Linfoide/classificação , Leucemia Linfoide/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pentostatina , Fenótipo , Linfócitos T Auxiliares-Indutores/imunologia , Linfócitos T Reguladores/imunologiaRESUMO
A 25-year-old man developed acute lymphoblastic leukemia, morphologically of Burkitt's type (L3-ALL, F.A.B. classification) but with immunologic and cytogenetic features not previously reported. The leukemic blasts were B1+, CALLA+, OKT3-, and OKT11-. Surface immunoglobulin and cytoplasmic IgM were not detected, but cytoplasmic IgG lambda was present. Karyotypic analysis of 20 metaphases was normal at presentation but abnormal after relapse. At that time, the predominant karyotype was 47XY, 1q-, 7q-, 12p-, M1. This case illustrates the following: (1) Burkitt cell morphology may accompany some uncommon pre-B-cell lymphoblastic leukemias, and (2) rearrangements involving chromosomes 14, 2 or 22 may not be found in all cases of L-3 ALL.
Assuntos
Linfoma de Burkitt/patologia , Leucemia Linfoide/genética , Receptores de Antígenos de Linfócitos B/análise , Adulto , Cromossomos Humanos 13-15 , Cromossomos Humanos 6-12 e X , Variação Genética , Humanos , Masculino , Translocação GenéticaRESUMO
Thrombocytopenia and hemolytic anemia with fragmented RBCs were discovered in a 70-year-old woman with a large mediastinal mass. Investigation of the cause of the hematologic findings was negative. The mass was surgically excised and proved to be a thymoma of spindle cell type. The hemogram findings returned to normal within a month of surgery. We postulate the thymoma as the source of the RBC fragmentation and thrombocytopenia and explore the relationship between tumors and the hematologic findings.
