RESUMO
Neoplastic plasma cell involvement of thyroid is an uncommon condition, and it may involve thyroid in multiple myeloma or solitary plasmacytoma. Its clinical and pathological features are not well understood. We present a rare case of synchronous thyroid involvement in plasma cell leukemia presenting as thyroid nodule with primary hypothyroidism. Simultaneous cytology, immunocytochemistry, FISH analysis with 13q14.3 and TP53 on cytology smears and haematology workup were performed to diagnose and to understand disease pathobiology with poor outcome.
Assuntos
Doença de Hashimoto/diagnóstico , Leucemia Plasmocitária/diagnóstico , Plasmocitoma/diagnóstico , Nódulo da Glândula Tireoide/diagnóstico , Citodiagnóstico , Feminino , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Pessoa de Meia-IdadeRESUMO
Hemophagocytic lymphohistiocytosis (HLH) is an immune disorder characterized by uncontrolled inflammation due to defective immune response. It may be familial or acquired, but both share a common feature of threatening the life of a patient and may lead to death unless treated by appropriate treatment. Here in we report a case of adult HLH.
RESUMO
Multiply transfused patients of severe aplastic anemia are at increased risk of graft rejection. Five such patients underwent peripheral blood stem cell transplantation from HLA-identical siblings with a fludarabine-based protocol. The conditioning consisted of fludarabine 30 mg/m(2)/day x 6 days, cyclophosphamide 60 mg/kg/day x 2 days and horse antithymocyte globulin (ATG) x 4 days. Two different ATG preparations were used: ATGAM (dose 30 mg/kg/day x 4 days) or Thymogam (dose 40 mg/kg/day x 4 days). Engraftment: median time to absolute neutrophil count (ANC) >0.5 x 10(9)/l was 11 days (range: 8-17) and median time to platelet count >20 x 10(9)/l was 11 days (range: 9-17). At a median follow-up of 171 days (range: 47-389), there has been no graft rejection and all patients are in complete remission. Acute GVHD (grade 1) occurred in one patient only. Chronic GVHD developed in two patients (extensive in one and limited in another). The transplants were performed in non-HEPA filter rooms. In only one patient, systemic antifungal therapy (voriconazole) was used. The use of Thymogam brand of ATG for conditioning is being reported for the first time. Our experience suggests that this fludarabine-based protocol allows rapid sustained engraftment in high-risk patients without significant immediate toxicity.