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Acute Megakaryoblastic Leukemia - A Case Report

急性巨核母細胞性白血病─病例報告

摘要


急性巨核母細胞性白血病(acute megakaryoblastic leukemia)為一種少見且不易診斷之急性白血病,通常診斷需要靠電子顯微鏡檢查血小板過氧化酶(platelet peroxidase)反應或以單株抗體測定細胞上第八因子有關之抗原作為依據。本報告為─41歲女性,臨床上呈現貧血、心悸等症狀,實驗室檢查顯示貧血以及血小板數目極度增高,周邊血液抹片檢查可見22%不成熟母細胞、骨髓穿刺及切山檢查亦顯示未分化母細胞、小型巨核球(micromegakaryocyte)以及非典型巨核球瀰漫性增生。骨髓細胞抹片,未分化母細胞佔47%,細胞染色包括PAS, peroxidase, SBB, NASD, NSE皆呈陰性。使用單株抗體(Dako, anti-factor VIII)與免疫過氧化酶(immunoperoxidase)酵素法作骨髓切片染色,從巨核母細胞至成熟巨核球系列可見呈現棕色陽性反應,因此診斷為急性巨核母細胞白血病。此病人經low-dose Ara-c化學治療,不幸於十天後因全血球減少而死於感染症。

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並列摘要


Acute megakaryoblastic leukemia is an uncommonly recognized disorder, usually diagnosed by using electron microscopy to detect platelet peroxidase or with monoclonal antibody to localize the factor VIII related antigen. We described a 41-year-old female patient with acute megakaryoblastic leukemia identified by localizing the factor VIII related antigen in megakaryoblastic with immunoperoxidase technique. Clinically she presented symptoms of anemia and markedly elevated platelet count. 47% unclassified agranular blasts were seen on a bone marrow asiration smear. Bone marrow biopsy showed diffuse proliferation of blasts, micromegakaryocytes and atypical megakaryocytes. The cytochemical staining, included periodic-acid shiff (PAS), Sudan-Black B (SBB), peroxidase, non-specific esterase (NSE) and naphthol ASD chloroacetate (NASD), all appeared negative on the blast cells. Using monoclonal antibody (DAKO, anti-factor VIII), various stages of maturation in megakaryocytic series were identified. She was treated with low-dose Ara-C, but died due to an infection in a pancytopenic state ten days after chemotherapy.

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