Acute Promyelocytic Leukemia in Children-Reports of Two Cases-

小兒急性前髓球性白血病－二病例報告－

劉漢宗(Han-Chung Lieu) ； 林正宗(Chen-Jzong Lin) ； 洪悠紀(Iou-Jih Hung)

《Acta Paediatrica Sinica》 27卷4期 (1986/08) Pp. 367-372

摘要

長庚紀念醫院小兒科，在3個月中診斷2例急性前髓球性白血病(Acute Promyelocytic Leukemia APL)，此症是急性非淋巴球性白血病(ANLL)中最惡性的一種，它主要出現在年輕的成年人，平均年齡約40多歲，約佔成人ANLL的10%，然而它可出現在任何年齡，甚至連3個月大的病例亦被報告過。小孩的APL是相當少的，根據追踪性分析，APL在ANLL中的出現率約佔4%。此二白血病例皆具嚴重出血現象，且血凝檢查均顯示散播性血管內凝集(Disseminated Intravascular Coagulation DIC)現象。第1例之細胞型態學診斷為典型的多顆粒性前髓球性白血病hypergranular APL（FAB分類M3）。第2例則為微顆粒性前髓球性白血病microgranular APL（FAB分類M3m）此型白血病甫自M3中被分敍出來，因其細胞核類似單核細胞系列，且細胞內顆粒僅能用電子顯徵鏡識別，其細胞生化及細胞基因檢查則證實其為APL M3系列。此2例雖然及時予輔助治療（多次血小板，新鮮冷凍血漿，肝素）及化學治療，不幸皆因，內出血而死亡。APL為血液科之急症，患者一經診斷卽需於48小時內開始治療，根據國外文獻報告，肝素可改進完全緩解率(complete remission rete)而Anthracycline 則是最好之引導治療劑之一，以這藥治療之病人其完全緩解率可達58~80%，平均緩解期可長達26個月。本院之2病例，不論臨床症狀，實驗檢驗結果，治療方法均屬典型，因其並不多見，故作文獻探討以供大家參考。

並列摘要

Two children with acute promyelocytic leukemia (APL) were admitted to the Department of Pediatrics, Chang Gung Memorial Hospital during a three-month period. Both patients showed the clinical syndrome of acute nonlymphoblastic leukemia and severe bleeding diathesis. Their coagulation parameters showed evidence of disseminated intravascular coagulation. The cytomorphological features were characterized as typical hypergranular APL (E4B classification M3) in Patient one and microgranular APL (FAB classification M3m) in Patient two. Both were treated within 48 hours after admission with heparin, platelet concentrate, fresh frozen Plasma, antibiotics and chemotherapy. Their courses were fulminant and each patient died of intracranial hemorrhage. Rapid demise was the main characteristic. These two cases are described herein, and the literature is reviewed.

並列關鍵字

childhood acute promyelocytic leukemia ； FAB classification ； disseminated intravascular coagulation