Sherazi Syed Furqan Haider, Butt Zeeshan
J Pak Med Assoc. 2012 Sep;62(9):989-90.
AML-M6 has a peak incidence in the seventh decade with slight male preponderance, and can also present at a younger age. The usual features are anaemia, thrombocytopenia, malaise, fatigue, easy bruising, epistaxis and petechiae. Splenomegaly may occur in 20-40 % of the cases but massive splenomegaly is rare presentation and have been only reported once in humans and once in animals. A 22 year Asian female, presented with fatigue, pallor, mild jaundice, exertional dyspnoea, epigastric pain, tender right hypochondrium and massive splenomegaly. Investigations revealed anaemia and thrombocytopenia, tear drop cells, basophilic stippling, piokilocytosis and anisochromia; increased uric acid and LDH. Abdominal ultrasound showed enlarged liver (22cm) and spleen (20cm). Bone marrow aspiration revealed 51% erythroid and 24% non-erythroid precursors, depressed leukopoeisis and megakarypoeisis. Erythroblasts were PAS and CD71 positive and also reacted to Antihaemoglobin-Antibody. This report highlights characteristic features and diagnostic criteria of erythroleukaemia, differential diagnosis of massive splenomegaly and their rare association.
急性髓系白血病M6型在70岁时发病率达到高峰，男性略占优势，也可在较年轻时出现。常见症状为贫血、血小板减少、不适、疲劳、易瘀伤、鼻出血和瘀点。20%-40%的病例可能出现脾肿大，但巨大脾肿大很少见，仅在人类和动物中各有一次报道。一名22岁的亚洲女性，表现为疲劳、面色苍白、轻度黄疸、劳力性呼吸困难、上腹部疼痛、右季肋部压痛和巨大脾肿大。检查发现贫血和血小板减少、泪滴状细胞、嗜碱性点彩、异形红细胞症和色素不均；尿酸和乳酸脱氢酶升高。腹部超声显示肝脏肿大（22cm）和脾脏肿大（20cm）。骨髓穿刺显示51%为红系前体细胞，24%为非红系前体细胞，白细胞生成和巨核细胞生成受抑制。成红细胞PAS和CD71阳性，也与抗血红蛋白抗体反应。本报告强调了红白血病的特征性表现和诊断标准、巨大脾肿大的鉴别诊断及其罕见关联。
