Kim J, Park C J, Seo E J, Lee J H, Yoo S J, Choi S J, Chi H S
Department of Clinical Pathology, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.
Ann Hematol. 2002 Oct;81(10):603-4. doi: 10.1007/s00277-002-0541-9. Epub 2002 Oct 17.
We report a first case of biphenotypic blast crisis of unclassified myeloproliferative disorder (MPD). A 20-year-old patient presented with fever, splenomegaly, marked leukocytosis (603 x 10(3)/ micro l), and blasts in the peripheral blood. Since Ph chromosome and bcr-abl gene rearrangement were absent, the diagnosis of an unclassified MPD in the blast crisis phase was established. Immunophenotyping confirmed a biphenotypic crisis of myeloid and T-lymphoid antigens. The patient went into a complete remission after chemotherapy, but marked granulocytic hyperplasia (M:E ratio of 5.7) and 90% cellularity remained. Blast crisis recurred during subsequent intensification chemotherapy and the patient did not go into a complete remission regardless of the intense chemotherapy. The blast crisis transformed from unclassified MPD had a grave prognosis as it responded poorly to chemotherapy. This unique blast crisis is distinguishable from the blast crisis of chronic myelogenous leukemia.
我们报告了首例未分类骨髓增殖性疾病（MPD）的双表型原始细胞危象病例。一名20岁患者出现发热、脾肿大、显著白细胞增多（603×10³/μl）以及外周血原始细胞。由于缺乏Ph染色体和bcr-abl基因重排，确诊为处于原始细胞危象期的未分类MPD。免疫表型分析证实为髓系和T淋巴细胞抗原的双表型危象。患者化疗后完全缓解，但仍存在显著的粒细胞增生（M:E比值为5.7）且细胞成分占90%。在随后的强化化疗期间原始细胞危象复发，无论化疗强度如何，患者均未完全缓解。由未分类MPD转变而来的原始细胞危象预后严重，因为其对化疗反应不佳。这种独特的原始细胞危象有别于慢性粒细胞白血病的原始细胞危象。
