Kuroda Junya, Kimura Shinya, Kobayashi Yutaka, Wada Katsuya, Uoshima Nobuhiko, Yoshikawa Toshikazu
First Department of Internal Medicine, Kyoto Prefectural University of Medicine, Japan.
Acta Haematol. 2002;108(3):139-43. doi: 10.1159/000064703.
Idiopathic thrombocytopenic purpura (ITP) and primary myelodysplastic syndrome (MDS) are hematological disorders that are frequently associated with thrombocytopenia, and both are heterogeneous disorders of uncertain etiology. Their diagnosis requires the exclusion of other hematological or immunological disorders whose diagnosis is usually not difficult. However, in some patients presenting with thrombocytopenia, the differential diagnosis is complex. We performed a retrospective study of 47 consecutive patients treated between 1990 and 2001; in 25 patients the initial diagnosis was ITP, in 22 it was MDS; we compared their backgrounds, laboratory data and clinical outcomes. Among the 25 ITP patients, there were 5 confusing cases. Following treatment, they presented with inexplicable refractory anemia and/or neutropenia. Cytopenia, the polyploidization pattern of megakaryocytes, and chromosomal aberrations were of diagnostic relevance in these patients' defective hematopoiesis. Their cytopenia progressed relatively slowly and none progressed to leukemic transformation. We suggest that these 5 patients should be classified into an unusual subtype of MDS with clinical characteristics resembling ITP.
特发性血小板减少性紫癜（ITP）和原发性骨髓增生异常综合征（MDS）是常与血小板减少相关的血液系统疾病，二者均为病因不明的异质性疾病。它们的诊断需要排除其他血液系统或免疫系统疾病，而这些疾病的诊断通常并不困难。然而，在一些出现血小板减少的患者中，鉴别诊断较为复杂。我们对1990年至2001年间连续治疗的47例患者进行了回顾性研究；其中25例患者最初诊断为ITP，22例为MDS；我们比较了他们的背景、实验室数据和临床结局。在25例ITP患者中，有5例情况令人困惑。治疗后，他们出现了无法解释的难治性贫血和/或中性粒细胞减少。血细胞减少、巨核细胞的多倍体化模式以及染色体畸变与这些患者造血功能缺陷的诊断相关。他们的血细胞减少进展相对缓慢，且无一例进展为白血病转化。我们建议将这5例患者归类为具有类似ITP临床特征的MDS不寻常亚型。
