Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto 602-8566, Japan.
Int J Hematol. 2010 Apr;91(3):516-21. doi: 10.1007/s12185-010-0502-3. Epub 2010 Feb 11.
A 72-year-old male patient was initially diagnosed with essential thrombocythemia (ET), a Philadelphia chromosome-negative (Ph1(-)) chronic myeloproliferative disorder (CMPD), and was treated with hydroxyurea (HU). After 9 years of diagnosis of ET, his peripheral leukocytes gradually increased, while his platelet count showed a decrease. Bone marrow analysis disclosed Ph-positive chronic myelogenous leukemia (CML) in the chronic phase. Administration of imatinib mesylate (IM), a Bcr-Abl tyrosine kinase inhibitor (TKI), induced complete hematologic response in a month, but was discontinued after 4 months because of Grade 3 pleural effusion (PE). The treatment was switched to nilotinib which successfully induced a complete cytogenetic response (CCyR) after 5 months of TKI therapy and resolved the PE. Despite CCyR, however, ET recurred. Since then, the patient has been treated for 8 months with a combination of nilotinib and HU which has successfully controlled both CML and ET. This report includes a review of the characteristics of 15 reported cases with co-occurrence of CML and Bcr-Abl-negative CMPDs, including ours. Although rare, care needs to be taken since, despite the often similar clinical features of the two diseases, they require completely different treatments.
一位 72 岁男性患者最初被诊断为原发性血小板增多症（ET），一种费城染色体阴性（Ph1(-)）的慢性骨髓增生性疾病（CMPD），并接受羟基脲（HU）治疗。在 ET 确诊 9 年后，他的外周白细胞逐渐增加，而血小板计数则下降。骨髓分析显示 Ph 阳性慢性粒细胞白血病（CML）处于慢性期。甲磺酸伊马替尼（IM）的治疗，一种 Bcr-Abl 酪氨酸激酶抑制剂（TKI），在一个月内诱导完全血液学反应，但由于 3 级胸腔积液（PE）在 4 个月后停止。治疗方案改为尼洛替尼，在 TKI 治疗 5 个月后成功诱导完全细胞遗传学反应（CCyR）并解决了 PE。然而，尽管达到 CCyR，ET 仍复发。此后，该患者接受尼洛替尼和 HU 的联合治疗 8 个月，成功控制了 CML 和 ET。本报告包括对 15 例报告病例的特征的回顾，包括我们的病例，这些病例同时发生 CML 和 Bcr-Abl 阴性 CMPD。尽管罕见，但需要注意，因为尽管这两种疾病的临床特征常常相似，但它们需要完全不同的治疗方法。
