Hadjadj Jérôme, Michel Marc, Chauveheid Marie-Paule, Godeau Bertrand, Papo Thomas, Sacre Karim
Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Paris, France; Département de Médecine Interne, Hôpital Bichat, Paris, France.
Eur J Haematol. 2014 Dec;93(6):521-6. doi: 10.1111/ejh.12393. Epub 2014 Jun 28.
Autoimmune disorders, including immune cytopenia, are encountered in the setting of chronic myelomonocytic leukemia (CMML). The aim of our study was to analyze the association of immune thrombocytopenia (ITP) with chronic myelomonocytic leukemia (CMML).
We carried out a retrospective cohort study on 565 patients with immune thrombocytopenia (ITP) followed in the French referral center for adult's immune cytopenia. A literature review using MEDLINE (National Library of Medicine, Bethesda, MD) was also performed.
Eight patients (5 male, 76.3 + 9.8 yr old) with ITP-associated CMML were identified in our national cohort. Thirteen cases were reported in literature from 1984 to 2013. Mean age was 65.3 ± 18.5 yr. Sex ratio (M/F) was 1/0.6. ITP unveiled CMML in all but four cases (17/21; 80.9%). ITP occurred in the setting of low-grade CMML in all cases, with neither reported progression nor acute myeloid leukemia transformation during follow-up. Overall, karyotype analysis revealed cytogenetic abnormalities in six cases (6/16; 37.5%). ITP had a chronic course in most cases and shares, according to the low level of bleeding complications and the high response rate to treatment such as corticosteroids and splenectomy, the usual characteristics of primary ITP.
Although the association of a well-defined ITP and CMML is rare, our study suggests that CMML-associated ITP should be treated according to current guidelines for primary ITP.
自身免疫性疾病，包括免疫性血细胞减少症，可在慢性粒单核细胞白血病（CMML）的背景下出现。我们研究的目的是分析免疫性血小板减少症（ITP）与慢性粒单核细胞白血病（CMML）之间的关联。
我们对法国成人免疫性血细胞减少症转诊中心随访的565例免疫性血小板减少症（ITP）患者进行了一项回顾性队列研究。还使用MEDLINE（美国国立医学图书馆，马里兰州贝塞斯达）进行了文献综述。
在我们的全国队列中确定了8例ITP相关的CMML患者（5例男性，年龄76.3±9.8岁）。1984年至2013年的文献报道了13例病例。平均年龄为65.3±18.5岁。性别比（男/女）为1/0.6。除4例（17/21；80.9%）外，ITP在所有病例中均揭示了CMML。所有病例中ITP均发生在低级别CMML的背景下，随访期间均未报告病情进展或急性髓系白血病转化。总体而言，核型分析显示6例（6/16；37.5%）存在细胞遗传学异常。大多数情况下ITP病程呈慢性，根据出血并发症水平低以及对皮质类固醇和脾切除术等治疗的高反应率，具有原发性ITP的常见特征。
虽然明确的ITP与CMML的关联很少见，但我们的研究表明，CMML相关的ITP应根据当前原发性ITP的指南进行治疗。
