Immune thrombocytopenia in chronic myelomonocytic leukemia

Objective 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). Methods 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. Results 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. Conclusion 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.