title: Immune thrombocytopenia in chronic myelomonocytic leukemia.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Paris, France; Département de Médecine Interne, Hôpital Bichat, Paris, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Hadjadj
authors.forename: Marc
authors.initials: M
authors.lastname: Michel
authors.forename: Marie-Paule
authors.initials: MP
authors.lastname: Chauveheid
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.forename: Karim
authors.initials: K
authors.lastname: Sacre
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
keywords: chronic myelomonocytic leukemia; immune thrombocytopenia; review