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Immune thrombocytopenia in chronic myelomonocytic leukemia
Jérôme Hadjadj1, Marc Michel, Marie-Paule Chauveheid
1Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Paris, France; Département de Médecine Interne, Hôpital Bichat, Paris, France.
Insights
This study found that immune thrombocytopenia (ITP) associated with chronic myelomonocytic leukemia (CMML) is rare but shares characteristics with primary ITP. Treatment for CMML-associated ITP should follow standard primary ITP guidelines.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Autoimmune disorders, including immune cytopenia, are frequently observed in patients with chronic myelomonocytic leukemia (CMML).
- Immune thrombocytopenia (ITP) is a specific type of immune cytopenia characterized by low platelet counts due to autoimmune destruction.
- The association between ITP and CMML requires further investigation to understand its clinical implications.
Observation:
- A retrospective cohort study identified eight patients with ITP-associated CMML in a French referral center.
- A literature review identified thirteen additional cases of ITP-associated CMML reported between 1984 and 2013.
- In most cases (80.9%), ITP preceded the diagnosis of CMML.
Findings:
- ITP associated with CMML typically presents as low-grade disease without progression to acute myeloid leukemia.
- Cytogenetic abnormalities were observed in 37.5% of analyzed cases.
- CMML-associated ITP generally follows a chronic course with a good response to treatments like corticosteroids and splenectomy, similar to primary ITP.
Implications:
- The study suggests that while rare, the co-occurrence of ITP and CMML is clinically significant.
- Current treatment guidelines for primary ITP are recommended for managing CMML-associated ITP.
- Further research may elucidate the underlying mechanisms linking ITP and CMML.
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.
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