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Chronic myelomonocytic leukemia — Clinical Guidelines

Overview

Chronic myelomonocytic leukemia (CMML) is a heterogeneous hematological disorder characterized by abnormal proliferation of monocytes and dysplastic changes in other myeloid cells, often presenting with features of both myelodysplastic syndromes and myeloproliferative neoplasms 1.

Diagnosis

Bone marrow biopsy showing monocytosis (≥500/μL) with dysplastic changes 1

Exclusion of other myeloid neoplasms through cytogenetic and molecular testing 1

Touch imprints from skin biopsies can aid in rapid diagnosis 8

Management

First-line treatments: Hydroxyurea for cytoreduction 2

Adjunctive treatments: Allogeneic hematopoietic cell transplantation (allo-HCT) considered for younger, fit patients with suitable donors 1 3

Supportive care: Management of symptoms like splenomegaly with analgesics (e.g., morphine) requiring caution due to risk of opioid toxicity 2

Special Populations

Elderly and comorbidities: Allo-HCT decision complex due to higher risks of complications; careful assessment of comorbidities and age is crucial 1 3

Comorbidities: Higher HCT Comorbidity Index correlates with increased mortality post-transplant 3

Key Recommendations

Allogeneic hematopoietic cell transplantation should be considered primarily for younger, fit patients with CMML and suitable donors, taking into account cytogenetic risk and comorbidities (Evidence: Moderate 1 3)

Prescribe opioids cautiously in CMML patients undergoing chemotherapy due to the risk of overlapping tumour lysis syndrome and opioid toxicity (Evidence: Weak 2)

Evaluate progression-free survival and relapse risk using prognostic factors such as karyotype and HCT Comorbidity Index before proceeding with allo-HCT (Evidence: Moderate 3)

References

1 Onida F, Gagelmann N, Chalandon Y, Kobbe G, Robin M, Symeonidis A et al.. Management of adult patients with CMML undergoing allo-HCT: recommendations from the EBMT PH&G Committee. Blood 2024. link 2 Vig S, Mishra S, Rustagi K, Bhan S. Opioid toxicity with underlying tumour lysis syndrome in a patient with CMML: a diagnostic and therapeutic challenge. BMJ case reports 2018. link 3 Eissa H, Gooley TA, Sorror ML, Nguyen F, Scott BL, Doney K et al.. Allogeneic hematopoietic cell transplantation for chronic myelomonocytic leukemia: relapse-free survival is determined by karyotype and comorbidities. Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation 2011. link 4 Rashid R, Hymes S. Folliculitis, follicular mucinosis, and papular mucinosis as a presentation of chronic myelomonocytic leukemia. Dermatology online journal 2009. link 5 Yamauchi K, Omata T. Leukemic pneumonitis as a poor prognostic factor in chronic myelomonocytic leukemia. Respiration; international review of thoracic diseases 1992. link 6 Manoharan A. Malignant pleural effusion in chronic myelomonocytic leukaemia. Thorax 1991. link 7 Dreno B, Gandon P, Bureau B, Milpied N, Barrière H. Skin lesions from hypersensitivity to cold during chronic myelomonocytic leukaemia. The British journal of dermatology 1986. link 8 Kenney RL, Reddy VV, Giri J, Adelman S. Chronic myelomonocytic leukemia: value of touch preps. The Journal of dermatologic surgery and oncology 1984. link

Chronic myelomonocytic leukemia