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Questions and Answers
What is chronic myelomonocytic leukemia (CMML)?
What is chronic myelomonocytic leukemia (CMML)?
A clonal hematopoietic stem cell disorder with overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms.
What percentage of CMML patients have an inherent risk for leukemic transformation over 3-5 years?
What percentage of CMML patients have an inherent risk for leukemic transformation over 3-5 years?
The 5th edition of WHO classification has reintroduced CMML-0 as a subcategory.
The 5th edition of WHO classification has reintroduced CMML-0 as a subcategory.
False
What are the two subtypes of CMML according to the revised WHO classification?
What are the two subtypes of CMML according to the revised WHO classification?
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What is the recommended white blood cell count for classifying a subtype as MP-CMML?
What is the recommended white blood cell count for classifying a subtype as MP-CMML?
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The diagnostic absolute monocyte count threshold for CMML was lowered to ≥______, according to new classification systems.
The diagnostic absolute monocyte count threshold for CMML was lowered to ≥______, according to new classification systems.
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Study Notes
Overview of Chronic Myelomonocytic Leukemia (CMML)
- CMML is a clonal hematopoietic stem cell disorder with features of both myelodysplastic syndromes and myeloproliferative neoplasms.
- It is characterized by significant monocytosis and has a 15%–20% risk of leukemic transformation over 3–5 years.
Classification Changes
- CMML has been classified as a unique myeloid neoplasm since the French-American-British (FAB) classification in 1982.
- The 4th edition of the WHO classification introduced two subtypes for CMML:
- Myeloproliferative CMML (MP-CMML)
- Myelodysplastic CMML (MD-CMML)
- MP-CMML is defined by a white blood cell count of ≥13 x 10^9/L.
Recent Developments in CMML Classification
- In 2022, two classification systems for myeloid neoplasms emerged:
- International Consensus Classification (ICC)
- 5th edition of the WHO classification
- Both systems revised the diagnostic absolute monocyte count (AMC) threshold from ≥1 x 10^9/L to ≥0.5 x 10^9/L.
- Patients with sustained AMC values between 0.5 and 1.0 x 10^9/L, previously known as Oligomonocytic CMML (O-CMML), show similar outcomes and phenotypic overlaps with CMML.
Revised Subcategories
- The 5th edition of the WHO classification and ICC eliminated CMML-0 as a subcategory due to insufficient risk stratification.
- Categorization of CMML is now based on peripheral blood (PB) and bone marrow (BM) blast percentage:
- CMML-1: Includes blasts and promonocytes.
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Description
This quiz delves into the exploration of Chronic Myelomonocytic Leukemia (CMML), a unique myeloid neoplasm that bridges myelodysplastic syndromes and myeloproliferative neoplasms. Key classification changes, recent developments, and clinical characteristics, including risk factors and diagnostic thresholds, will be addressed.