Overview of Chronic Myelomonocytic Leukemia
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Questions and Answers

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?

  • 20%–25%
  • 10%–15%
  • 5%–10%
  • 15%–20% (correct)
  • 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?

    <p>Myeloproliferative CMML (MP-CMML) and myelodysplastic CMML (MD-CMML).</p> Signup and view all the answers

    What is the recommended white blood cell count for classifying a subtype as MP-CMML?

    <p>≥13 x 10^9/L</p> Signup and view all the answers

    The diagnostic absolute monocyte count threshold for CMML was lowered to ≥______, according to new classification systems.

    <p>0.5 x 10^9/L</p> Signup and view all the answers

    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.

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