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Atypical chronic myeloid leukemia, BCR-ABL1 negative(ACML)

MedGen UID:
266233
Concept ID:
C1292772
Neoplastic Process
Synonyms: ACML; Atypical chronic myeloid leukemia
SNOMED CT: Atypical chronic myeloid leukemia, Philadelphia chromosome (Ph1) negative (128826001); Atypical chronic myeloid leukemia, BCR/ABL negative (128826001); Atypical chronic myeloid leukemia (277589003); Myelodysplastic and myeloproliferative neoplasm with neutrophilia (128826001)
 
Monarch Initiative: MONDO:0004653
OMIM®: 608232
Orphanet: ORPHA98824

Definition

A rare myelodysplastic/myeloproliferative neoplasm characterized by peripheral blood leukocytosis due to increased numbers of morphologically dysplastic neutrophils and their precursors, hypercellular bone marrow with granulocytic proliferation and dysplasia (with or without dysplasia in the erythroid and megakaryocytic lineages), and prominent dysgranulopoiesis, but no or minimal absolute basophilia or monocytosis. Blasts account for less than 20% of leukocytes in the blood and bone marrow. BCR-ABL1 fusion is absent, as well as PDGFRA, PDGFRB or FGFR1 rearrangement, or PCM1-JAK2. Patients may present with signs and symptoms related to splenomegaly, anemia, or thrombocytopenia. Prognosis is generally poor. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAtypical chronic myeloid leukemia, BCR-ABL1 negative

Supplemental Content

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    Clinical resources

    Practice guidelines

    • Bookshelf
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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myelodysplastic Syndromes, 2024
    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Chronic Myeloid Leukemia, 2023

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