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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

Professional guidelines

PubMed

Castellino A, Santambrogio E, Rapezzi D, Massaia M
Medicina (Kaunas) 2021 Oct 14;57(10) doi: 10.3390/medicina57101104. PMID: 34684141Free PMC Article
Itonaga H, Ota S, Ikeda T, Taji H, Amano I, Hasegawa Y, Ichinohe T, Fukuda T, Atsuta Y, Tanizawa A, Kondo T, Miyazaki Y
Leuk Res 2018 Dec;75:50-57. Epub 2018 Nov 13 doi: 10.1016/j.leukres.2018.11.003. PMID: 30458320

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Myelodysplastic Syndromes, 2024

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Chronic Myeloid Leukemia, 2023

Recent clinical studies

Etiology

Castellino A, Santambrogio E, Rapezzi D, Massaia M
Medicina (Kaunas) 2021 Oct 14;57(10) doi: 10.3390/medicina57101104. PMID: 34684141Free PMC Article
Hahm C, Huh HJ, Mun YC, Seong CM, Chung WS, Huh J
Int J Lab Hematol 2015 Apr;37(2):181-9. Epub 2014 May 21 doi: 10.1111/ijlh.12257. PMID: 24845343

Diagnosis

Castellino A, Santambrogio E, Rapezzi D, Massaia M
Medicina (Kaunas) 2021 Oct 14;57(10) doi: 10.3390/medicina57101104. PMID: 34684141Free PMC Article
Crisà E, Nicolosi M, Ferri V, Favini C, Gaidano G, Patriarca A
Int J Mol Sci 2020 Sep 18;21(18) doi: 10.3390/ijms21186862. PMID: 32962122Free PMC Article
Gotlib J
Blood 2017 Feb 16;129(7):838-845. Epub 2016 Nov 29 doi: 10.1182/blood-2016-08-693630. PMID: 27899359
Freedman JL, Desai AV, Bailey LC, Aplenc R, Burnworth B, Zehentner BK, Teachey DT, Wertheim G
Pediatr Blood Cancer 2016 Jan;63(1):156-9. Epub 2015 Aug 14 doi: 10.1002/pbc.25694. PMID: 26274939
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044

Therapy

Gotlib J
Blood 2017 Feb 16;129(7):838-845. Epub 2016 Nov 29 doi: 10.1182/blood-2016-08-693630. PMID: 27899359
Saqlain N, Ahmed N
J Coll Physicians Surg Pak 2016 Nov;26(11):103-105. PMID: 28666496
Freedman JL, Desai AV, Bailey LC, Aplenc R, Burnworth B, Zehentner BK, Teachey DT, Wertheim G
Pediatr Blood Cancer 2016 Jan;63(1):156-9. Epub 2015 Aug 14 doi: 10.1002/pbc.25694. PMID: 26274939

Prognosis

Castellino A, Santambrogio E, Rapezzi D, Massaia M
Medicina (Kaunas) 2021 Oct 14;57(10) doi: 10.3390/medicina57101104. PMID: 34684141Free PMC Article
Gotlib J
Blood 2017 Feb 16;129(7):838-845. Epub 2016 Nov 29 doi: 10.1182/blood-2016-08-693630. PMID: 27899359
Saqlain N, Ahmed N
J Coll Physicians Surg Pak 2016 Nov;26(11):103-105. PMID: 28666496
Hahm C, Huh HJ, Mun YC, Seong CM, Chung WS, Huh J
Int J Lab Hematol 2015 Apr;37(2):181-9. Epub 2014 May 21 doi: 10.1111/ijlh.12257. PMID: 24845343
Cazzola M, Malcovati L, Invernizzi R
Hematology Am Soc Hematol Educ Program 2011;2011:264-72. doi: 10.1182/asheducation-2011.1.264. PMID: 22160044

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    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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