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Microgranular Acute Promyelocytic Leukemia

MedGen UID:
108266
•Concept ID:
C0563634
•
Neoplastic Process
Synonyms: Acute promyelocytic leukemia - hypogranular variant; Hypogranular Acute Promyelocytic Leukemia; M3V - Acute promyelocytic leukemia - hypogranular variant
SNOMED CT: Acute promyelocytic leukemia - hypogranular variant (285769009); M3V - Acute promyelocytic leukemia - hypogranular variant (285769009)

Definition

Acute promyelocytic leukemia in which the promyelocytes in the peripheral blood have paucity or absence of cytoplasmic granules and characteristic bilobed nuclei. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMicrogranular Acute Promyelocytic Leukemia

Professional guidelines

PubMed

The management of hyperleukocytosis in 2017: Do we still need leukapheresis?
Korkmaz S
Transfus Apher Sci 2018 Feb;57(1):4-7. Epub 2018 Feb 20 doi: 10.1016/j.transci.2018.02.006. PMID: 29477941
Treatment of paediatric APL: how does the therapeutic approach differ from adults?
Kutny MA, Gregory J Jr, Feusner JH
Best Pract Res Clin Haematol 2014 Mar;27(1):69-78. Epub 2014 Apr 26 doi: 10.1016/j.beha.2014.04.007. PMID: 24907019
Management and treatment results in patients with acute promyelocytic leukaemia (APL) not enrolled in clinical trials.
Micol JB, Raffoux E, Boissel N, Lengliné E, Canet E, Daniel MT, Labarthe Ad, Maarek O, Cassinat B, Adès L, Baruchel A, Degos L, Azoulay E, Dombret H
Eur J Cancer 2014 Apr;50(6):1159-68. Epub 2014 Jan 15 doi: 10.1016/j.ejca.2013.11.023. PMID: 24440088

Recent clinical studies

Etiology

Cutaneous promyelocytic sarcoma at sites of vascular access and marrow aspiration. A characteristic localization of chloromas in acute promyelocytic leukemia?
Sanz MA, Larrea L, Sanz G, Martín G, Sempere A, Gomis F, Martínez J, Regadera A, Saavedra S, Jarque I, Jiménez C, Cervera J, de La Rubia J
Haematologica 2000 Jul;85(7):758-62. PMID: 10897129
Short-term treatment for adult hypergranular and microgranular acute promyelocytic leukemia.
Bassan R, Battista R, Viero P, d'Emilio A, Buelli M, Montaldi A, Rambaldi A, Tremul L, Dini E, Barbui T
Leukemia 1995 Feb;9(2):238-43. PMID: 7869758
Hand-mirror variant of microgranular acute promyelocytic leukemia.
Sun T, Weiss R
Leukemia 1991 Mar;5(3):266-9. PMID: 2013982
Microgranular acute promyelocytic leukemia--a case with multiple Auer rods demonstrable only after staining for chloroacetate esterase.
Edelman BB, Grossman NJ
Am J Clin Pathol 1983 May;79(5):621-5. doi: 10.1093/ajcp/79.5.621. PMID: 6188368

Diagnosis

Microgranular acute promyelocytic leukemia with expression of T-cell markers mimicking mixed-phenotype acute leukemia.
Liu W, Xu J
Blood 2020 Mar 12;135(11):880. doi: 10.1182/blood.2019004434. PMID: 32163562
Microgranular acute promyelocytic leukemia presenting with leukopenia and an unusual immunophenotype.
McDonnell MH, Smith ET Jr, Lipford EH, Gerber JM, Grunwald MR
Hematol Oncol Stem Cell Ther 2017 Mar;10(1):35-38. Epub 2016 Jan 16 doi: 10.1016/j.hemonc.2015.12.004. PMID: 26806461
A rare case of microgranular acute promyelocytic leukemia associated with ider(17)(q10)t(15;17) in an old-age patient.
Kim MJ, Cho SY, Lim G, Yoon HS, Lee HJ, Suh JT, Lee J, Lee WI, Cho KS, Park TS
Korean J Lab Med 2011 Apr;31(2):86-90. doi: 10.3343/kjlm.2011.31.2.86. PMID: 21474981Free PMC Article
Microgranular acute promyelocytic leukemia--a case with multiple Auer rods demonstrable only after staining for chloroacetate esterase.
Edelman BB, Grossman NJ
Am J Clin Pathol 1983 May;79(5):621-5. doi: 10.1093/ajcp/79.5.621. PMID: 6188368
"Microgranular" acute promyelocytic leukemia: a distinct clinical, ultrastructural, and cytogenetic entity.
Golomb HM, Rowley JD, Vardiman JW, Testa JR, Butler A
Blood 1980 Feb;55(2):253-9. PMID: 6928105

Therapy

Microgranular acute promyelocytic leukemia presenting with leukopenia and an unusual immunophenotype.
McDonnell MH, Smith ET Jr, Lipford EH, Gerber JM, Grunwald MR
Hematol Oncol Stem Cell Ther 2017 Mar;10(1):35-38. Epub 2016 Jan 16 doi: 10.1016/j.hemonc.2015.12.004. PMID: 26806461
Atypical t(15;17)(q13;q12) in a patient with all-trans retinoic acid refractory secondary acute promyelocytic leukemia: a case report and review of the literature.
Kurian S, Hogan TF, Bleigh OC, Dowdy YG, Merghoub T, Pandolfi PP, Wenger SL
Cancer Genet Cytogenet 2002 Oct 15;138(2):143-8. doi: 10.1016/s0165-4608(02)00591-5. PMID: 12505260
Cutaneous promyelocytic sarcoma at sites of vascular access and marrow aspiration. A characteristic localization of chloromas in acute promyelocytic leukemia?
Sanz MA, Larrea L, Sanz G, Martín G, Sempere A, Gomis F, Martínez J, Regadera A, Saavedra S, Jarque I, Jiménez C, Cervera J, de La Rubia J
Haematologica 2000 Jul;85(7):758-62. PMID: 10897129
Short-term treatment for adult hypergranular and microgranular acute promyelocytic leukemia.
Bassan R, Battista R, Viero P, d'Emilio A, Buelli M, Montaldi A, Rambaldi A, Tremul L, Dini E, Barbui T
Leukemia 1995 Feb;9(2):238-43. PMID: 7869758

Prognosis

A rare case of microgranular acute promyelocytic leukemia associated with ider(17)(q10)t(15;17) in an old-age patient.
Kim MJ, Cho SY, Lim G, Yoon HS, Lee HJ, Suh JT, Lee J, Lee WI, Cho KS, Park TS
Korean J Lab Med 2011 Apr;31(2):86-90. doi: 10.3343/kjlm.2011.31.2.86. PMID: 21474981Free PMC Article
Short-term treatment for adult hypergranular and microgranular acute promyelocytic leukemia.
Bassan R, Battista R, Viero P, d'Emilio A, Buelli M, Montaldi A, Rambaldi A, Tremul L, Dini E, Barbui T
Leukemia 1995 Feb;9(2):238-43. PMID: 7869758
Hand-mirror variant of microgranular acute promyelocytic leukemia.
Sun T, Weiss R
Leukemia 1991 Mar;5(3):266-9. PMID: 2013982
Morphology and cytochemistry of "microgranular" acute promyelocytic leukemia (FAB M3m).
Savage RA, Hoffman GC, Lucas FV Jr
Am J Clin Pathol 1981 Apr;75(4):548-52. doi: 10.1093/ajcp/75.4.548. PMID: 6939328

Clinical prediction guides

Microgranular acute promyelocytic leukemia: a proposed role for a greater deformability of the leukemic cell.
Cantin G, Bernier V, Jacob S, Lyonnais J
Nouv Rev Fr Hematol (1978) 1989;31(1):23-6. PMID: 2915920

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