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Acute undifferentiated leukemia

MedGen UID:
79081
Concept ID:
C0280141
Neoplastic Process
Synonyms: acute leukaemia not otherwise specified; Acute myeloid leukemia, minimal differentiation, FAB M0; acute myeloid leukemia, minimal differentiation, FAB M0; Acute Undifferentiated Leukemia; acute undifferentiated leukemia; AUL; AUL - acute undifferentiated leukemia; leukaemia stem cell; Leukemia Stem Cell; leukemia stem cell; stem cell acute leukaemia; Stem Cell Acute Leukemia; stem cell acute leukemia; stem cell leukaemia; Stem Cell Leukemia; stem cell leukemia; undifferentiated acute leukaemia; Undifferentiated Acute Leukemia; undifferentiated acute leukemia
SNOMED CT: AUL - acute undifferentiated leukemia (1264458000); Acute undifferentiated leukemia (1264458000)
 
Monarch Initiative: MONDO:0020321
Orphanet: ORPHA98835

Definition

A rare acute leukaemia of ambiguous lineage characterised by clonal proliferation of primitive haematopoietic cells, primarily in the bone marrow and blood, lacking lineage-specific markers and detectable genotypic alterations. The patient presents with leucocytosis, anaemia, variable platelet count and a variety of nonspecific symptoms related to ineffective haematopoesis (fatigue, bleeding and bruising, recurrent infections, bone pain) and/or extramedullary site involvement (lymphadenopathy, splenomegaly, hepatomegaly). [from SNOMEDCT_US]

Professional guidelines

PubMed

Atas U, Turgut S, Ulas T, Iltar U, Salim O, Karauzum SB, Undar L
Clin Ter 2022 Jul-Aug;173(4):297-298. doi: 10.7417/CT.2022.2435. PMID: 35857043
Ahmadi A, Poorfathollah AA, Aghaiipour M, Rezaei M, Nikoo-ghoftar M, Abdi M, Gharib A, Amini A
Tumour Biol 2014 Jul;35(7):6763-8. Epub 2014 Apr 11 doi: 10.1007/s13277-014-1899-8. PMID: 24722823
Walters RS, Kantarjian HM, Keating MJ, Estey EH, McCredie KB, Freireich EJ
Cancer 1987 Jul 15;60(2):149-55. doi: 10.1002/1097-0142(19870715)60:2<149::aid-cncr2820600204>3.0.co;2-f. PMID: 3474052

Recent clinical studies

Etiology

Rai V, Brahmbhatt B, Saha A, Thayakaran IP
Indian J Pathol Microbiol 2024 Jan-Mar;67(1):121-127. doi: 10.4103/ijpm.ijpm_59_22. PMID: 38358200
Qasrawi A, Gomes V, Chacko CA, Mansour A, Kesler M, Arora R, Wei S, Ramlal R, Munker R
Leuk Res 2020 Feb;89:106301. Epub 2020 Jan 17 doi: 10.1016/j.leukres.2020.106301. PMID: 31982153
Weinberg OK, Hasserjian RP, Baraban E, Ok CY, Geyer JT, Philip JKSS, Kurzer JH, Rogers HJ, Nardi V, Stone RM, Garcia JS, Hsi ED, Bagg A, Wang SA, Orazi A, Arber DA
Mod Pathol 2019 Sep;32(9):1373-1385. Epub 2019 Apr 18 doi: 10.1038/s41379-019-0263-3. PMID: 31000771
Imamura T, Taga T, Takagi M, Kawasaki H, Koh K, Taki T, Adachi S, Manabe A, Ishida Y; Leukemia/Lymphoma Committee; Japanese Society of Pediatric Hematology Oncology (JSPHO)
Int J Hematol 2018 Jul;108(1):91-97. Epub 2018 Mar 24 doi: 10.1007/s12185-018-2439-x. PMID: 29574603
van't Veer MB
Ann Hematol 1992 Apr;64(4):161-5. doi: 10.1007/BF01696217. PMID: 1581403

Diagnosis

Hennawi M, Pakasticali N, Tashkandi H, Hussaini M
Int J Mol Sci 2022 Sep 24;23(19) doi: 10.3390/ijms231911259. PMID: 36232559Free PMC Article
Weinberg OK, Arber DA
Am J Clin Pathol 2022 Jul 1;158(1):27-34. doi: 10.1093/ajcp/aqac070. PMID: 35775438
Kurzer JH, Weinberg OK
Surg Pathol Clin 2019 Sep;12(3):687-697. doi: 10.1016/j.path.2019.03.008. PMID: 31352981
Porwit A, Béné MC
Am J Clin Pathol 2015 Sep;144(3):361-76. doi: 10.1309/AJCPSTU55DRQEGTE. PMID: 26276768
van't Veer MB
Ann Hematol 1992 Apr;64(4):161-5. doi: 10.1007/BF01696217. PMID: 1581403

Therapy

Cui Y, Mi R, Chen L, Wang L, Li D, Wei X
Hematology 2024 Dec;29(1):2293494. Epub 2023 Dec 14 doi: 10.1080/16078454.2023.2293494. PMID: 38095304
Caldwell KJ, Budhraja A, Opferman JT, Pui CH, Klco JM, Rubnitz JE
Cancer 2021 Aug 1;127(15):2608-2611. Epub 2021 Apr 6 doi: 10.1002/cncr.33531. PMID: 33823069Free PMC Article
Kampa-Schittenhelm KM, Salitzky O, Akmut F, Illing B, Kanz L, Salih HR, Schittenhelm MM
BMC Cancer 2016 Jan 16;16:25. doi: 10.1186/s12885-015-2029-8. PMID: 26775260Free PMC Article
Campana D, Hansen-Hagge TE, Matutes E, Coustan-Smith E, Yokota S, Shetty V, Bartram CR, Janossy G
Leukemia 1990 Sep;4(9):620-4. PMID: 2395382
Hoelzer D, Thiel E, Löffler H, Bodenstein H, Plaumann L, Büchner T, Urbanitz D, Koch P, Heimpel H, Engelhardt R
Blood 1984 Jul;64(1):38-47. PMID: 6375764

Prognosis

Ma J, Liu YC, Voss RK, Ma J, Palagani A, Caldwell E, Rosikiewicz W, Cardenas M, Foy S, Umeda M, Wilkinson MR, Inaba H, Klco JM, Rubnitz JE, Wang L
Leukemia 2024 May;38(5):981-990. Epub 2024 Mar 1 doi: 10.1038/s41375-024-02194-x. PMID: 38429501
Hennawi M, Pakasticali N, Tashkandi H, Hussaini M
Int J Mol Sci 2022 Sep 24;23(19) doi: 10.3390/ijms231911259. PMID: 36232559Free PMC Article
Caldwell KJ, Budhraja A, Opferman JT, Pui CH, Klco JM, Rubnitz JE
Cancer 2021 Aug 1;127(15):2608-2611. Epub 2021 Apr 6 doi: 10.1002/cncr.33531. PMID: 33823069Free PMC Article
Qasrawi A, Gomes V, Chacko CA, Mansour A, Kesler M, Arora R, Wei S, Ramlal R, Munker R
Leuk Res 2020 Feb;89:106301. Epub 2020 Jan 17 doi: 10.1016/j.leukres.2020.106301. PMID: 31982153
Kurzer JH, Weinberg OK
Surg Pathol Clin 2019 Sep;12(3):687-697. doi: 10.1016/j.path.2019.03.008. PMID: 31352981

Clinical prediction guides

Rai V, Brahmbhatt B, Saha A, Thayakaran IP
Indian J Pathol Microbiol 2024 Jan-Mar;67(1):121-127. doi: 10.4103/ijpm.ijpm_59_22. PMID: 38358200
Wang J, Bhakta N, Ayer Miller V, Revsine M, Litzow MR, Paietta E, Fedoriw Y, Roberts KG, Gu Z, Mullighan CG, Jones CD, Alexander TB
JCO Precis Oncol 2022 Apr;6:e2100326. doi: 10.1200/PO.21.00326. PMID: 35442720Free PMC Article
Qasrawi A, Gomes V, Chacko CA, Mansour A, Kesler M, Arora R, Wei S, Ramlal R, Munker R
Leuk Res 2020 Feb;89:106301. Epub 2020 Jan 17 doi: 10.1016/j.leukres.2020.106301. PMID: 31982153
Kampa-Schittenhelm KM, Salitzky O, Akmut F, Illing B, Kanz L, Salih HR, Schittenhelm MM
BMC Cancer 2016 Jan 16;16:25. doi: 10.1186/s12885-015-2029-8. PMID: 26775260Free PMC Article
Heesch S, Neumann M, Schwartz S, Bartram I, Schlee C, Burmeister T, Hänel M, Ganser A, Heuser M, Wendtner CM, Berdel WE, Gökbuget N, Hoelzer D, Hofmann WK, Thiel E, Baldus CD
Ann Hematol 2013 Jun;92(6):747-58. Epub 2013 Feb 15 doi: 10.1007/s00277-013-1694-4. PMID: 23412561

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