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Plasma cell leukemia

MedGen UID:
9733
Concept ID:
C0023484
Neoplastic Process
Synonyms: Leukemia, Plasma Cell; Leukemia, Plasmacytic; Leukemias, Plasma Cell; Leukemias, Plasmacytic; Plasma Cell Leukemia; Plasma Cell Leukemias; Plasmacytic Leukemia; Plasmacytic Leukemias
SNOMED CT: Plasma cell leukemia (95210003); Plasmacytic leukemia (128922003); Plasma cell leukemia (128922003); Plasma cell leukemia, disease (95210003); Plasma cell leukemia, morphology (128922003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018689
Orphanet: ORPHA454714

Definition

A rare plasma cell neoplasm characterized by peripheral plasmacytosis, usually with extensive and diffuse infiltration of the bone marrow, and monoclonal paraproteinemia. Neoplastic plasma cells may also be found in extramedullary sites, such as the liver or spleen, among others. Most cases present as primary plasma cell leukemia without previous diagnosis of myeloma. The condition can also represent leukemic transformation of plasma cell myeloma (secondary plasma cell leukemia). Clinical manifestations include lymphadenopathy, organomegaly, renal failure, bone marrow failure, and peripheral neuropathies. High serum levels of lactate dehydrogenase and beta2-microglobulin, as well as hypercalcemia (potentially leading to hypercalcemic crisis) are typically observed. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPlasma cell leukemia

Professional guidelines

PubMed

Gowin K, Skerget S, Keats JJ, Mikhael J, Cowan AJ
Leuk Res 2021 Dec;111:106687. Epub 2021 Aug 17 doi: 10.1016/j.leukres.2021.106687. PMID: 34425325
Gundesen MT, Lund T, Moeller HEH, Abildgaard N
Curr Oncol Rep 2019 Jan 28;21(1):8. doi: 10.1007/s11912-019-0754-x. PMID: 30689121Free PMC Article
Jelinek T, Kryukov F, Rihova L, Hajek R
Eur J Haematol 2015 Jul;95(1):16-26. Epub 2015 Mar 16 doi: 10.1111/ejh.12533. PMID: 25778450

Recent clinical studies

Etiology

van de Donk NWCJ
J Clin Oncol 2023 Mar 1;41(7):1342-1349. Epub 2022 Nov 30 doi: 10.1200/JCO.22.02114. PMID: 36450104
Jelinek T, Bezdekova R, Zihala D, Sevcikova T, Anilkumar Sithara A, Pospisilova L, Sevcikova S, Polackova P, Stork M, Knechtova Z, Venglar O, Kapustova V, Popkova T, Muronova L, Chyra Z, Hrdinka M, Simicek M, Garcés JJ, Puig N, Cedena MT, Jurczyszyn A, Castillo JJ, Penka M, Radocha J, Mateos MV, San-Miguel JF, Paiva B, Pour L, Rihova L, Hajek R
J Clin Oncol 2023 Mar 1;41(7):1383-1392. Epub 2022 Oct 31 doi: 10.1200/JCO.22.01226. PMID: 36315921Free PMC Article
Fernández de Larrea C, Kyle R, Rosiñol L, Paiva B, Engelhardt M, Usmani S, Caers J, Gonsalves W, Schjesvold F, Merlini G, Lentzch S, Ocio E, Garderet L, Moreau P, Sonneveld P, Badros A, Gahrton G, Goldschmidt H, Tuchman S, Einsele H, Durie B, Wirk B, Musto P, Hayden P, Kaiser M, Miguel JS, Bladé J, Rajkumar SV, Mateos MV
Blood Cancer J 2021 Dec 2;11(12):192. doi: 10.1038/s41408-021-00587-0. PMID: 34857730Free PMC Article
Alsouqi A, Khan M, Dhakal B, Du L, Harrell S, Hari P, Cornell RF
Clin Lymphoma Myeloma Leuk 2021 Aug;21(8):526-535. Epub 2021 Apr 6 doi: 10.1016/j.clml.2021.03.013. PMID: 33985931
Neri A, Todoerti K, Lionetti M, Simeon V, Barbieri M, Nozza F, Vona G, Pompa A, Baldini L, Musto P
Expert Rev Hematol 2016 Nov;9(11):1063-1073. Epub 2016 Oct 24 doi: 10.1080/17474086.2016.1244002. PMID: 27759436

Diagnosis

Fernández de Larrea C, Kyle R, Rosiñol L, Paiva B, Engelhardt M, Usmani S, Caers J, Gonsalves W, Schjesvold F, Merlini G, Lentzch S, Ocio E, Garderet L, Moreau P, Sonneveld P, Badros A, Gahrton G, Goldschmidt H, Tuchman S, Einsele H, Durie B, Wirk B, Musto P, Hayden P, Kaiser M, Miguel JS, Bladé J, Rajkumar SV, Mateos MV
Blood Cancer J 2021 Dec 2;11(12):192. doi: 10.1038/s41408-021-00587-0. PMID: 34857730Free PMC Article
Gowin K, Skerget S, Keats JJ, Mikhael J, Cowan AJ
Leuk Res 2021 Dec;111:106687. Epub 2021 Aug 17 doi: 10.1016/j.leukres.2021.106687. PMID: 34425325
Gundesen MT, Lund T, Moeller HEH, Abildgaard N
Curr Oncol Rep 2019 Jan 28;21(1):8. doi: 10.1007/s11912-019-0754-x. PMID: 30689121Free PMC Article
Kazandjian D
Semin Oncol 2016 Dec;43(6):676-681. Epub 2016 Nov 10 doi: 10.1053/j.seminoncol.2016.11.004. PMID: 28061985Free PMC Article
O'Connell TX, Horita TJ, Kasravi B
Am Fam Physician 2005 Jan 1;71(1):105-12. PMID: 15663032

Therapy

Jelinek T, Bezdekova R, Zihala D, Sevcikova T, Anilkumar Sithara A, Pospisilova L, Sevcikova S, Polackova P, Stork M, Knechtova Z, Venglar O, Kapustova V, Popkova T, Muronova L, Chyra Z, Hrdinka M, Simicek M, Garcés JJ, Puig N, Cedena MT, Jurczyszyn A, Castillo JJ, Penka M, Radocha J, Mateos MV, San-Miguel JF, Paiva B, Pour L, Rihova L, Hajek R
J Clin Oncol 2023 Mar 1;41(7):1383-1392. Epub 2022 Oct 31 doi: 10.1200/JCO.22.01226. PMID: 36315921Free PMC Article
Alsouqi A, Khan M, Dhakal B, Du L, Harrell S, Hari P, Cornell RF
Clin Lymphoma Myeloma Leuk 2021 Aug;21(8):526-535. Epub 2021 Apr 6 doi: 10.1016/j.clml.2021.03.013. PMID: 33985931
Kazandjian D
Semin Oncol 2016 Dec;43(6):676-681. Epub 2016 Nov 10 doi: 10.1053/j.seminoncol.2016.11.004. PMID: 28061985Free PMC Article
Albarracin F, Fonseca R
Blood Rev 2011 May;25(3):107-12. Epub 2011 Feb 3 doi: 10.1016/j.blre.2011.01.005. PMID: 21295388Free PMC Article
Hayman SR, Fonseca R
Curr Treat Options Oncol 2001 Jun;2(3):205-16. doi: 10.1007/s11864-001-0034-4. PMID: 12057120

Prognosis

Fernández de Larrea C, Kyle R, Rosiñol L, Paiva B, Engelhardt M, Usmani S, Caers J, Gonsalves W, Schjesvold F, Merlini G, Lentzch S, Ocio E, Garderet L, Moreau P, Sonneveld P, Badros A, Gahrton G, Goldschmidt H, Tuchman S, Einsele H, Durie B, Wirk B, Musto P, Hayden P, Kaiser M, Miguel JS, Bladé J, Rajkumar SV, Mateos MV
Blood Cancer J 2021 Dec 2;11(12):192. doi: 10.1038/s41408-021-00587-0. PMID: 34857730Free PMC Article
Gowin K, Skerget S, Keats JJ, Mikhael J, Cowan AJ
Leuk Res 2021 Dec;111:106687. Epub 2021 Aug 17 doi: 10.1016/j.leukres.2021.106687. PMID: 34425325
Gundesen MT, Lund T, Moeller HEH, Abildgaard N
Curr Oncol Rep 2019 Jan 28;21(1):8. doi: 10.1007/s11912-019-0754-x. PMID: 30689121Free PMC Article
O'Connell TX, Horita TJ, Kasravi B
Am Fam Physician 2005 Jan 1;71(1):105-12. PMID: 15663032
Hayman SR, Fonseca R
Curr Treat Options Oncol 2001 Jun;2(3):205-16. doi: 10.1007/s11864-001-0034-4. PMID: 12057120

Clinical prediction guides

Kaiser MF, Hall A, Walker K, Sherborne A, De Tute RM, Newnham N, Roberts S, Ingleson E, Bowles K, Garg M, Lokare A, Messiou C, Houlston RS, Jackson G, Cook G, Pratt G, Owen RG, Drayson MT, Brown SR, Jenner MW
J Clin Oncol 2023 Aug 10;41(23):3945-3955. Epub 2023 Jun 14 doi: 10.1200/JCO.22.02567. PMID: 37315268
van de Donk NWCJ
J Clin Oncol 2023 Mar 1;41(7):1342-1349. Epub 2022 Nov 30 doi: 10.1200/JCO.22.02114. PMID: 36450104
Gundesen MT, Lund T, Moeller HEH, Abildgaard N
Curr Oncol Rep 2019 Jan 28;21(1):8. doi: 10.1007/s11912-019-0754-x. PMID: 30689121Free PMC Article
Kazandjian D
Semin Oncol 2016 Dec;43(6):676-681. Epub 2016 Nov 10 doi: 10.1053/j.seminoncol.2016.11.004. PMID: 28061985Free PMC Article
O'Connell TX, Horita TJ, Kasravi B
Am Fam Physician 2005 Jan 1;71(1):105-12. PMID: 15663032

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