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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
Gavriatopoulou M, Musto P, Caers J, Merlini G, Kastritis E, van de Donk N, Gay F, Hegenbart U, Hajek R, Zweegman S, Bruno B, Straka C, Dimopoulos MA, Einsele H, Boccadoro M, Sonneveld P, Engelhardt M, Terpos E
Leukemia 2018 Sep;32(9):1883-1898. Epub 2018 Jul 23 doi: 10.1038/s41375-018-0209-7. PMID: 30038381

Recent clinical studies

Etiology

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
Hofste Op Bruinink D, Kuiper R, van Duin M, Cupedo T, van der Velden VHJ, Hoogenboezem R, van der Holt B, Beverloo HB, Valent ET, Vermeulen M, Gay F, Broijl A, Avet-Loiseau H, Munshi NC, Musto P, Moreau P, Zweegman S, van de Donk NWCJ, Sonneveld P
J Clin Oncol 2022 Sep 20;40(27):3132-3150. Epub 2022 Mar 31 doi: 10.1200/JCO.21.01217. PMID: 35357885Free 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
Jagosky MH, Usmani SZ
Curr Hematol Malig Rep 2020 Apr;15(2):62-71. doi: 10.1007/s11899-020-00568-3. PMID: 32198576

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

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
Tuazon SA, Holmberg LA, Nadeem O, Richardson PG
Blood Cancer J 2021 Feb 4;11(2):23. doi: 10.1038/s41408-021-00414-6. PMID: 33563906Free PMC Article
Al Saleh AS, Sher T, Gertz MA
Acta Haematol 2020;143(5):410-416. Epub 2020 Apr 17 doi: 10.1159/000507690. PMID: 32305989Free PMC Article
Jagosky MH, Usmani SZ
Curr Hematol Malig Rep 2020 Apr;15(2):62-71. doi: 10.1007/s11899-020-00568-3. PMID: 32198576
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

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
Tuazon SA, Holmberg LA, Nadeem O, Richardson PG
Blood Cancer J 2021 Feb 4;11(2):23. doi: 10.1038/s41408-021-00414-6. PMID: 33563906Free PMC Article
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

Clinical prediction guides

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
Wang D, Wang J, Hu G, Wang W, Xiao Y, Cai H, Jiang L, Meng L, Yang Y, Zhou X, Hong Z, Yao Z, Xiao M, Chen L, Mao X, Zhu L, Wang J, Qiu L, Li C, Zhou J
Blood 2021 May 27;137(21):2890-2901. doi: 10.1182/blood.2020008936. PMID: 33512480
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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