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Splenic diffuse red pulp small B-cell lymphoma

MedGen UID:
397437
•Concept ID:
C2699508
•
Neoplastic Process
Synonyms: SDRPL; Splenic diffuse red pulp lymphoma; splenic diffuse red pulp lymphoma; Splenic Diffuse Red Pulp Small B-Cell Lymphoma; splenic diffuse red pulp small B-cell lymphoma
SNOMED CT: Splenic diffuse red pulp small B-cell lymphoma (734067001); Splenic diffuse red pulp small B-cell lymphoma (763884007)
 
Monarch Initiative: MONDO:0017599
Orphanet: ORPHA300869

Definition

A rare indolent B-cell non-Hodgkin lymphoma with characteristics of abnormal proliferation of small monomorphous basophilic B-lymphocytes with villous cytoplasm in the splenic red pulp, bone marrow and peripheral blood. It typically presents in the late clinical stages with splenomegaly and moderate lymphocytosis. Cytopenia is rare and likely associated with hypersplenism. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSplenic diffuse red pulp small B-cell lymphoma

Professional guidelines

PubMed

New insights in the management of patients with hairy cell leukemia.
Cornet E, Damaj G, Troussard X
Curr Opin Oncol 2015 Sep;27(5):371-6. doi: 10.1097/CCO.0000000000000214. PMID: 26154707

Recent clinical studies

Etiology

CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen.
Schmieg JJ, Muir JM, Aguilera NS, Auerbach A
Curr Oncol 2021 Dec 4;28(6):5124-5147. doi: 10.3390/curroncol28060430. PMID: 34940069Free PMC Article
Targeted next-generation sequencing reveals molecular heterogeneity in non-chronic lymphocytic leukemia clonal B-cell lymphocytosis.
Defrancesco I, Zibellini S, Boveri E, Frigeni M, Ferretti VV, Rizzo E, Bonometti A, Capuano F, Candido C, Rattotti S, Tenore A, Picone C, Flospergher E, Zerbi C, Bergamini F, Fabbri N, Cristinelli C, Varettoni M, Paulli M, Arcaini L
Hematol Oncol 2020 Dec;38(5):689-697. Epub 2020 Aug 19 doi: 10.1002/hon.2784. PMID: 32738175
Clinicopathological features of splenic tumours of lymphoid tissue.
Chen DB, Shen DH, Yang SM, Fang XZ
Pathol Res Pract 2018 Dec;214(12):1952-1958. Epub 2018 Jul 27 doi: 10.1016/j.prp.2018.07.021. PMID: 30087035
NOTCH1, TP53, and MAP2K1 Mutations in Splenic Diffuse Red Pulp Small B-cell Lymphoma Are Associated With Progressive Disease.
Martinez D, Navarro A, Martinez-Trillos A, Molina-Urra R, Gonzalez-Farre B, Salaverria I, Nadeu F, Enjuanes A, Clot G, Costa D, Carrio A, Villamor N, Colomer D, Martinez A, Bens S, Siebert R, Wotherspoon A, Beà S, Matutes E, Campo E
Am J Surg Pathol 2016 Feb;40(2):192-201. doi: 10.1097/PAS.0000000000000523. PMID: 26426381
Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features.
Kanellis G, Mollejo M, Montes-Moreno S, Rodriguez-Pinilla SM, Cigudosa JC, Algara P, Montalban C, Matutes E, Wotherspoon A, Piris MA
Haematologica 2010 Jul;95(7):1122-9. Epub 2010 Mar 10 doi: 10.3324/haematol.2009.013714. PMID: 20220064Free PMC Article

Diagnosis

The many faces of nodal and splenic marginal zone lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop.
Zamò A, van den Brand M, Climent F, de Leval L, Dirnhofer S, Leoncini L, Ng SB, Ondrejka SL, Quintanilla-Martinez L, Soma L, Wotherspoon A
Virchows Arch 2023 Sep;483(3):317-331. Epub 2023 Sep 1 doi: 10.1007/s00428-023-03633-3. PMID: 37656249Free PMC Article
A Review on Splenic Diffuse Red Pulp Small B-Cell Lymphoma.
Yilmaz E, Chhina A, Nava VE, Aggarwal A
Curr Oncol 2021 Dec 6;28(6):5148-5154. doi: 10.3390/curroncol28060431. PMID: 34940070Free PMC Article
CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen.
Schmieg JJ, Muir JM, Aguilera NS, Auerbach A
Curr Oncol 2021 Dec 4;28(6):5124-5147. doi: 10.3390/curroncol28060430. PMID: 34940069Free PMC Article
Bone marrow and splenic histology in hairy cell leukaemia.
Wotherspoon A, Attygalle A, Mendes LS
Best Pract Res Clin Haematol 2015 Dec;28(4):200-7. Epub 2015 Oct 24 doi: 10.1016/j.beha.2015.10.019. PMID: 26614898
Splenic diffuse red pulp small-B cell lymphoma: toward the emergence of a new lymphoma entity.
Traverse-Glehen A, Baseggio L, Salles G, Coiffier B, Felman P, Berger F
Discov Med 2012 Apr;13(71):253-65. PMID: 22541613

Therapy

New insights in the management of patients with hairy cell leukemia.
Cornet E, Damaj G, Troussard X
Curr Opin Oncol 2015 Sep;27(5):371-6. doi: 10.1097/CCO.0000000000000214. PMID: 26154707

Prognosis

Clinicopathological features of splenic tumours of lymphoid tissue.
Chen DB, Shen DH, Yang SM, Fang XZ
Pathol Res Pract 2018 Dec;214(12):1952-1958. Epub 2018 Jul 27 doi: 10.1016/j.prp.2018.07.021. PMID: 30087035
Splenic diffuse red-pulp small B-cell lymphoma associated with hepatitis B virus: a report of two cases.
Kerbauy MN, Fernandes CM, Bezerra ED, Lage LA, Siqueira SA, Pereira J
Sao Paulo Med J 2016 Jul-Sep;134(4):359-65. Epub 2016 Jul 18 doi: 10.1590/1516-3180.2016.0035130416. PMID: 27581334Free PMC Article
NOTCH1, TP53, and MAP2K1 Mutations in Splenic Diffuse Red Pulp Small B-cell Lymphoma Are Associated With Progressive Disease.
Martinez D, Navarro A, Martinez-Trillos A, Molina-Urra R, Gonzalez-Farre B, Salaverria I, Nadeu F, Enjuanes A, Clot G, Costa D, Carrio A, Villamor N, Colomer D, Martinez A, Bens S, Siebert R, Wotherspoon A, Beà S, Matutes E, Campo E
Am J Surg Pathol 2016 Feb;40(2):192-201. doi: 10.1097/PAS.0000000000000523. PMID: 26426381
New insights in the management of patients with hairy cell leukemia.
Cornet E, Damaj G, Troussard X
Curr Opin Oncol 2015 Sep;27(5):371-6. doi: 10.1097/CCO.0000000000000214. PMID: 26154707
Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features.
Kanellis G, Mollejo M, Montes-Moreno S, Rodriguez-Pinilla SM, Cigudosa JC, Algara P, Montalban C, Matutes E, Wotherspoon A, Piris MA
Haematologica 2010 Jul;95(7):1122-9. Epub 2010 Mar 10 doi: 10.3324/haematol.2009.013714. PMID: 20220064Free PMC Article

Clinical prediction guides

CD27-positive hairy cell leukemia-Japanese variant.
Tabata R, Tabata C, Iwama H, Yasumizu R, Kojima M
Virchows Arch 2016 Mar;468(3):375-9. Epub 2016 Feb 11 doi: 10.1007/s00428-015-1881-x. PMID: 26868143
NOTCH1, TP53, and MAP2K1 Mutations in Splenic Diffuse Red Pulp Small B-cell Lymphoma Are Associated With Progressive Disease.
Martinez D, Navarro A, Martinez-Trillos A, Molina-Urra R, Gonzalez-Farre B, Salaverria I, Nadeu F, Enjuanes A, Clot G, Costa D, Carrio A, Villamor N, Colomer D, Martinez A, Bens S, Siebert R, Wotherspoon A, Beà S, Matutes E, Campo E
Am J Surg Pathol 2016 Feb;40(2):192-201. doi: 10.1097/PAS.0000000000000523. PMID: 26426381
New insights in the management of patients with hairy cell leukemia.
Cornet E, Damaj G, Troussard X
Curr Opin Oncol 2015 Sep;27(5):371-6. doi: 10.1097/CCO.0000000000000214. PMID: 26154707
Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL).
Baseggio L, Traverse-Glehen A, Callet-Bauchu E, Morel D, Magaud JP, Berger F, Salles G, Felman P
Hematol Oncol 2011 Mar;29(1):47-51. doi: 10.1002/hon.957. PMID: 20677173

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