Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features

Abstract

BackgroundSplenic diffuse red pulp small B-cell lymphoma is an uncommon B-cell lymphoma, now recognized as a provisional entity in the 2008 update of the WHO Classification. Additional work is required to review this entity and establish its diagnostic features. Design and MethodsWe have retrospectively analyzed the disease features in a highly selected series of 17 patients diagnosed as splenic diffuse red pulp small B-cell lymphoma. ResultsThe median age was 65.5 years (range 40-79 years) and there was a predominance of males (male/female ratio: 2.4). Clinical manifestations were mainly derived from splenomegaly. Splenectomy was the front-line treatment in 11 symptomatic patients; the remaining 6 received chemotherapy initially followed by splenectomy. After a mean follow-up of 72 months, the five-year overall survival was 93%. All cases showed a purely diffuse pattern of splenic infiltration by monomorphous small cells with small round nuclei and pale cytoplasm. All bone marrow biopsies showed tumoral infiltration, with intrasinusoidal infiltration. Peripheral blood cells were small to medium-sized, with clumped chromatin and round nuclear outline and villous cytoplasm. ConclusionsOur data suggest that splenic diffuse red pulp small B-cell lymphoma is a distinct entity with morphological and immunophenotypical features that differ from those of other splenic lymphomas.Key words: splenic lymphoma, leukemia, villous cells. Haematologica 2010;95:1122-1129. doi:10.3324/haematol.2009 This is an open-access paper. Citation: Kanellis G, Mollejo M, Montes-Moreno S, Rodriguez-Pinilla S-M, Cigudosa JC, Algara P, Montalban C, Matutes E, Wotherspoon A, and Piris MA. Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features. Splenic diffuse red pulp small B-cell lymphoma: revision of a series of cases reveals characteristic clinico-pathological features

Extranodal and splenic small B-cell lymphoma

“…There may be blood lakes. 68,69 In the bone marrow, there is usually striking intrasinusoidal infiltration usually with an interstitial component ( Figure 5). The peripheral blood shows small cells that have scanty cytoplasm and nuclei with clumped chromatin.…”

Pathomorphological Diagnosis of Splenic Diffuse Red Pulp Small B-Cell Lymphoma

“…Однако критерии диагностики остаются малоизученным вопросом. В литературе присутствуют крайне немногочисленные сведения, посвященные диффузной В-клеточной лимфоме красной пульпы селезенки с описанием диагностических критериев при исследовании операционного материала (спленэктомия) [1]. Нередко публикации по данному вопросу содержат описания отдельных наблюдений с диа-гностикой по материалу трепанобиоптатов костного мозга.…”

Splenic diffuse red-pulp small B-cell lymphoma associated with hepatitis B virus: a report of two cases

“…In order to make the search as wide as possible, no limits were applied regarding the date of publication, the language or the research design The most common finding in the bone marrow biopsies was predominance of intrasinusoidal lymphoid infiltration, some- Kanellis et al 2 found p53 inactivation in all cases of splenic diffuse red-pulp small B-cell lymphoma, comprising either p53 mutation (2/4 cases) or anomalous p53 staining. Mollejo et al 4 reported that TP53 abnormality was present in 2 out of 13 cases (15.3%): one of these patients showed disease progression and ultimately died of the disease.…”

“…1 This new WHO update recognized two categories of primary splenic lymphomas; splenic marginal-zone lymphoma (SMZL) and the provisional group of splenic B-cell lymphoma/leukemia unclassifiable (SLLU), that includes splenic diffuse red-pulp small B-cell lymphoma and hairy-cell leukemia variant (HCL-v). 1 The median age at which patients are diagnosed with splenic diffuse red-pulp small B-cell lymphoma is 72 years (range: 69-74 years), 2 and the disease is seen predominantly among males, with a male/female ratio of 2.4:1.…”