title: A Single-center Experience in Splenic Diffuse Red Pulp Lymphoma Diagnosis.
abstract: The World Health Organization 2008 classification highlighted a new nosology-splenic diffuse red pulp lymphoma (SDRPL) with clinical and laboratory features similar to both splenic marginal zone lymphoma and hairy cell leukemia (HCL) and variant form of HCL. Experience of hematologists on the diagnosis and differential diagnosis of SDRPL is extremely limited. The aim of our report was to characterize the clinical and immunomorphologic features of SDRPL on our own observations. During 2013-2014, in National Research Center for Hematology, 87 spleen specimens removed from various B-cell lymphomas were analyzed. In four (4.6%) cases, the diagnosis SDRPL was made based on morphologic, immunohistochemical, immunophenotypic, molecular examination of spleen biopsies, blood and bone marrow samples. In all cases of SDRPL were observed significant splenomegaly, lymphocytosis from 56% to 94% (in two cases with leukocytosis 55.000 and 75.000 109/l). The circulating "villous" lymphocytes phenotype was CD20+ (bright), CD11c+/±, CD103 (weakly)+/±, LAIR-1+, CD25-, CD5-, CD10-, and CD23-. Mutation BRAFV600E was not detected. Bone marrow with minor lymphoid CD20+, CD25-, Annexin1-, Cyclin D1- cell infiltration. The average weight of the spleen was 3900 g (1450-9500 g), and morphologically, there was revealed lymphoid infiltration of red pulp with phenotype CD20+, DBA.44+, CD25-, Annexin1-, Cyclin D1-, CD103-, CD123-, CD27-, focal SD11c± and TRAP±. Now patients are observed in remission: two patients after splenectomy, two after splenectomy and cladribine+rituximab chemotherapy. SRDPL-a rare lymphoma that is suspected in the cases with significant splenomegaly and lymphocytosis with villous lymphocytes forms that have only a part of the classic markers HCL, with minor bone marrow infiltration. The standard diagnosis and treatment is splenectomy. Differential diagnosis of SMZL and HCL has clear criteria, but criteria of differentiation with variant HCL are still unknown.
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia. Electronic address: oncohematologist@mail.ru.
authors.forename: Hunan L
authors.initials: HL
authors.lastname: Julhakyan
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: L S
authors.initials: LS
authors.lastname: Al-Radi
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: T N
authors.initials: TN
authors.lastname: Moiseeva
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: K I
authors.initials: KI
authors.lastname: Danishyan
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: A M
authors.initials: AM
authors.lastname: Kovrigina
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: S M
authors.initials: SM
authors.lastname: Glebova
authors.affiliation: Russian Medical Academy of Postdegree Education, Moscow, Russia.
authors.forename: S A
authors.initials: SA
authors.lastname: Lugovskaya
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: V N
authors.initials: VN
authors.lastname: Dvirnik
authors.affiliation: Federal Scientific Clinical Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
authors.forename: A N
authors.initials: AN
authors.lastname: Khvastunova
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: I A
authors.initials: IA
authors.lastname: Yakutik
authors.affiliation: National Research Center for Hematology, Ministry of Healthcare of the Russian Federation, Moscow, Russia.
authors.forename: V G
authors.initials: VG
authors.lastname: Savchenko
mesh_terms: D000368:Aged; D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology; D015415:Biomarkers; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009154:Mutation; D013154:Spleen / Q000378:metabolism; D013160:Splenic Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000628:therapy
chemical_list: D015415:Biomarkers
keywords: Hairy cell leukemia; Splenic diffuse red pulp lymphoma; Splenic lymphoma; Splenic marginal cell lymphoma