title: Splenic small B-cell lymphoma with predominant red pulp involvement: a diffuse variant of splenic marginal zone lymphoma?
abstract: OBJECTIVE
  Splenic marginal zone lymphoma (SMZL) has been characterized by a micronodular pattern of infiltration, biphasic cytology, follicular replacement and the presence of marginal zone differentiation. Here we describe four cases with some distinctive features, such as diffuse splenic infiltration, lack of micronodules, marginal zone cytology, p53 inactivation and cutaneous involvement.
  
  
  RESULTS
  In the course of a review of cases of SMZL, we recognized the existence of a subset of four cases of splenic B-cell lymphoma, with predominantly red pulp involvement, absence of follicular replacement, and a monomorphous population of tumoral cells resembling marginal zone B-cells, with scattered nucleolated blast cells. The immunophenotype (bcl2+, CD5-, CD10-, CD43-, CD23-, cyclin D1-, IgD- (3/4)) was consistent with SMZL. Bone marrow infiltration (4/4) and peripheral blood involvement (2/4) showed similar findings to those described for SMZL in these locations. However, unlike classical SMZL, 2/4 had cutaneous involvement, and 4/4 cases showed either p53 mutation or anomalous p53 staining (p53+, p21-). CONCLUSIONS; In spite of a diffuse pattern of splenic infiltration, cutaneous involvement and p53 alterations, these cases have findings that overlap with those corresponding to classic SMZL (symptomatology, morphology of bone marrow, lymph nodes, peripheral blood involvement, and immunophenotype). We suggest that these cases be considered a putative variant of SMZL.
authors.affiliation: Department of Pathology and Genetics, Hospital Virgen de la Salud, Toledo, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Mollejo
authors.forename: P
authors.initials: P
authors.lastname: Algara
authors.forename: M S
authors.initials: MS
authors.lastname: Mateo
authors.forename: M
authors.initials: M
authors.lastname: Sánchez-Beato
authors.forename: E
authors.initials: E
authors.lastname: Lloret
authors.forename: M T
authors.initials: MT
authors.lastname: Medina
authors.forename: M A
authors.initials: MA
authors.lastname: Piris
mesh_terms: D000368:Aged; D004252:DNA Mutational Analysis; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D016158:Genes, p53 / Q000235:genetics; D006801:Humans; D007124:Immunoenzyme Techniques; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D008297:Male; D009154:Mutation; D013160:Splenic Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D016159:Tumor Suppressor Protein p53 / Q000032:analysis / Q000235:genetics
chemical_list: D004273:DNA, Neoplasm; D016159:Tumor Suppressor Protein p53