Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity?
The presence of circulating villous lymphocytes (VLs) in lymphoma patients usually points to splenic marginal zone B-cell lymphoma (SMZL), even if the VLs can be found occasionally in other small B-cell lymphomas. However, those cells are variably described, and detailed cytologic characterization is often lacking. We identified lymphoma cases with numerous basophilic VLs among the large group of splenic lymphoma with VLs, and for further delineation, 37 cases with this particular cytology were analyzed. Patients, predominantly older men, presented with moderate lymphocytosis and splenomegaly without pancytopenia. The monoclonal B cells expressed IgM + D, IgM + G, IgM or IgG, as well as CD76 and CD11c, frequently CD103, and rarely CD123. Spleen sections were peculiar, with atrophic white pulp and a monomorphic diffuse lymphoma infiltration in a congested red pulp. Bone marrow infiltration was interstitial and intrasinusoidal without extensive fibrosis. Cytogenetic analysis showed a frequent absence of clonal aberrations (68%). Most cases (79%) were IgH mutated, with an overrepresentation of V(H)3 and V(H)4 gene families. These results, as well as the clinical evolution, show that those lymphoma cases represent a homogeneous group distinct from SMZL and reminiscent of hairy cell leukemia variant, perhaps corresponding to a separate lymphoma entity.
Traverse-Glehen
Alexandra
A
Service d'Anatomie Pathologique Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France.
Baseggio
Lucile
L
Bauchu
Evelyne Callet-
EC
Morel
Dominique
D
Gazzo
Sophie
S
Ffrench
Martine
M
Verney
Aurélie
A
Rolland
Delphine
D
Thieblemont
Catherine
C
Magaud
Jean-Pierre
JP
Salles
Gilles
G
Coiffier
Bertrand
B
Berger
Françoise
F
Felman
Pascale
P
D015703:Antigens, CD / Q000032:analysis; D001491:Basophils / Q000473:pathology*; D001853:Bone Marrow / Q000473:pathology; D004252:DNA Mutational Analysis; D005434:Flow Cytometry; D006801:Humans; D008214:Lymphocytes / Q000473:pathology*; D016393:Lymphoma, B-Cell / Q000145:classification* / Q000235:genetics / Q000473:pathology*; D008871:Microvilli / Q000473:pathology; D009154:Mutation; D012189:Retrospective Studies; D013154:Spleen / Q000473:pathology; D013160:Splenic Neoplasms / Q000145:classification* / Q000235:genetics / Q000473:pathology*
D015703:Antigens, CD