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.
Service d'Anatomie Pathologique Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Lyon, France.
Alexandra
A
Traverse-Glehen
Lucile
L
Baseggio
Evelyne Callet-
EC
Bauchu
Dominique
D
Morel
Sophie
S
Gazzo
Martine
M
Ffrench
Aurélie
A
Verney
Delphine
D
Rolland
Catherine
C
Thieblemont
Jean-Pierre
JP
Magaud
Gilles
G
Salles
Bertrand
B
Coiffier
Françoise
F
Berger
Pascale
P
Felman
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