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Background
This is a low grade Non-Hodgkin's lymphoma (NHL), B-cell immunophenotype. Unlike most cases of diffuse small cell lymphomas, this lymphoma tends to occur with localized rather than generalized lymphadenopathy. This lymphoma is the lymph node equivalent of MALT lymphoma, lymphomas arising within mucosal-associated tissues such as the gastrointestinal tract and salivary glands. It is more accurately diagnosed as a marginal zone lymphoma.
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Marginal zone lymphoma INCIDENCE Less common than other types of diffuse small cell lymphomas Females are 2-5x more likely
DISEASE ASSOCIATIONS CHARACTERIZATION Autoimmune disorders Sjogren's syndrome
PATHOGENESIS CHARACTERIZATION Primary nodal marginal zone B-cell lymphoma arising from more than one clonal neoplastic population.
Finch CN, Nichols M, Shrimpton A, Liu D, Hutchison RE.
Department of Pathology, State University of New York Upstate Medical University, Syracuse, New York 13210, USA.
Arch Pathol Lab Med 2000 Dec;124(12):1816-9 Abstract quote
Primary nodal marginal zone B-cell lymphoma is an uncommon monoclonal B-cell lymphoproliferative disorder.
We report a case of a 79-year-old woman who presented with generalized lymphadenopathy. Histologic and immunohistochemical examinations of biopsy sections from an axillary lymph node were consistent with nodal marginal zone B-cell lymphoma. Flow cytometry analysis showed 2 distinct clonal B-cell populations expressing lambda or kappa light chain restriction. Subsequently, genomic deoxyribonucleic acid (DNA) isolated from a paraffin-embedded lymph node section was analyzed for the presence of gene rearrangements. Polymerase chain reaction (PCR) analysis of immunoglobulin heavy chain genes revealed 3 rearranged DNA bands, confirming the presence of more than one clonal B-cell population. These immunophenotypic and genotypic findings have not been previously described in association with this type of lymphoma.
To our knowledge, this represents the first reported case of biclonal nodal marginal zone B-cell lymphoma.
HISTOLOGICAL TYPES CHARACTERIZATION Lymph Node Prominent sinusoidal pattern
Medium to large cells with abundant clear cytoplasm and irregular or folded nuclei
Resemble reactive monocytoid cells seen in the sinuses of lymph nodes
Frequent presence of PMNs
Frequent follicular colonizationPeripheral Blood Infrequently involved
SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION Positive Surface Ig (mostly IgM)
Cytoplasmic Ig in about 40%
Pan B (CD19, CD20, CD22, CD 79a)
BCL-2 proteinNegative CD5, CD10, CD23
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES PERIPERHAL T-CELL LYMPHOMA
Peripheral T-cell lymphoma mimicking marginal zone B-cell lymphoma.Uherova P, Ross CW, Finn WG, Singleton TP, Kansal R, Schnitzer B.
Department of Pathology, University of Michigan Medical School, Ann Arbor, Michigan 48109-0602, USA.
Mod Pathol 2002 Apr;15(4):420-5 Abstract quote Peripheral T-cell lymphoma (PTCL) may assume a variety of histologic and cytologic appearances.
We describe eight cases of PTCL morphologically simulating marginal zone B-cell lymphoma. We reviewed PTCL cases diagnosed in our institution between 1990 and 2000 and selected eight cases for study based on the following criteria: small-cell morphology with abundant, clear cytoplasm and either marginal zone involvement by the neoplastic infiltrate in lymph node biopsies or lymphoepithelial lesions in extranodal biopsies. Histologic features and ancillary studies were reviewed. Patients included six women and two men with a median age of 53 years (range, 35 to 74 years). Six patients were diagnosed with primary nodal PTCL, and two presented with primary extranodal disease. The original diagnosis was PTCL in only four cases; three cases were diagnosed as atypical lymphoid infiltrate, and one case as benign lymphoepithelial lesion.
Lymph node biopsies revealed partial effacement of the architecture with residual follicles surrounded by the neoplastic small cells. Extranodal sites included hard palate, tongue, tonsil, and submandibular glands; all but one case demonstrated lymphoepithelial lesions. Monoclonality was demonstrated in six of eight cases (rearrangement of T-cell receptor gene), and three of eight had an aberrant T-cell population by flow cytometry. The differential diagnosis of atypical lymphoid infiltrates with morphologic features of marginal zone B-cell lymphoma should include PTCL.
This uncommon morphological mimicry should be recognized, because PTCL is an aggressive disease regardless of morphology and should be treated accordingly.
PROGNOSIS AND TREATMENT CHARACTERIZATION Prognostic Factors Transformation to a large cell lymphoma is a poor prognostic feature Atlas of Tumor Pathology-Tumors of the Lymph Nodes and Spleen. Third Series. Volume 14. AFIP Press. 1995.
Last Updated 5/20/2002
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