Splenic marginal zone lymphoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [5]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [6]

Overview

Genes involved in the pathogenesis of splenic marginal zone lymphoma include immunoglobulin genes , 7q21-32, and CDK6 gene. On microscopic histopathological analysis, B-cells, villous lymphocytes, and sinus invasion are characteristic findings of splenic marginal zone lymphoma.

Genetics

Genes involved in the pathogenesis of splenic marginal zone lymphoma include:

  • Clonal rearrangements of the immunoglobulin genes (heavy and light chains) [1]
  • Deletion 7q21-32 is seen in 40% of SMZL patients
  • Translocations of the CDK6 gene located at 7q21 have also been reported[2]

Immunophenotype

Antigen Splenic marginal zone lymphoma

CD20

Positive

CD79a

Positive

CD5

Negative

CD10

Negative

CD23

Negative

CD43

Negative

cyclin D1

Negative

Microscopic Pathology

  • Splenic marginal zone lymphoma (SMZL) is a lymphoma comprised of B-cells that replace the normal architecture of the white pulp of the spleen.
  • The neoplastic cells are both small lymphocytes and larger, transformed blasts; and they invade the mantle zone of splenic follicles and erode the marginal zone, ultimately invading the red pulp of the spleen.
  • The neoplastic cells circulating in the peripheral blood are termed villious lymphocytes due to their characteristic appearance.[3]
  • Other features that may been seen include sinus invasion, epithelial histocytes, and plasmacytic differentiation of neoplastic cells.
  • Aside from the uniform involvement of the spleen, the bone marrow is frequently positive in patients with splenic marginal zone lymphoma. Nodal and extranodal involvement are rare.[3]
  • Splenic Hilar Lymph Nodes
  • Bone Marrow Biopsy
  • Splenic marginal zone lymphoma in bone marrow displays a nodular pattern with morphology similar to what is observed in the splenic hilar lymph nodes.[4]

References

  1. [1] Dunn-Walters DK, Boursier L, Spencer J, Isaacson PG. "Analysis of immunoglobulin genes in splenic marginal zone lymphoma suggests ongoing mutation." Hum Pathol. 1998 Jun;29(6):585-93. PMID: 9635678
  2. [2] Corcoran MM, Mould SJ, Orchard JA, Ibbotson RE, Chapman RM, Boright AP, Platt C, Tsui LC, Scherer SW, Oscier DG. "Dysregulation of cyclin dependent kinase 6 expression in splenic marginal zone lymphoma through chromosome 7q translocations." Oncogene. 1999 Nov 4;18(46):6271-7. PMID: 10597225
  3. 3.0 3.1 3.2 [3] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001
  4. [4] Franco V, Florena AM, Campesi G. "Intrasinusoidal bone marrow infiltration: a possible hallmark of splenic lymphoma." Histopathology. 1996 Dec;29(6):571-5. PMID: 8971565

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