What is Marginal Zone Lymphoma?

Marginal zone lymphoma is a slow-growing type of B-cell non-Hodgkin lymphoma that begins forming in certain areas (marginal zones) of lymph tissue. There are three types of marginal zone lymphomas: the extranodal MZL (EMZL) of mucosa-associated lymphoid tissue (MALT or gastric GALT), the splenic MZL, and the nodal MZL.

What is the Pathology of Marginal Zone Lymphoma?

The pathology of marginal zone lymphoma is:

-Etiology: The cause of marginal zone lymphoma is unknown. There is a lot of evidence supporting the notion that antigen stimulation is important for the development and progression of marginal zone lymphoma.

-Genes involved: NOTCH gene, KLF2 gene, PTPRD gene.

-Pathogenesis: The sequence of events that lead to marginal zone lymphoma often associated with chronic antigenic stimulation of post-germinal center marginal zone B-lymphocytes.

-Histology: The histology associated with marginal zone lymphoma shows small to medium sized lymphocytes surrounding a reactive follicle. Plasmacytic differentiation is common.

Marginal Zone Lymphoma. Morphology of Splenic marginal zone lymphoma. Characteristic micronodular pattern in the SMZL centered in the white pulp, with variable red pulp infiltration (H&E, original magnification ×100). Coexistence of splenic marginal zone lymphoma with hepatocellular carcinoma: a case report. Zhang SH, Xu AM, Zheng JM, He MX - Diagnostic pathology (2007).
Splenic bleeding of splenic marginal zone lymphoma (SMZL). (A) A dynamic CT scan of the abdomen on admission. (B) Histology of the excised spleen. Hematoxylin and Eosin (H&E) staining. Magnification, ×100, inset×1,000. (C) Histology of the excised lymph node. H&E staining. Magnification, ×20. Inset, ×400. (D) PCR amplification of complementarity determining region-3 (CDR-3). The spleen (Sp) and lymph node (LN) lanes show monoclonal bands of the same size. VH26, forward primer. VLJH, reverse primer for second PCR. JH, reverse primer for first PCR. M, size marker. Neg, negative control. Pos, positive control. Splenic Marginal Zone Lymphoma with Acquired von Willebrand Syndrome Diagnosed via Splenic Bleeding, Internal Medicine.
Marginal Zone Lymphoma. Splenic marginal zone B-cell lymphoma, nodular and diffuse pattern of infiltration. a H&E (×10); b CD20 (×10); c TCL1A results negative in the neoplastic cells (×10); d MNDA results positive in the neoplastic component (×10). Absence of TCL1A expression is a useful diagnostic feature in splenic marginal zone lymphoma. Munari E, Rinaldi M, Ambrosetti A, Bonifacio M, Bonalumi A, Chilosi M, Zamò A - Virchows Archiv : an international journal of pathology (2012). Not altered. CC.

How does Marginal Zone Lymphoma Present?

Patients with marginal zone lymphoma typically involve women than men present at age range of 60 years (average age at diagnosis). The symptoms, features, and clinical findings associated with marginal zone lymphoma include fever without an infection, night sweats, unexplained weight loss, skin rash, chest or abdominal pain and tiredness.

How is Marginal Zone Lymphoma Diagnosed?

Marginal zone lymphoma is diagnosed by X-rays, ultrasounds, CT scans, and MRI scans.

How is Marginal Zone Lymphoma Treated?

Marginal zone lymphoma is treated by chemotherapy to kill cancer cells, radiation to shrink tumors and surgery to remove tumors.

What is the Prognosis of Marginal Zone Lymphoma?

The prognosis of marginal zone lymphoma is good and most patients experience long survival.