Splenic marginal zone lymphoma overview

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Pathophysiology

Causes

Differentiating Splenic marginal zone lymphoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Biopsy

CT

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Splenic marginal zone lymphoma (SMZL) is a lymphoma comprised of B-cells that replace the normal architecture of the white pulp of the spleen. Genes involved in the pathogenesis of splenic marginal zone lymphoma include immunoglobulin genes and CDK6 gene. On microscopic histopathological analysis, B-cells, villous lymphocytes, and sinus invasion are characteristic findings of splenic marginal zone lymphoma.There are no established causes for splenic marginal zone lymphoma. Splenic marginal zone lymphoma must be differentiated from other diseases such as chronic lymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma. The incidence of splenic marginal zone lymphoma increases with age; the median age at diagnosis is 50 years. Splenic marginal zone lymphoma affects men and women equally. There are no established risk factors for splenic marginal zone lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for splenic marginal zone lymphoma. Low Hemoglobin levels, high lactate dehydrogenase levels, low blood serum albumin levels, and genetic mutations such as mutations in NOTCH2 are associated with poor prognosis among patients with splenic marginal zone lymphoma. According to the Lugano classification, there are four stages of splenic marginal zone lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of splenic marginal zone lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of splenic marginal zone lymphoma include fever, rash, ulcer, splenomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy. Laboratory tests for splenic marginal zone lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, genetic testing, FISH, PCR, and immunophenotyping. Lymph node or extranodal tissue biopsy is diagnostic of splenic marginal zone lymphoma. CT scan may be helpful in the diagnosis of splenic marginal zone lymphoma. MRI may be helpful in the diagnosis of splenic marginal zone lymphoma. Abdomen ultrasound may be helpful in the diagnosis of splenic marginal zone lymphoma. Findings on ultrasound abdomen suggestive of splenic marginal zone lymphoma include splenomegaly and lymphadenopathy. PET scan may be helpful in the diagnosis of splenic marginal zone lymphoma. Other diagnostic studies for splenic marginal zone lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy. The predominant therapy for splenic marginal zone lymphoma is surgery. Adjunctive radiotherapy, chemotherapy, and biological therapy may be required. The optimal therapy for splenic marginal zone lymphoma depends on the clinical presentation. The feasibility of surgery depends on the stage of splenic marginal zone lymphoma at diagnosis.

Pathophysiology

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

Causes

There are no established causes for splenic marginal zone lymphoma.

Differential Diagnosis

Splenic marginal zone lymphoma must be differentiated from other diseases such as chronic lymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma.

Epidemiology and demographics

The incidence of splenic marginal zone lymphoma increases with age; the median age at diagnosis is 50 years. Splenic marginal zone lymphoma affects men and women equally.

Risk Factors

There are no established risk factors for splenic marginal zone lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for splenic marginal zone lymphoma

Prognosis

Low Hemoglobin levels, high lactate dehydrogenase levels, low blood serum albumin levels, and genetic mutations such as mutations in NOTCH2 are associated with poor prognosis among patients with splenic marginal zone lymphoma.

Diagnosis

Staging

According to the Lugano classification, there are four stages of splenic marginal zone lymphoma based on the number of nodes and extranodal involvement.

Symptoms

The most common symptoms of splenic marginal zone lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.

Physical Examination

Common physical examination findings of splenic marginal zone lymphoma include fever, rash, ulcer, splenomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.

Laboratory tests

Laboratory tests for splenic marginal zone lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, genetic testing, FISH, PCR, and immunophenotyping.

Biopsy

Lymph node or extranodal tissue biopsy is diagnostic of splenic marginal zone lymphoma.

CT

CT scan may be helpful in the diagnosis of splenic marginal zone lymphoma.

MRI

MRI may be helpful in the diagnosis of splenic marginal zone lymphoma.

Ultrasound

Abdomen ultrasound may be helpful in the diagnosis of splenic marginal zone lymphoma. Findings on ultrasound abdomen suggestive of splenic marginal zone lymphoma include splenomegaly and lymphadenopathy.

Other Imaging Studies

PET scan may be helpful in the diagnosis of splenic marginal zone lymphoma.

Other Diagnostic Studies

Other diagnostic studies for splenic marginal zone lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy.

Treatment

Medical Therapy

The predominant therapy for splenic marginal zone lymphoma is surgery. Adjunctive radiotherapy, chemotherapy, and biological therapy may be required. The optimal therapy for splenic marginal zone lymphoma depends on the clinical presentation.

Surgery

The feasibility of surgery depends on the stage of splenic marginal zone lymphoma at diagnosis.