Splenic marginal zone lymphoma overview
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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.