Small cell lymphoma
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Synonyms and Keywords: Small cell lymphocytic leukemia; SLL
Small cell lymphoma (also known as SLL) is a type of mature B-cell chronic lymphocytic leukemia that presents primarily in the lymph nodes. In 1924, small cell lymphoma was first discovered by the American physician Dr. George Minot. There is no standardized classification system for small cell lymphoma. The pathogenesis of small cell lymphoma is characterized by the malignant infiltration of lymphocytic cells into the bone marrow where they interfere with the production of other hematopoeitic cell lines such as red blood cells and platelets. Small cell lymphoma arises from pre-follicular center B cells that are normally involved in the process of human immunoglobulins production. Small cell lymphoma represents 90 % of all B-cell chronic lymphocytic leukemias in the United States. Small cell lymphoma presents at a median age of 65 years. Males are slightly more affected with small cell lymphoma than females. Symptoms of small cell lymphoma may include fever,weight loss, generalized weakness, anorexia, and night sweats. The mainstay therapy of small cell lymphoma is chemotherapy and radiotherapy.
In 1924, small cell lymphoma was first discovered by the American physician Dr. George Minot.
- The pathogenesis of small cell lymphoma is characterized by the malignant infiltration of lymphocytic cells into the bone marrow, where they interfere with the production of red blood cells and platelets.
- Small cell lymphoma arises from pre-follicular center B cells that are normally involved in the process of human immunoglobulins production.
Genes involved in the development of small cell lymphoma include:
- Chromosome 11 long arm deletion
- Chromosome 13 long arm deletion (most common genetic mutation)
- Chromosome 17 short arm deletion
- Trisomy 12
There are no remarkable gross pathological findings in small cell lymphoma.
On microscopic histopathological analysis, characteristic findings of small cell lymphoma include:
- Small lymphoid cells
- Thin cytoplasmic border
- Dense nucleus
- Lack of nucleolus
- Clumped chromatin aggregates
- The presence of smudge cells
- The presence of gumprecht nuclear shadows
On immunohistopathological analysis, characteristic findings of small cell lymphoma may include:
There are no established causes for small cell lymphoma.
Differentiating Small Cell Lymphoma from Other Diseases
Epidemiology and Demographics
The median age at diagnosis is 65 years.
Males are more commonly affected with small cell lymphoma than females.
There is no racial predilection for small cell lymphoma.
Natural History, Complications and Prognosis
The majority of patients with small cell lymphoma are symptomatic. Early clinical features include generalized weakness, anorexia, and night sweats. If left untreated, patients with small cell lymphoma may progress to develop recurrent infections.
Common complications of small cell lymphoma include:
The prognosis of small cell lymphoma is generally poor; the 5-year survival rate of patients with small cell lymphoma is approximately 51-67%.
Symptoms of small cell lymphoma may include the following:
Patients with small cell lymphoma often appear pale and malnourished. Physical examination may be remarkable for:
- Leukemia cutis (infiltration of neoplastic leukocytes or their precursors into the skin resulting in clinically identifiable cutaneous lesions)
- Enlarged and palpable cervical lymph nodes
Laboratory findings consistent with the diagnosis of small cell lymphoma include:
- Absolute lymphocytosis (>5000 cells/μl)
- Decreased hemoglobin concentration
- Decreased platelets count
There are no specific imaging findings associated with small cell lymphoma. CT scan may be helpful in the diagnosis small cell lymphoma, findings may include enlarged lymph nodes (>2 cm).
The mainstay of therapy for small cell lymphoma is chemotherapy and radiotherapy.
Surgery is not recommended among patients with small cell lymphoma.
- The diagnosis of small cell lymphoma is made with a excisional lymph node biopsy combined with a bone marrow biopsy.
There are no primary preventive measures available for small cell lymphoma.
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- Review Article: Diagnostic Workup of Small B Cell Lymphomas: A Laboratory Perspective http://www.hindawi.com/journals/lymph/2012/346084/A Accessed on May 4, 2016
- Nabhan C, Rosen ST (2014). "Chronic lymphocytic leukemia: a clinical review". JAMA. 312 (21): 2265–76. doi:10.1001/jama.2014.14553. PMID 25461996.
- Chronic Lymphocytic Leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/B-cell_chronic_lymphocytic_leukemia Accessed on October,12 2015