Small cell lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: Small cell lymphocytic leukemia; SLL

Overview

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.[1] 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.

Historical Perspective

In 1924, small cell lymphoma was first discovered by the American physician Dr. George Minot.[1]

Classification

There is no classification system for small cell lymphoma.[2] Small cell lymphoma is a subtype of of B-cell chronic lymphocytic leukemia.

Pathophysiology

Pathogenesis

  • 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.

Genetics

Genes involved in the development of small cell lymphoma include:

Gross Pathology

There are no remarkable gross pathological findings in small cell lymphoma.

Microscopic Pathology

On microscopic histopathological analysis, characteristic findings of small cell lymphoma include:

On immunohistopathological analysis, characteristic findings of small cell lymphoma may include:

Causes

There are no established causes for small cell lymphoma.

Differentiating Small Cell Lymphoma from Other Diseases

Small cell lymphoma must be differentiated from other diseases that cause fever, anorexia, and weakness such as:[3]

Epidemiology and Demographics

Prevalence

The prevalence of small cell lymphoma is low. Small cell lymphoma represents 90 % of all B-cell chronic lymphocytic leukemias in the United States.[3]

Age

The median age at diagnosis is 65 years.

Gender

Males are more commonly affected with small cell lymphoma than females.

Race

There is no racial predilection for small cell lymphoma.

Risk Factors

The risk factors for the development of small cell lymphoma are not well established. The most consistent risk factor is a family history of non-hodgkins lymphoma.[4]

Natural History, Complications and Prognosis

Natural History

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.

Complications

Common complications of small cell lymphoma include:[3]

Prognosis

The prognosis of small cell lymphoma is generally poor; the 5-year survival rate of patients with small cell lymphoma is approximately 51-67%.[3]

Diagnosis

Symptoms

Symptoms of small cell lymphoma may include the following:

Physical Examination

Patients with small cell lymphoma often appear pale and malnourished. Physical examination may be remarkable for:

Laboratory Findings

Laboratory findings consistent with the diagnosis of small cell lymphoma include:[5]


Imaging Findings

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).

Treatment

Medical Therapy

The mainstay of therapy for small cell lymphoma is chemotherapy and radiotherapy.

Surgery

Surgery is not recommended among patients with small cell lymphoma.[3]

  • The diagnosis of small cell lymphoma is made with a excisional lymph node biopsy combined with a bone marrow biopsy.

Prevention

Primary Prevention

There are no primary preventive measures available for small cell lymphoma.

Secondary Prevention

Secondary prevention strategies following small cell lymphoma include follow up every 6 to 12 months. Follow-up testing includes a complete blood count and physical examination.

References

  1. 1.0 1.1 Rai KR (1993). "Progress in chronic lymphocytic leukaemia: a historical perspective.". Baillieres Clin Haematol. 6 (4): 757–65. PMID 8038488. 
  2. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL (Eds), IARC Press, Lyon 2008.
  3. 3.0 3.1 3.2 3.3 3.4 Schwock J, Geddie WR (2012). "Diagnosis of B-cell non-hodgkin lymphomas with small-/intermediate-sized cells in cytopathology". Patholog Res Int. 2012: 164934. PMC 3368210Freely accessible. PMID 22693682. doi:10.1155/2012/164934. 
  4. Slager SL, Benavente Y, Blair A, Vermeulen R, Cerhan JR, Costantini AS; et al. (2014). "Medical history, lifestyle, family history, and occupational risk factors for chronic lymphocytic leukemia/small lymphocytic lymphoma: the InterLymph Non-Hodgkin Lymphoma Subtypes Project.". J Natl Cancer Inst Monogr. 2014 (48): 41–51. PMC 4155456Freely accessible. PMID 25174025. doi:10.1093/jncimonographs/lgu001. 
  5. 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
  6. Nabhan C, Rosen ST (2014). "Chronic lymphocytic leukemia: a clinical review.". JAMA. 312 (21): 2265–76. PMID 25461996. doi:10.1001/jama.2014.14553. 
  7. Chronic Lymphocytic Leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/B-cell_chronic_lymphocytic_leukemia Accessed on October,12 2015

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