Small cell lymphoma

WikiDoc Resources for Small cell lymphoma

Articles

Most recent articles on Small cell lymphoma

Most cited articles on Small cell lymphoma

Review articles on Small cell lymphoma

Articles on Small cell lymphoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Small cell lymphoma

Images of Small cell lymphoma

Photos of Small cell lymphoma

Podcasts & MP3s on Small cell lymphoma

Videos on Small cell lymphoma

Evidence Based Medicine

Cochrane Collaboration on Small cell lymphoma

Bandolier on Small cell lymphoma

TRIP on Small cell lymphoma

Clinical Trials

Ongoing Trials on Small cell lymphoma at Clinical Trials.gov

Trial results on Small cell lymphoma

Clinical Trials on Small cell lymphoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Small cell lymphoma

NICE Guidance on Small cell lymphoma

NHS PRODIGY Guidance

FDA on Small cell lymphoma

CDC on Small cell lymphoma

Books

Books on Small cell lymphoma

News

Small cell lymphoma in the news

Be alerted to news on Small cell lymphoma

News trends on Small cell lymphoma

Commentary

Blogs on Small cell lymphoma

Definitions

Definitions of Small cell lymphoma

Patient Resources / Community

Patient resources on Small cell lymphoma

Discussion groups on Small cell lymphoma

Patient Handouts on Small cell lymphoma

Directions to Hospitals Treating Small cell lymphoma

Risk calculators and risk factors for Small cell lymphoma

Healthcare Provider Resources

Symptoms of Small cell lymphoma

Causes & Risk Factors for Small cell lymphoma

Diagnostic studies for Small cell lymphoma

Treatment of Small cell lymphoma

Continuing Medical Education (CME)

CME Programs on Small cell lymphoma

International

Small cell lymphoma en Espanol

Small cell lymphoma en Francais

Business

Small cell lymphoma in the Marketplace

Patents on Small cell lymphoma

Experimental / Informatics

List of terms related to Small cell lymphoma

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[edit | edit source]

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[edit | edit source]

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

## Classification[edit | edit source]

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

## Pathophysiology[edit | edit source]

### Pathogenesis[edit | edit source]

* 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[edit | edit source]

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

### Gross Pathology[edit | edit source]

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

### Microscopic Pathology[edit | edit source]

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:

* CD19
* CD20
* CD23
* CD5

## Causes[edit | edit source]

There are no established causes for small cell lymphoma.

## Differentiating Small Cell Lymphoma from Other Diseases[edit | edit source]

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

* Anemia
* Plasma cell neoplasm
* Hairy cell leukemia
* Mantle cell lymphoma

## Epidemiology and Demographics[edit | edit source]

### Prevalence[edit | edit source]

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[edit | edit source]

The median age at diagnosis is 65 years.

### Gender[edit | edit source]

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

### Race[edit | edit source]

There is no racial predilection for small cell lymphoma.

## Risk Factors[edit | edit source]

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[edit | edit source]

### Natural History[edit | edit source]

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[edit | edit source]

Common complications of small cell lymphoma include:[3]

* Severe immunodeficiency
* Warm autoimmune hemolytic anemia
* Richter's transformation

### Prognosis[edit | edit source]

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[edit | edit source]

### Symptoms[edit | edit source]

Symptoms of small cell lymphoma may include the following:

* Fever
* Weight loss
* Muscle wasting
* Generalized weakness
* Anorexia
* Night sweats

### Physical Examination[edit | edit source]

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

* Pallor
* Petechiae
* Leukemia cutis (infiltration of neoplastic leukocytes or their precursors into the skin resulting in clinically identifiable cutaneous lesions)
* Enlarged and palpable cervical lymph nodes
* Dacryoadenitis

### Laboratory Findings[edit | edit source]

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

* Absolute lymphocytosis (>5000 cells/μl)[6][7]
* Decreased hemoglobin concentration
* Decreased platelets count

### Imaging Findings[edit | edit source]

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[edit | edit source]

### Medical Therapy[edit | edit source]

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

### Surgery[edit | edit source]

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[edit | edit source]

#### Primary Prevention[edit | edit source]

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

#### Secondary Prevention[edit | edit source]

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[edit | edit source]

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. doi:10.1155/2012/164934. PMC 3368210. PMID 22693682.
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. doi:10.1093/jncimonographs/lgu001. PMC 4155456. PMID 25174025.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
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. doi:10.1001/jama.2014.14553. PMID 25461996.
7. ↑ Chronic Lymphocytic Leukemia. Wikipedia (2015) https://en.wikipedia.org/wiki/B-cell_chronic_lymphocytic_leukemia Accessed on October,12 2015

Template:WH Template:WS