Anaplastic large cell lymphoma overview

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Anaplastic large cell lymphoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

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Laboratory Findings

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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

Anaplastic large cell lymphoma is an aggressive (fast-growing) type of non-Hodgkin lymphoma that is usually of the T-cell type. ALK negative ALCL T-cells express CD30, but not the ALK (Anaplastic Lymphoma Kinase) chimeric protein. Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the peripheral T-cell lymphoma classification. Anaplastic large cell lymphoma may be classified into several subtypes based on immunophenotype.

Historical Perspective

Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+ LBCL) was first described in 1997 by Delsol. In 2008, the World Health Organization (WHO) added anaplastic lymphoma kinase (ALK) negative anaplastic large cell lymphoma and anaplastic lymphoma kinase positive large B-cell lymphoma as provisional entities in the peripheral T-cell lymphoma classification.

Classification

Anaplastic large cell lymphoma may be classified into several subtypes based on immunophenotype, clinical presentation, and histology.

Pathophysiology

The ALK gene is involved in the pathogenesis of ALK positive anaplastic large cell lymphoma. The DUSP22 gene is involved in the pathogenesis of ALK negative anaplastic large cell lymphoma. On microscopic histopathological analysis, medium sized cells, abundant cytoplasm, kidney shaped nuclei, and paranuclear eosinophilic region are characteristic findings of anaplastic large cell lymphoma.

Causes

There are no established causes for anaplastic large cell lymphoma.

Differential Diagnosis

Anaplastic large cell lymphoma must be differentiated from other diseases such as metastatic carcinoma, B cell lymphoma, primary cutaneous T-cell lymphoma, rhabdomyosarcoma, peripheral T-cell lymphoma-not otherwise specified, classical Hodgkin's lymphoma, and diffuse large B cell lymphoma.

Epidemiology and Demographics

Anaplastic large cell lymphoma is a common disease that tends to affect children or young adults. Males are more commonly affected with anaplastic large cell lymphoma than females.[1]

Risk Factors

There are no established risk factors for anaplastic large cell lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for anaplastic large cell lymphoma.

Prognosis

The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%.

Diagnosis

Staging

According to the Lugano classification, there are four stages of anaplastic large cell lymphoma based on the number of nodes and extranodal involvement.

Symptoms

The most common symptoms of anaplastic cell 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 mantle cell lymphoma include fever, rash, ulcer, splenomegaly, hepatomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[2]

Laboratory tests

Laboratory tests for anaplastic large cell lymphoma include complete blood count (CBC), blood chemistry studies, flow cytometry, FISH, immunohistochemistry, and immunophenotyping.[3][4][5]

Chest X ray

Chest X ray may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on chest X ray, suggestive of anaplastic large cell lymphoma include pulmonary nodules and pleural effusion.

CT Scan

Thorax CT scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on CT scan suggestive of anaplastic large cell lymphoma include mediastinal lymphadenopathy and bilateral pleural effusion.

MRI Scan

Thorax MRI scan may be helpful in the diagnosis of anaplastic large cell lymphoma. Findings on MRI scan suggestive of anaplastic large cell lymphoma include large heterogenous, lobulated mass and lymphadenopathy.

Biopsy

Lymph node or extranodal tissue biopsy is diagnostic of anaplastic large cell lymphoma.

Ultrasound

Abdomen and shoulder ultrasound may be helpful in the diagnosis of anaplastic large cell lymphoma.

Other Imaging Studies

PET scan may be helpful in the diagnosis of anaplastic large cell lymphoma.

Other Diagnostic Studies

Other diagnostic studies for anaplastic large cell lymphoma include laparoscopy, laparotomy, bone marrow aspiration, and bone marrow biopsy.

Treatment

Medical Therapy

The predominant therapy for anaplastic lymphoma is chemotherapy. Adjunctive radiotherapy, stem cell transplantation, and surgery may be required. The optimal therapy for anaplastic large cell lymphoma depends on the type and extent of anaplastic large cell lymphoma.

Surgery

The feasibility of surgery depends on the type of anaplastic large cell lymphoma.

References

  1. Anaplastic large cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/anaplastic-large-cell-lymphoma/?region=on Accessed on October 7, 2015
  2. Anaplastic large cell lymphoma, ALK-positive. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52fd/ Accessed on October 16, 2015
  3. "ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project".
  4. Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma: An Underrecognized Aggressive Lymphoma. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2012/529572/#B1 Accessed on October 13, 2015
  5. Dovepress. Anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma with involvement of the urinary bladder: a case report and review of literature. https://www.dovepress.com/anaplastic-lymphoma-kinase-positive-anaplastic-large-cell-lymphoma-wit-peer-reviewed-article-OTT Accessed on October 13, 2015

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