Anaplastic large cell lymphoma
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| Anaplastic large cell lymphoma Classification and external resources | |
| ICD-O: | M9714/3 |
|---|---|
| eMedicine | derm/534 |
| MeSH | D017728 |
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Overview
Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin lymphoma that features in the World Health Organisation (WHO) classification of lymphomas.
Diagnosis
To make this diagnosis under its present system of classification, the WHO:
Requires
- The presence of "hallmark" cells
- Immunopositivity for CD30
Acknowledges as typical, but does not require
- Immunopositivity for ALK (anaplastic lymphoma kinase) protein
Specifically excludes
- Primary cutaneous T-cell lymphomas
- Other specific types of anaplastic lymphoma (particularly those of B-cell lineage) with CD30 positivity
The hallmark cells are of medium size and feature abundant cytoplasm (which may be clear, amphophilic or eosinophilic), kidney shaped nuclei, and a paranuclear eosinophilic region. Occasional cells may be identified in which the plane of section passes through the nucleus in such a way that it appears to enclose a region of cytoplasm within a ring; such cells are called "doughnut" cells.
By definition, on histological examination, hallmark cells are always present. Where they are not present in large numbers, they are usually located around blood vessels. Morphologic variants include the following types:
- Common (featuring a predominance of hallmark cells)
- Small cell (featuring smaller cells with the same immunophenotype as the hallmark cells)
- Lymphohistiocytic
- Sarcomatoid
- Signet ring
Clinical features
The lymphoma is more common in the young and in males. It occurs in both nodal and extranodal locations. It typically presents at a late stage and is often associated with systemic symptoms ("B symptoms"). During treatment, relapses may occur but these typically remain sensitive to chemotherapy.
Immunophenotype
The hallmark cells (and variants) show immunopositivity for CD30 (also known as Ki-1). True positivity requires localisation of signal to the cell membrane and/or paranuclear region (cyptolasmic positivity is considered non-specific and non-informative). Another useful marker which helps to differentiate this lesion from Hodgkin lymphoma is Clusterin. The neoplastic cells have a golgi staining pattern (hence paranuclear staining), which is characteristic of this lymphoma. The cells are also typically positive for a subset of markers of T-cell lineage. However, as with other T-cell lymphomas, they are usually negative for the pan T-cell marker CD3. Occasional examples are of null (neither T nor B) cell type. These lymphomas show immunopositivity for ALK protein in 70% of cases. They are also typically positive for EMA. In contrast to many B-cell anaplastic CD30 positive lymphomas, they are negative for markers of Epstein-Barr Virus (EBV).
Molecular biology
The majority of cases, greater than 90%, contain a clonal rearrangement of the T-cell receptor. This may be identified using PCR techniques, such as T-gamma multiplex PCR. Oncogenetic potential is conferred by upregulation of a tyrosine kinase gene on chromosome 2. Several different translocations involving this gene have been identified in different cases of this lymphoma. The most common is a chromosomal translocation involving the nucleophosmin gene on chromosome 5. The translocation may be identified by analysis of giemsa-banded metaphase spreads of tumour cells and is characterised by t(2;5)(p23;q35). The product of this fusion gene may be identified by immunohistochemistry using antiserum to ALK protein. Probes are available to identify the translocation by fluorescent in situ hybridization. The nucleophosmin component associated with the commonest translocation results in nuclear positivity as well as cytoplasmic positivity. Positivity with the other translocations may be confined to the cytoplasm.
Differential diagnosis and diagnostic pitfalls
As the appearance of the hallmark cells, pattern of growth (nesting within lymph nodes) and positivity for EMA may mimic metastatic carcinoma, it is important to include markers for cytokeratin in any diagnostic panel (these will be negative in the case of anaplastic lymphoma). Other mimics include CD30 positive B-cell lymphomas with anaplastic cells (including Hodgkin lymphomas). These are identified by their positivity for markers of B-cell lineage and frequent presence of markers of EBV. Primary cutaneous T-cell lymphomas may also be positive for CD30; these are excluded by their anatomic distribution. ALK positivity may also be seen in some large cell B-cell lymphomas and occasionally in rhabdomyosarcomas.
Prognostic factors
Those with ALK positivity have a better prognosis. It is possible that ALK-negative anaplastic large cell lymphomas represent other T-cell lymphomas that are morphologic mimics of ALCL in a final common pathway of disease progression. It is possible that existing systems of classification will be revised in the future to exclude such lymphomas from this specific diagnosis.
Treatment Overview
- Managed under "Aggressive Lymphoma" guidelines
- CHOP is first line of treatment, CHOP-Rituxan in the unlikely scenario that CD20 is positive, given that CD20 is a B-cell marker.
- Radiation therapy as per institutional preference (based on ECOG, SWOG, and GELA trials), but usually added for bulky disease
- Overall better prognosis than other "Aggressive Lymphomas"
- ALK+ 5-year survival 70-80%
- ALK- 5-year survival 30-40%
External links
WikiDoc Research Resources for Anaplastic large cell lymphoma | |
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| Articles on Anaplastic large cell lymphoma | Most recent articles on Anaplastic large cell lymphoma • Most cited articles on Anaplastic large cell lymphoma • Review articles on Anaplastic large cell lymphoma • Articles on Anaplastic large cell lymphoma in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Anaplastic large cell lymphoma | Powerpoint slides on Anaplastic large cell lymphoma • Images of Anaplastic large cell lymphoma • Photos of Anaplastic large cell lymphoma • Podcasts & MP3s on Anaplastic large cell lymphoma • Videos on Anaplastic large cell lymphoma |
| Evidence Based Medicine Regarding Anaplastic large cell lymphoma | Cochrane Collaboration on Anaplastic large cell lymphoma • Bandolier on Anaplastic large cell lymphoma • TRIP on Anaplastic large cell lymphoma |
| Cost Effectiveness of Anaplastic large cell lymphoma | Cost Effectiveness of Anaplastic large cell lymphoma |
| Clinical Trials Involving Anaplastic large cell lymphoma | Ongoing Trials on Anaplastic large cell lymphoma at Clinical Trials.gov • Trial results on Anaplastic large cell lymphoma • Clinical Trials on Anaplastic large cell lymphoma at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Anaplastic large cell lymphoma | US National Guidelines Clearinghouse on Anaplastic large cell lymphoma • NICE Guidance on Anaplastic large cell lymphoma • NHS PRODIGY Guidance • FDA on Anaplastic large cell lymphoma • CDC on Anaplastic large cell lymphoma |
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| Genetics, Pharmacogenomics, and Proteinomics of Anaplastic large cell lymphoma | Genetics of Anaplastic large cell lymphoma • Pharmacogenomics of Anaplastic large cell lymphoma • Proteomics of Anaplastic large cell lymphoma |
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| Healthcare Provider Resources on Anaplastic large cell lymphoma | Symptoms of Anaplastic large cell lymphoma • Causes & Risk Factors for Anaplastic large cell lymphoma • Diagnostic studies for Anaplastic large cell lymphoma • Treatment of Anaplastic large cell lymphoma |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
