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{{DiseaseDisorder infobox |
{{Anaplastic large cell lymphoma}}
  Name          = {{PAGENAME}} |
{{Patient}}
  ICD10          = |
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  ICDO          = {{ICDO|9714|3}} |
  Image          = |
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  OMIM          = |
  MedlinePlus    = |
  eMedicineSubj  = derm |
  eMedicineTopic = 534 |
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  MeshID        = D017728 |
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{{SI}}
{{CMG}}


{{CMG}}; {{AE}} {{UA}} {{AP}} {{AS}}; {{GRR}} {{Nat}}


{{SK}}: ALCL; Anaplastic large-cell lymphoma; Primary cutaneous anaplastic large cell lymphoma; Primary systemic  anaplastic large cell lymphoma; Anaplastic large cell lymphoma, ALK positive; Anaplastic large cell lymphoma, ALK negative; ALCL-ALK(+); ALCL-ALK(-); ALK positive ALCL; ALK negative ALCL


==Overview==
==[[Anaplastic large cell lymphoma overview|Overview]]==


'''Anaplastic large cell lymphoma''' (ALCL) is a type of [[non-Hodgkin lymphoma]] that features in the World Health Organisation ([[WHO]]) classification of [[lymphoma]]s.
==[[Anaplastic large cell lymphoma historical perspective|Historical Perspective]]==


===Diagnosis===
==[[Anaplastic large cell lymphoma classification|Classification]]==
To make this diagnosis under its present system of classification, the WHO:


Requires
==[[Anaplastic large cell lymphoma pathophysiology|Pathophysiology]]==
*The presence of "hallmark" cells
*Immunopositivity for [[CD30]]


Acknowledges as typical, but does not require
==[[Anaplastic large cell lymphoma causes|Causes]]==
*Immunopositivity for ALK (anaplastic lymphoma kinase) protein


Specifically excludes
==[[Anaplastic large cell lymphoma differential diagnosis|Differentiating Anaplastic large cell lymphoma from Other Diseases]]==
*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 [[cell nucleus|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.
==[[Anaplastic large cell lymphoma epidemiology and demographics|Epidemiology and Demographics]]==


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:
==[[Anaplastic large cell lymphoma risk factors|Risk Factors]]==
*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===
==[[Anaplastic large cell lymphoma screening|Screening]]==
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===
==[[Anaplastic large cell lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
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 ===
==Diagnosis==
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 [[Gene fusion|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.
[[Anaplastic large cell lymphoma staging|Staging]] | [[Anaplastic large cell lymphoma history and symptoms|History and Symptoms]] | [[Anaplastic large cell lymphoma physical examination|Physical Examination]] | [[Anaplastic large cell lymphoma laboratory findings|Laboratory Findings]] | [[Anaplastic large cell lymphoma chest x ray|Chest X Ray]] | [[Anaplastic large cell lymphoma CT|CT]] | [[Anaplastic large cell lymphoma MRI|MRI]] | [[Anaplastic large cell lymphoma biopsy|Biopsy]] | [[Anaplastic large cell lymphoma ultrasound|Ultrasound]] | [[Anaplastic large cell lymphoma other imaging findings|Other Imaging Findings]] | [[Anaplastic large cell lymphoma other diagnostic studies|Other Diagnostic Studies]]


=== Differential diagnosis and diagnostic pitfalls ===
==Treatment==
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 [[rhabdomyosarcoma]]s.
[[Anaplastic large cell lymphoma medical therapy|Medical Therapy]] | [[Anaplastic large cell lymphoma surgery|Surgery]] | [[Anaplastic large cell lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Anaplastic large cell lymphoma future or investigational therapies|Future or Investigational Therapies]]


===Prognostic factors===
==Case Studies==
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.
[[Anaplastic large cell lymphoma case study one|Case #1]]
 
=== 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==
* [http://www.lymphomainfo.net/nhl/types/alcl.html Lymphoma Information Network]


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{{Hematological malignancy histology}}
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Latest revision as of 01:55, 21 March 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Umar Ahmad, M.D.[2] Alberto Plate [3] Sowminya Arikapudi, M.B,B.S. [4]; Grammar Reviewer: Natalie Harpenau, B.S.[5]

Synonyms and keywords:: ALCL; Anaplastic large-cell lymphoma; Primary cutaneous anaplastic large cell lymphoma; Primary systemic anaplastic large cell lymphoma; Anaplastic large cell lymphoma, ALK positive; Anaplastic large cell lymphoma, ALK negative; ALCL-ALK(+); ALCL-ALK(-); ALK positive ALCL; ALK negative ALCL

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

Staging | History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Biopsy | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

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