Anaplastic large cell lymphoma classification

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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 may be classified into several subtypes based on immunophenotype, clinical presentation and histology.

Classification

Anaplastic large cell lymphoma (peripheral T-cell lymphoma Non-Hodgkin Lymphoma) may be classified into 2 subtypes:

Based on clinical presentations, anaplastic large cell lymphoma may be classified into 2 subtypes:^[1]

Primary cutaneous anaplastic large cell lymphoma
Primary systemic anaplastic large cell lymphoma

Based on histology, anaplastic large cell lymphoma may be classified into 3 subtypes:

Classical Variants
Atypical Variants
Rare Variants

**Classification based on the clinical presentation**^[1]
Name	Description
Primary cutaneous anaplastic large cell lymphoma	Confined to the skin Usually have a single lump or tumor in the skin May also spread to lymph nodes in the area Associated with a rare skin condition called lymphomatoid papulosis Less aggressive than primary systemic anaplastic large cell lymphoma Occasionally individuals have a spontaneous remission Have a fairly good prognosis
Primary systemic anaplastic large cell lymphoma	Usually involves the lymph nodes Can also occur in organs or tissues other than the lymph nodes (extranodal sites), including: Lungs, liver, bone marrow, bone, gastrointestinal tract, skin, and soft tissue Most individuals have advanced stage (stage III or IV) disease when they are diagnosed Usually a fast-growing (aggressive) lymphoma

**Histologic Classification** ^[2]^[3]
Name	Description
Classical Variants
Common pattern	ALK positive anaplastic large cell lymphoma Most common morphological variant (75%)^[4] In large cells, nucleoli tend to be more prominent The cytoplasm may be either basophilic or eosinophilic and the cell might have many nuclei with dispersed or clumped chromatin Given that the lymphomatous cells grow in the lymph node's sinuses, this variant may resemble a metastatic tumor
Atypical Variants
Small cell	ALK positive anaplastic large cell lymphoma Cells have nuclear irregularity and perivascular/intravascular distribution^[5] Occasionally, lymphomatous cells have a pale cytoplasm with a central nucleus, described as "fried egg cell"^[3]
Lymphohistiocytic	ALK positive anaplastic large cell lymphoma Histiocytes have an acidophilic cytoplasm and a perinuclear clear area, with an eccentric nuclei and condensed chromatin^[6] Lymphomatous cells cluster around the perivascular area as demonstrated by immunostaining with CD30 and ALK antibodies^[3]
Giant cell	ALK positive anaplastic large cell lymphoma
Hodgkin's like	The morphological characteristics of this pattern are similar to the nodular sclerosis variant of Hodgkin's lymphoma^[7] This pattern is predominately more common among female There are two immunophenotype:^[7] Positive: CD30, ALK, epithelial membrane antigen (EMA), CD43 (only 66% of the times), and perforin Negative: CD15, CD20, Pax5/BSAP, and EBV
Rare Variants
Sarcomatoid	ALK positive anaplastic large cell lymphoma

References

↑ ^1.0 ^1.1 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
↑ The anaplastic lymphoma kinase in the pathogenesis of cancer. http://go.galegroup.com/ps/retrieve.dosgHitCountType=None&sort=RELEVANCE&inPS=true&prodId=HRCA&userGroupName=mlin_b_bethidmc&tabID=T002&searchId=R1&resultListType=RESULT_LIST&contentSegment=&searchType=AdvancedSearchForm&currentPosition=1&contentSet=GALE%7CA188154738&&docId=GALE Accessed on October 8, 2015
↑ ^3.0 ^3.1 ^3.2 Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
↑ Falini B, Bigerna B, Fizzotti M, Pulford K, Pileri SA, Delsol G; et al. (1998). "ALK expression defines a distinct group of T/null lymphomas ("ALK lymphomas") with a wide morphological spectrum". Am J Pathol. 153 (3): 875–86. doi:10.1016/S0002-9440(10)65629-5. PMC 1853018. PMID 9736036.
↑ Kinney MC, Collins RD, Greer JP, Whitlock JA, Sioutos N, Kadin ME (1993). "A small-cell-predominant variant of primary Ki-1 (CD30)+ T-cell lymphoma". Am J Surg Pathol. 17 (9): 859–68. PMID 8394652.
↑ "Frequent Expression ofthe NPM-ALK Chimeric Fusion Protein inAnaplastic Large-Cell Lymphoma, Lympho-Histiocytic Type" (PDF).
↑ ^7.0 ^7.1 Vassallo J, Lamant L, Brugieres L, Gaillard F, Campo E, Brousset P; et al. (2006). "ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin's lymphoma: report of 10 cases". Am J Surg Pathol. 30 (2): 223–9. PMID 16434897.

Template:WH Template:WS

[canadian.cancer-1] 1.0 ^1.1 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] The anaplastic lymphoma kinase in the pathogenesis of cancer. http://go.galegroup.com/ps/retrieve.dosgHitCountType=None&sort=RELEVANCE&inPS=true&prodId=HRCA&userGroupName=mlin_b_bethidmc&tabID=T002&searchId=R1&resultListType=RESULT_LIST&contentSegment=&searchType=AdvancedSearchForm&currentPosition=1&contentSet=GALE%7CA188154738&&docId=GALE Accessed on October 8, 2015

[Swerdlow-3] 3.0 ^3.1 ^3.2 Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.

[pmid9736036-4] Falini B, Bigerna B, Fizzotti M, Pulford K, Pileri SA, Delsol G; et al. (1998). "ALK expression defines a distinct group of T/null lymphomas ("ALK lymphomas") with a wide morphological spectrum". Am J Pathol. 153 (3): 875–86. doi:10.1016/S0002-9440(10)65629-5. PMC 1853018. PMID 9736036.

[pmid8394652-5] Kinney MC, Collins RD, Greer JP, Whitlock JA, Sioutos N, Kadin ME (1993). "A small-cell-predominant variant of primary Ki-1 (CD30)+ T-cell lymphoma". Am J Surg Pathol. 17 (9): 859–68. PMID 8394652.

[6] "Frequent Expression ofthe NPM-ALK Chimeric Fusion Protein inAnaplastic Large-Cell Lymphoma, Lympho-Histiocytic Type" (PDF).

[pmid16434897-7] 7.0 ^7.1 Vassallo J, Lamant L, Brugieres L, Gaillard F, Campo E, Brousset P; et al. (2006). "ALK-positive anaplastic large cell lymphoma mimicking nodular sclerosis Hodgkin's lymphoma: report of 10 cases". Am J Surg Pathol. 30 (2): 223–9. PMID 16434897.

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Anaplastic large cell lymphoma classification

Overview

Classification

References

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