Anaplastic large cell lymphoma, ALK positive: Difference between revisions
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***Negative: [[CD15]], [[CD20]], Pax5/BSAP and [[EBV]]. | ***Negative: [[CD15]], [[CD20]], Pax5/BSAP and [[EBV]]. | ||
*'''Small cell pattern (8.3%)''': Present nuclear irregularity and perivascular/intravascular distribution<ref name="pmid8394652">{{cite journal| author=Kinney MC, Collins RD, Greer JP, Whitlock JA, Sioutos N, Kadin ME| title=A small-cell-predominant variant of primary Ki-1 (CD30)+ T-cell lymphoma. | journal=Am J Surg Pathol | year= 1993 | volume= 17 | issue= 9 | pages= 859-68 | pmid=8394652 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8394652 }} </ref>. Occasionally, this lymphomatous cells have pale cytoplasm with a central nucleus ("fried egg cell")<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>. | *'''Small cell pattern (8.3%)''': Present nuclear irregularity and perivascular/intravascular distribution<ref name="pmid8394652">{{cite journal| author=Kinney MC, Collins RD, Greer JP, Whitlock JA, Sioutos N, Kadin ME| title=A small-cell-predominant variant of primary Ki-1 (CD30)+ T-cell lymphoma. | journal=Am J Surg Pathol | year= 1993 | volume= 17 | issue= 9 | pages= 859-68 | pmid=8394652 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8394652 }} </ref>. Occasionally, this lymphomatous cells have pale cytoplasm with a central nucleus ("fried egg cell")<ref name=Swerdlow>{{cite book | last = Swerdlow | first = Steven | title = WHO classification of tumours of haematopoietic and lymphoid tissues | publisher = International Agency for Research on Cancer | location = Lyon, France | year = 2008 | isbn = 9789283224310 }}</ref>. | ||
*'''Giant cell pattern (3.3%)''' | *'''Giant cell pattern (3.3%)''' | ||
<i>One single patient may present more than one morphologic pattern in the same or progressive biopsies</i><ref name="pmid9490693">{{cite journal| author=Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ et al.| title=ALK-positive lymphoma: a single disease with a broad spectrum of morphology. | journal=Blood | year= 1998 | volume= 91 | issue= 6 | pages= 2076-84 | pmid=9490693 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9490693 }} </ref>. | <i>One single patient may present more than one morphologic pattern in the same or progressive biopsies</i><ref name="pmid9490693">{{cite journal| author=Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ et al.| title=ALK-positive lymphoma: a single disease with a broad spectrum of morphology. | journal=Blood | year= 1998 | volume= 91 | issue= 6 | pages= 2076-84 | pmid=9490693 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9490693 }} </ref>. | ||
Revision as of 21:19, 18 February 2015
Template:DiseaseDisorder infobox Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alberto Plate [2]
Synonyms and keywords: ALCL-ALK(+)
Overview
The Anaplastic large cell lymphoma ALK-positive (Anaplastic Llymphoma Kinase) consist of CD30-positive T-cells with abundant cytoplasm, a pleomorphic nucleus (horseshoe-shaped nucleus) and a eosinophilic paranuclear region[1]. This ALK-positive lymphoma has a translocation in the ALK gene [T(2;5)(p23;q35)], which will in turn, express the ALK protein[2].
Historical Perspective
Classification
Pathophysiology
ALCL morphologic features are variable, which allowed to classify this entity into five morphologic patterns[2]:
- "Common" pattern: Its the most common morphologic variant (75%)[3]. Cytoplasm may be basophilic or eosinophilic and the cell might have many nuclei with dispersed or clumped chromatin. In large cells, nucleoli tends to be more prominent. Given that the lymphomatous cells grow in the lymph node's sinuses, it may resemble a metastatic tumor.
- Lymphohistiocytic pattern (10%): histiocytes with acidophilic cytoplasm and a perinuclear clear area, which represents the Golgi apparatus. This cells have eccentric nuclei with condensed chromatin[4]. Lymphomatous cells tend tu cluster around the perivascular area, evidenced by immunostaining with CD30 and ALK antibodies[2].
- Hodgkin's like pattern (3.3%): Morphological characteristics reassemble Hodgkin's lymphoma, variant nodular sclerosis[5]. Predominates in female patients over male patients.
- Small cell pattern (8.3%): Present nuclear irregularity and perivascular/intravascular distribution[6]. Occasionally, this lymphomatous cells have pale cytoplasm with a central nucleus ("fried egg cell")[2].
- Giant cell pattern (3.3%)
One single patient may present more than one morphologic pattern in the same or progressive biopsies[1].
Causes
Differential Diagnosis
Epidemiology and Demographics
This entity affects primarily young, male patients[7] and accounts for 3% of all NHL, 40% of all large cell lymphomas[8] and 10%-20% of childhood lymphomas, although it is particularly incident in patients between 10 and 29 years[9].
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Treatment
References
- ↑ 1.0 1.1 Benharroch D, Meguerian-Bedoyan Z, Lamant L, Amin C, Brugières L, Terrier-Lacombe MJ; et al. (1998). "ALK-positive lymphoma: a single disease with a broad spectrum of morphology". Blood. 91 (6): 2076–84. PMID 9490693.
- ↑ 2.0 2.1 2.2 2.3 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.
- ↑ "Frequent Expression ofthe NPM-ALK Chimeric Fusion Protein inAnaplastic Large-Cell Lymphoma, Lympho-Histiocytic Type" (PDF).
- ↑ 5.0 5.1 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.
- ↑ 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.
- ↑ Stein H, Foss HD, Dürkop H, Marafioti T, Delsol G, Pulford K; et al. (2000). "CD30(+) anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features". Blood. 96 (12): 3681–95. PMID 11090048.
- ↑ "ALK+ Lymphoma: Clinico-Pathological Findings and Outcome".
- ↑ "ALK+ Lymphoma: Clinico-Pathological Findings and Outcome".