Anaplastic large cell lymphoma, ALK positive
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 cell, nucleoli tends to be more prominent.
- Lymphohistiocytic patter (10%)
- Small cell (8.3%)
- Giant cell (3.3%)
- Hodgkin's like pattern (3.3%)
Causes
Differential Diagnosis
Epidemiology and Demographics
This entity affects primarily young, male patients[4] and accounts for 3% of all NHL, 40% of all large cell lymphomas[5] and 10%-20% of childhood lymphomas, although it is particularly incident in patients between 10 and 29 years[6].
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Treatment
References
- ↑ 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 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.
- ↑ 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".