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anaplastic large cell lymphoma (ALCL), CD30+, T-cell type

Etiology: - rare complication of textured breast implants [2] Epidemiology: - ~3% of adult non-Hodgkin's lymphomas - ~10-30% of childhood lymphomas - male predominance Microscopic Pathology: - ALCL, common variant ~70% - ALCL, lymphohistiocytic variant ~10% - ALCL, small cell variant ~5-10% Immunophenotype: - CD30+ - ALK+ (most cases) Genetics: - clonal rearrangement of T cell receptor ~90% - EBV sequences absent - chromosomal translocation involving ALK, most commonly t(2;5) involving nucleophosmin, others involving tropomyosin 3, Trk fused gene, clathrin heavy chain & ATIC (gene for 5-aminomidazole-4-carboxamide-ribonucleotide transformylase-IMP cyclohydrolase) - chromosomal translocation t(2;17)(p23;q25) involving ALO17 with ALK Clinical manifestations: - ~70% present with stage III or IV disease, peripheral &/or abdominal adenopathy often with extranodal infiltrates & bone marrow involvement. - ~75% show B symptoms, especially high fever

Interactions

disease interactions

Related

ALK tyrosine kinase receptor; anaplastic lymphoma kinase; CD246 (ALK) nucleophosmin; NPM; nucleolar phosphoprotein B23; numatrin; nucleolar protein NO38; nucleoplasmin 1 (NPM1, NPM)

Specific

anaplastic large cell lymphoma T-cell type/t[2/5]2

General

peripheral T-cell lymphoid neoplasm

Database Correlations

OMIM 105590 MORBIDMAP 105590

References

  1. WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. Jaffe et al. eds. IARC Press 2001
  2. Harrison P ALCL Increasing Rapidly in US Women as Breast Implants Rise. Medscape. July 21, 2022 https://www.medscape.com/viewarticle/977675 - Kinslow CJ, Kim A, Sanchez GI et al Incidence of Anaplastic Large-Cell Lymphoma of the Breast in the US, 2000 to 2018. JAMA Oncol. Published online July 21, 2022 PMID: 35862042 https://jamanetwork.com/journals/jamaoncology/article-abstract/2794631