NK/T-cell lymphomas in children

Abstract

In children, T and NK-cell lymphomas are uncommon in Western Countries. While there has been significant experience treating T-cell lymphoblastic lymphoma (T-LBL) and anaplastic large cell lymphoma (ALCL), other subtypes are very rarely encountered and there are no standard approaches to their management. There are many challenges in defining optimal therapy for many of these diseases but recent progress in elucidating their biology has led to new molecular insights and identified interesting targets for novel drug discovery. In this review, we discuss these disorders in children, how they are approached therapeutically and what lies on the horizon with respect to novel treatment approaches.

When a Lump is More Than Just a Lump: Anaplastic Large Cell Lymphoma Presenting as a Pediatric Breast Mass

“…Leukemic pictures and neurological involvement are rare modes of presentation 5. ALCL can also be associated with rashes and hemophagocytic lymphohistiocytosis 8…”

“…5 ALCL can also be associated with rashes and hemophagocytic lymphohistiocytosis. 8 Systemic ALCLs are classified by ALK positivity or lack thereof. Although more than 90% of pediatric cases are ALK +1 and have a relatively favorable prognosis, 8 ALK − ALCL has a median age of presentation of 65 years and carries a worse prognosis.…”

“…8 Systemic ALCLs are classified by ALK positivity or lack thereof. Although more than 90% of pediatric cases are ALK +1 and have a relatively favorable prognosis, 8 ALK − ALCL has a median age of presentation of 65 years and carries a worse prognosis. 6 Unlike ALK + ALCL, which has been widely studied, ALK − ALCL is less understood.…”

Linfoma NK/T extra nodal, tipo nasal, con compromiso cutáneo primario. Reporte de un caso

“…El linfoma extranodal NK/T tiene una presentación clínica similar tanto en niños como en adultos, la enfermedad tiende a tener un predominio en hombres, la mayoría de los casos son diagnosticados en etapa precoz y frecuentemente se acompañan de síntomas B (baja de peso, sudoración nocturna y/o fiebre) y niveles de lactato deshidrogenasa (LDH) elevados. El más común de los linfomas extranodales es NK/T nasal, en el que el tumor puede causar destrucción ósea alrededor de los senos, el septum nasal u obstrucción de la vía aérea (4,5) , manifestándose con síntomas de obstrucción nasal, epistaxis y/o masa que comprometa la nariz, los senos o el paladar (1) . El linfoma extranodal NK/T de tipo nasal, se caracteriza por una ubicación primaria extra nasal (1) , el más frecuente de los compromisos extranasales es la piel, otros compromisos pueden ser vía aérea superior, el anillo linfático de waldeyer, tracto gastrointestinal, testículos, pulmón, ojos y partes blandas.…”

Panniculitis-like T-cell lymphoma in children

“…Около 10% всех случаев неходжкинских лимфом у детей приходится на периферические Т-клеточные лимфомы [2]. Самое частое заболевание в данной группе -анапластическая крупноклеточная лимфома, другие составляют всего 1-2% [3,4]. Редкость и неоднородность этих заболеваний являются основными причинами отсутствия четких протоколов и стратегий лечения.…”