ALK positive lymphohistiocytic variant of anaplastic large cell lymphoma in an adult.
Haematologica
; 86(3): 260-5, 2001 Mar.
Article
em En
| MEDLINE
| ID: mdl-11255272
ABSTRACT
BACKGROUND AND OBJECTIVES:
The lymphohistiocytic (LH) variant of anaplastic large cell lymphoma (ALCL) has, for a long time, been considered typical of children and adolescents. The aim of this study is a detailed characterization of a case of this peculiar ALCL subtype affecting an adult patient. DESIGN ANDMETHODS:
A 36-year old male presented with diffuse adenopathy and systemic symptoms (high fever, anorexia, asthenia); a diagnosis of CD30+/ALK+ ALCL, LH variant, was morphologically suspected and corroborated by immunohistochemistry that was crucial for the definitive diagnosis and subtyping.RESULTS:
The neoplastic population consisted of cells highly variable in size and shape but more often isolated and largely obscured by a predominant reactive cellular infiltrate of histiocytes and plasma cells. The lymphoma cells exhibited a null non-B non-T antigenic profile, but reacted strongly for the Ber-H2/CD30, EMA, ALKc anti-TIA-1 monoclonal antibodies. The patient underwent chemotherapy plus bone marrow transplantation and, one year after diagnosis, he is well and in complete remission. INTERPRETATION ANDCONCLUSIONS:
Our findings provide additional evidence that a) ALK+ lymphoma represents a single disease with a broad spectrum of morphology; b) clinicians and pathologists should be aware of the possible occurrence of LH variant of ALK+ ALCL also in adults in whom a favorable response to therapy may be expected despite systemic disease and an aggressive clinical presentation.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Proteínas Tirosina Quinases
/
Histiocitose de Células não Langerhans
/
Linfoma Difuso de Grandes Células B
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Haematologica
Ano de publicação:
2001
Tipo de documento:
Article
País de afiliação:
Itália