Congenital acute T lymphoblastic leukaemia: report of a case with immunohistochemical and molecular characterisation.
J Clin Pathol
; 53(2): 150-2, 2000 Feb.
Article
en En
| MEDLINE
| ID: mdl-10767833
ABSTRACT
A newborn infant with congenital T cell lymphoblastic leukaemia presented with hepatosplenomegaly and pancytopenia at birth and died on the 21st day of multi-organ failure. Biopsy and necropsy examination showed extensive atypical lymphoid infiltrates in the lungs, liver, spleen, kidneys, lymph nodes, and bone marrow. Immunohistochemically, the lymphoid cells were TdT+, CD3+, CD45RO+, and CD10-, CD79a-, CD20-. Genotypic analysis using polymerase chain reaction showed T cell receptor gamma chain gene rearrangement and absence of immunoglobulin heavy chain gene rearrangement. This appears to be the first documented case of congenital T cell lymphoblastic leukaemia. The case had unusual histological and immunogenotypic features, disseminated early, and pursued a highly aggressive course. Consideration of the diagnosis is of paramount importance. The immunophenotypic studies and molecular characterisation of such congenital leukaemias are critical in arriving at a definite diagnosis.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Leucemia-Linfoma de Células T del Adulto
Límite:
Female
/
Humans
/
Newborn
Idioma:
En
Revista:
J Clin Pathol
Año:
2000
Tipo del documento:
Article
País de afiliación:
Estados Unidos