[Non-Hodgkin lymphoma arising in a case of Waldmann's intestinal lymphangiectasis].
A case of high grade malignant lymphoma in a young woman with congenital disseminated lymphangiectasis, exudative enteropathy and immunodeficiency is reported. Lymphoid malignancies occur with a high frequency in the course of this rare disease, possibly as a consequence of immunodeficiency. In this case, a selective defect of the OKT4+ peripheral blood lymphocyte subset has been shown. The relationships between congenital lymphangiectasis, immunodeficiency and malignant lymphoma are discussed.
D000328:Adult; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008201:Lymphangiectasis, Intestinal / Q000150:complications* / Q000151:congenital; D008223:Lymphoma / Q000209:etiology*; D011504:Protein-Losing Enteropathies / Q000150:complications*
Lymphome non Hodgkinien survenant au cours d'une lymphangiectasie intestinale de Waldmann.