A familial lymphoproliferative disorder presenting with primary pulmonary manifestations

B. B. Rogers, I. Browning, H. Rosenblatt, K. McClain, J. Kemp, L. L. Glasser, M. V. Gresik, C. Sumaya, M. L. Wagner, M. S. Pollack, C. Langston

Resultado de la investigación: Articlerevisión exhaustiva

12 Citas (Scopus)


A familial lymphoproliferative disorder presented in three male siblings with primary pulmonary involvement manifested as either lymphoid interstitial pneumonia or an angiodestructive polymorphous infiltrate morphologically resembling lymphomatoid granulomatosis. The polymorphous infiltrate consisted chiefly of mature T-cells with a few B-cells and plasma cells, and gene rearrangement studies failed to show clonality. Epstein-Barr virus, frequently associated with proliferative lesions in males in the X-linked lymphoproliferative syndrome, was not demonstrated in any of the pulmonary lesions. An HLA haplotype shared among the affected siblings was A1, B8, DR4. The unusual clinical presentation plus the lack of involvement by EBV in the pulmonary lesions suggests that this is a previously undescribed familial lymphoproliferative disorder.

Idioma originalEnglish (US)
Páginas (desde-hasta)203-208
Número de páginas6
PublicaciónAmerican Review of Respiratory Disease
EstadoPublished - 1992
Publicado de forma externa

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


Profundice en los temas de investigación de 'A familial lymphoproliferative disorder presenting with primary pulmonary manifestations'. En conjunto forman una huella única.

Citar esto