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

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)203-208
Number of pages6
JournalAmerican Review of Respiratory Disease
Volume145
Issue number1
DOIs
StatePublished - 1992
Externally publishedYes

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Fingerprint

Dive into the research topics of 'A familial lymphoproliferative disorder presenting with primary pulmonary manifestations'. Together they form a unique fingerprint.

Cite this