Pathologic findings in adenosine deaminase deficient-severe combined immunodeficiency. II. Thymus, spleen, lymph node, and gastrointestinal tract lymphoid tissue alterations

H. Ratech, R. Hirschhorn, M. A. Greco

Research output: Contribution to journalArticle

22 Scopus citations

Abstract

Eight autopsies of patients with adenosine deaminase deficient-severe combined immunodeficiency disease (ADA- · -SCID) were reviewed with special emphasis on the lymphoid tissues. The thymus histology in five cases was remarkedly uniform, whether or not prior ADA enzyme replacement or immunologic reconstitution therapy had been administered. Lymph nodes and spleens in all cases examined showed a residual nonlymphoid architectural framework corresponding to usual T and B cell zones found in normals. The development of an extranodal, monoclonal IgA lambda B cell immunoblastic lymphoma as a terminal event in one patient after several years of successful ADA enzyme replacement therapy through multiple red blood cell transfusions is described. In another patient with long-term ADA enzyme replacement, a terminal autoimmune hemolytic anemia developed. Autopsy revealed severe deposits of iron in the B cell zones of the lymph nodes, which is an unusual location. In addition, iron deposits outlined the splenic trabeculae, as well as the ring fibers and bridging fibers of the splenic sinuses.

Original languageEnglish (US)
Pages (from-to)1145-1156
Number of pages12
JournalAmerican Journal of Pathology
Volume135
Issue number6
StatePublished - 1989

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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