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American Journal of Pathology, Vol 135, 1145-1156, Copyright © 1989 by American Society for Investigative Pathology

REGULAR ARTICLES

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

H Ratech, R Hirschhorn and MA Greco
Department of Pathology, New York University Medical Center, New York.

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 remarkably 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.


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Copyright © 1989 by the American Society for Investigative Pathology.