title: Coincident Kaposi sarcoma and T-cell lymphoma in a patient with the Wiskott-Aldrich syndrome.
abstract: A 24 year old male with a history of eczema, recurrent mild infections, and thrombocytopenia consistent with the Wiskott-Aldrich syndrome (WAS) presented with a mediastinal mass, generalized lymphadenopathy, splenomegaly, and severe thrombocytopenia. Studies of immune function including immunoglobulin levels and T-cell subsets were normal. Furthermore, his T lymphocytes proliferated normally in response to phytohemagglutinin, concanavalin A, and the combination of neuraminidase/galactose oxidase. However, their proliferative responses to anti-CD43 antibody and periodate were diminished, consistent with the clinical diagnosis of WAS. An initial inguinal lymph node biopsy surprisingly revealed Kaposi sarcoma. However, following splenectomy to increase the platelet count, biopsy of the mediastinal mass revealed T-cell large cell lymphoma. Studies of biopsied tissue for the presence of Epstein-Barr virus and cytomegalovirus were negative, as were studies of blood, including the polymerase chain reaction, for the presence of the human immunodeficiency virus (HIV). This is the first report of Kaposi sarcoma arising in a patient with a congenital immunodeficiency syndrome. Although Kaposi sarcoma can arise in the face of the severe immunosuppression that follows allograft transplantation and in patients infected with HIV, we postulate that longevity in the face of mild immunosuppression was the major factor in the development of Kaposi sarcoma in this patient.
authors.affiliation: Hematology-Oncology Section, Hospital of the University of Pennsylvania, Philadelphia 19104.
authors.forename: N J
authors.initials: NJ
authors.lastname: Meropol
authors.forename: D
authors.initials: D
authors.lastname: Hicks
authors.forename: J J
authors.initials: JJ
authors.lastname: Brooks
authors.forename: K A
authors.initials: KA
authors.lastname: Siminovitch
authors.forename: N O
authors.initials: NO
authors.lastname: Fishman
authors.forename: J A
authors.initials: JA
authors.lastname: Kant
authors.forename: J S
authors.initials: JS
authors.lastname: Bennett
mesh_terms: D000328:Adult; D001706:Biopsy; D002455:Cell Division / Q000187:drug effects; D003587:Cytomegalovirus / Q000235:genetics / Q000276:immunology; D004279:DNA, Viral / Q000032:analysis / Q000235:genetics; D004797:Enzyme-Linked Immunosorbent Assay; D006678:HIV / Q000235:genetics / Q000276:immunology; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000276:immunology; D006801:Humans; D007165:Immunosuppression Therapy; D008198:Lymph Nodes / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D016133:Polymerase Chain Reaction; D012514:Sarcoma, Kaposi / Q000150:complications* / Q000276:immunology / Q000473:pathology; D013154:Spleen / Q000473:pathology; D013601:T-Lymphocytes / Q000187:drug effects / Q000473:pathology; D013997:Time Factors; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000276:immunology / Q000473:pathology
chemical_list: D004279:DNA, Viral