Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentat...

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Bibliographic Details
Published inMedicina Vol. 80; no. 1; p. 81
Main Authors Odstrcil-Bobillo, M Silvina, Kohan, Dana, Heller, Paula G, Otero, Victoria, Russo, María Paula, Basquiera, Ana L
Format Journal Article
LanguageSpanish
Published Argentina 01.02.2020
Subjects
Adult
Biopsy
Diagnosis, Differential
Humans
Ileal Neoplasms - diagnosis
Ileal Neoplasms - pathology
Ileitis - diagnosis
Ileitis - pathology
Immunohistochemistry
Lymphoma - diagnosis
Lymphoma - pathology
Male
Wiskott-Aldrich Syndrome - diagnosis
Wiskott-Aldrich Syndrome - pathology
ileitis
Wiskott-Aldrich
lymphoproliferative syndrome
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Summary:Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.
ISSN:1669-9106