Sclerosing cholangitis and intracranial lymphoma in a child with classical Wiskott–Aldrich syndrome. Issue 1 (27th August 2016)

• Record Type: Journal Article
• Authors: Vignesh, Pandiarajan; Suri, Deepti; Rawat, Amit; Lau, Yu Lung; Bhatia, Anmol; Das, Ashim; Srinivasan, Anirudh; Dhandapani, Sivashanmugam
• Is Part Of: Pediatric blood & cancer. Volume 64:Issue 1(2017)
• Page Start: 106
• Page End: 109
• Publication Date: 2016-08-27
• Subjects: CNS lymphoma -- primary sclerosing cholangitis -- Wiskott–Aldrich syndrome; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26196
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Patients with Wiskott–Aldrich syndrome (WAS) are predisposed to malignancy and autoimmunity in addition to infections. We report a male child with WAS, who had presented with recurrent pneumonia, eczema, thrombocytopenia, autoimmune hemolytic anemia, and vasculitic skin lesions. Genetic analysis revealed a classical genotype WAS 155C>T; R41X. At 2 years of follow‐up, he developed persistent headache and progressive hepatomegaly. Brain imaging showed a mass in the right frontal region, which on histopathology was shown to be high‐grade non‐Hodgkin lymphoma. Magnetic resonance cholangiopancreatography showed features of sclerosing cholangitis. This report extends the clinical spectrum and highlights unusual manifestations of sclerosing cholangitis and intracranial lymphoma in a patient with WAS.