A Case of Uveitis in a Patient With Juvenile Myelomonocytic Leukemia Successfully Treated With Adalimumab. Issue 5 (July 2020)

• Record Type: Journal Article
• Authors: Cortellazzo Wiel, Luisa; Pastore, Serena; Taddio, Andrea; Tommasini, Alberto
• Is Part Of: Journal of pediatric hematology/oncology. Volume 42:Issue 5(2020)
• Publication Date: 2020-07
• Subjects: JMML -- RALD -- CBL -- RAS -- autoimmune -- uveitis -- adalimumab; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000001448
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : Patients with juvenile myelomonocytic leukemia due to germline CBL mutation (10% to 15%) may have a subacute course occasionally associated with autoimmune disorders, which may resemble RAS-associated autoimmune lymphoproliferative disorder. In both conditions, prognosis and standard treatment for autoimmune phenomena remain poorly understood. We report the case of a 7-year-old boy with juvenile myelomonocytic leukemia with severe steroid-dependent uveitis, who did not respond to several therapeutic attempts with immunosuppressant agents, including sirolimus, and was finally successfully treated with adalimumab. This case offers further insight into the management of autoimmune disorders in the context of predisposing genetic conditions.