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Weekly Rituximab Followed by Monthly Rituximab Treatment for Autoimmune Disease Associated With RAS-associated Autoimmune Leukoproliferative Disease

Toyoda, Hidemi, MD, PhD; Deguchi, Takao, MD, PhD; Iwamoto, Shotaro, MD, PhD; Kihira, Kentaro, MD; Hori, Hiroki, MD, PhD; Komada, Yoshihiro, MD, PhD; Hirayama, Masahiro, MD, PhD

Journal of Pediatric Hematology/Oncology: November 2018 - Volume 40 - Issue 8 - p e516–e518
doi: 10.1097/MPH.0000000000001276
Online Articles: Original Articles

Recently, a new disease of lymphocyte homeostasis caused by somatic mosaicism for the RAS mutation has been discovered (known as RALD, RAS-associated leukoproliferative disorder). Since few cases have been reported in literature, the prognosis and standard treatment for autoimmune diseases associated with RALD remain poorly understood. Standard rituximab therapy (375 mg/m2 for 4 wk) is effective in patients with autoimmune diseases, but early recurrences are common. We highlight the potential for monthly administration of rituximab in a patient with autoimmune thrombocytopenia and hemolytic anemia associated with RALD. RALD was diagnosed in an 11-year-old girl following a 9-year history of severe hepatosplenomegaly and autoimmune cytopenias. Genetic analyses confirmed somatic mosaicism for the G13C KRAS mutation without an autoimmune lymphoproliferative syndrome–related gene mutation. Rituximab therapy was used because of the refractory character of the autoimmune cytopenias which failed to respond to steroids and other immunosuppressive agents. Her treatment consisted of weekly infusions of rituximab for 4 weeks followed by monthly rituximab for 11 months. She maintained her response in hematologic parameters for 2 years after monthly rituximab was ceased and her scores representing quality of life were improved. Rituximab could improve clinical responses and quality of life of the patients with RALD.

Department of Pediatrics, Mie University, School of Medicine, Tsu, Japan

The authors declare no conflict of interest.

Reprints: Hidemi Toyoda, MD, PhD, Mie University School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan (e-mail: htoyoda@clin.medic.mie-u.ac.jp).

Received October 19, 2017

Accepted June 24, 2018

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