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Case Reports
Pure White Cell Aplasia Complicated by Systemic Sclerosis with Accompanying Scleroderma Renal Crisis: A Case Report.
- Eiji Suzuki, Ryoma Oda, Takashi Kanno, Satoru Kimura, Yurie Saito, Hiroyuki Kanbayashi, Shin Matsuda, and Kiyoshi Migita.
- Department of Rheumatology, Ohta-Nishinouchi Hospital, Japan.
- Intern. Med. 2022 Jun 15; 61 (12): 1907-1912.
AbstractPure white cell aplasia (PWCA) is a rare neutropenic disorder caused by absence of neutrophil-lineage cells. A 49-year-old man was diagnosed with scleroderma renal crisis 2 months prior to admission to Ohta-Nishinouchi Hospital after experiencing a fever and abdominal pain. Blood tests revealed severe neutropenia, and bone marrow aspirate showed the absence of neutrophil-lineage cells. He was diagnosed with PWCA. Steroids alone were not effective, but adding cyclosporine A and high-dose immunoglobulin recovered his neutropenia and improved his condition. Cyclosporine A and high-dose immunoglobulin are thus considered effective for treating PWCA in autoimmune diseases.
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