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Pure White Cell Aplasia Complicated by Systemic Sclerosis with Accompanying Scleroderma Renal Crisis
Pure 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 seve...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Japanese Society of Internal Medicine
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9259819/ https://www.ncbi.nlm.nih.gov/pubmed/34803102 http://dx.doi.org/10.2169/internalmedicine.8436-21 |
Sumario: | Pure 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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