DELAYED SPONTANEOUS REMISSION IN A CHILD WITH PRIMARY ACQUIRED CHRONIC PURE RED CELL APLASIA
العنوان: | DELAYED SPONTANEOUS REMISSION IN A CHILD WITH PRIMARY ACQUIRED CHRONIC PURE RED CELL APLASIA |
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المؤلفون: | Ayelet Ben Barak, Myriam Weyl Ben Arush, Ronit Elhasid, Abdalla Khalil |
المصدر: | Pediatric Hematology and Oncology. 27:574-580 |
بيانات النشر: | Informa UK Limited, 2010. |
سنة النشر: | 2010 |
مصطلحات موضوعية: | Male, Pathology, medicine.medical_specialty, Adolescent, Anemia, Hepatosplenomegaly, Pure red cell aplasia, Spontaneous remission, Red-Cell Aplasia, Pure, urologic and male genital diseases, Pallor, hemic and lymphatic diseases, medicine, Humans, Reticulocytopenia, business.industry, Remission Induction, Hematology, medicine.disease, Oncology, Erythropoietin, Chronic Disease, Pediatrics, Perinatology and Child Health, Rituximab, medicine.symptom, business, medicine.drug |
الوصف: | The acquired form of pure red cell aplasia (PRCA) presents either as an acute self-limited disease, predominantly seen in children, or as a chronic illness more frequently seen in adults with rare spontaneous remissions. A 14-year-old boy presented with pallor, without hepatosplenomegaly, jaundice, lymphadenopathy, petechiae, or any other apparent abnormalities. Isolated anemia in the presence of normal white cell and platelet counts with a marrow of normal cellularity and absence of erythroblasts but normal myeloid cells and megakaryocytes revealed the diagnosis of PRCA. All possible investigations excluded secondary causes of PRCA. The patient required packed red cell transfusions every 2 to 3 weeks. He failed therapy with intravenous immunoglobulin, corticosteroids, cyclosporine A plus corticosteroids, antithymocyte globulin, anti-CD 20 (rituximab), and erythropoietin (EPO). He showed a severe, resistant, and transfusion-dependent PRCA. Spontaneous remission with normal hemoglobin and reticulocyte levels was dramatic 6.5 years after the diagnosis of PRCA and 3.6 years after his last treatment. |
تدمد: | 1521-0669 0888-0018 |
DOI: | 10.3109/08880018.2010.493577 |
URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9a9b04c4538c1c2b35ebca3053187954 https://doi.org/10.3109/08880018.2010.493577 |
رقم الانضمام: | edsair.doi.dedup.....9a9b04c4538c1c2b35ebca3053187954 |
قاعدة البيانات: | OpenAIRE |
تدمد: | 15210669 08880018 |
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DOI: | 10.3109/08880018.2010.493577 |