British journal of haematology, 2005-05, Vol.129 (4), p.534-538
2005
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Details
- Autor(en) / Beteiligte
- Titel
- Use of mycophenolate mofetil for chronic, refractory immune cytopenias in children with autoimmune lymphoproliferative syndrome
- Ist Teil von
- British journal of haematology, 2005-05, Vol.129 (4), p.534-538
- Ort / Verlag
- Oxford, UK: Blackwell Science Ltd
- Erscheinungsjahr
- 2005
- Quelle
- MEDLINE
- Beschreibungen/Notizen
- Summary Autoimmune lymphoproliferative syndrome (ALPS) is a disorder of apoptosis associated most often with heritable FAS mutations leading to lymphadenopathy, hypersplenism and chronic refractory autoimmune cytopenias. Mycophenolate mofetil (MMF) was used to treat cytopenias in 13 ALPS patients aged 9 months to 17 years from a cohort of 118 children (aged < 18 years) and 82 adults. Twelve responded for a median follow‐up of 49 weeks (range 38–240 weeks), defined by maintenance of adequate blood counts and reduction in dosage or cessation of other immunosuppressive agents. This preliminary experience suggests that MMF may spare steroid usage in patients with ALPS‐associated cytopenias.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0007-1048eISSN: 1365-2141DOI: 10.1111/j.1365-2141.2005.05496.xTitel-ID: cdi_proquest_journals_198585454
- Format
- –
- Schlagworte
- Adolescent, Anemia, Hemolytic, Autoimmune - drug therapy, Anemia, Hemolytic, Autoimmune - immunology, Anemias. Hemoglobinopathies, Autoimmune Diseases - drug therapy, Autoimmune Diseases - immunology, autoimmune haemolytic anaemia, autoimmune lymphoproliferative syndrome, autoimmune neutropenia, Biological and medical sciences, Child, Child, Preschool, Diseases of red blood cells, Follow-Up Studies, Hematologic and hematopoietic diseases, Hematology, Humans, immune‐mediated thrombocytopenia, Immunosuppressive Agents - adverse effects, Immunosuppressive Agents - therapeutic use, Infant, Leukocyte Count, Lymphoproliferative Disorders - drug therapy, Lymphoproliferative Disorders - immunology, Male, Medical sciences, mycophenolate mofetil, Mycophenolic Acid - adverse effects, Mycophenolic Acid - analogs & derivatives, Mycophenolic Acid - therapeutic use, Neutropenia - drug therapy, Neutropenia - immunology, Other diseases. Hematologic involvement in other diseases, Pain - chemically induced, Prodrugs, Thrombocytopenia - drug therapy, Thrombocytopenia - immunology