Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Greinix, HT; Storb, R; Sanders, JE; Deeg, HJ; Doney, KC; Sullivan, KM; Witherspoon, RP.
Long-term survival and cure after marrow transplantation for congenital hypoplastic anaemia (Diamond-Blackfan syndrome).
Br J Haematol. 1993; 84(3):515-520
Doi: 10.1111/j.1365-2141.1993.tb03109.x
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- Führende Autor*innen der Med Uni Graz
- Greinix Hildegard
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- Abstract:
- Four patients with Diamond-Blackfan syndrome (congenital hypoplastic anaemia) whose disease was resistant to corticosteroid treatment and who were red blood cell transfusion-dependent, were given marrow grafts from allogeneic human-leucocyte-antigen (HLA)-identical siblings. The patients were conditioned with regimens including cyclophosphamide and busulfan. Three of four patients had sustained and complete marrow engraftment. One patient showed early signs of haematopoietic recovery but died on day 35 of pulmonary toxicity. The three surviving patients are well with normal haematopoiesis and Karnofsky performance scores of 100%, 3.0, 7.4 and 10.6 years after transplantation. Congenital hypoplastic anaemia can be treated successfully by allogeneic marrow grafts.
- Find related publications in this database (using NLM MeSH Indexing)
- Acute Disease -
- Adult -
- Blood Cell Count -
- Bone Marrow Transplantation -
- Child -
- Child, Preschool -
- Chronic Disease -
- Fanconi Anemia - blood Fanconi Anemia - mortality Fanconi Anemia - therapy
- Female -
- Follow-Up Studies -
- Graft Survival -
- Graft vs Host Disease - etiology
- Hematopoiesis - physiology
- Humans -
- Male -