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Titolo:
Severe canine hereditary hemolytic anemia treated by nonmyeloablative marrow transplantation
Autore:
Zaucha, JM; Yu, C; Lothrop, CD; Nash, RA; Sale, G; Georges, G; Kiem, HP; Niemeyer, GP; Dufresne, M; Cao, QF; Storb, R;
Indirizzi:
Fred Hutchinson Canc Res Ctr, Div Clin Res, Seattle, WA 98109 USA Fred Hutchinson Canc Res Ctr Seattle WA USA 98109 , Seattle, WA 98109 USA Univ Washington, Dept Med, Seattle, WA 98195 USA Univ Washington Seattle WA USA 98195 ton, Dept Med, Seattle, WA 98195 USA Auburn Univ, Coll Vet Med, Scott Ritchey Res Ctr, Auburn, AL 36849 USA Auburn Univ Auburn AL USA 36849 ott Ritchey Res Ctr, Auburn, AL 36849 USA
Titolo Testata:
BIOLOGY OF BLOOD AND MARROW TRANSPLANTATION
fascicolo: 1, volume: 7, anno: 2001,
pagine: 14 - 24
SICI:
1083-8791(2001)7:1<14:SCHHAT>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
PYRUVATE-KINASE DEFICIENCY; MIXED HEMATOPOIETIC CHIMERISM; TOTAL-BODY IRRADIATION; IDENTICAL LITTERMATE DOGS; BONE-MARROW; PHARMACOLOGICAL IMMUNOSUPPRESSION; THALASSEMIC PATIENTS; CELL; LEUKEMIA; BASENJIS;
Keywords:
pyruvate kinase deficiency anemia nonmyeloablative stem cell transplantation mixed chimerism;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
58
Recensione:
Indirizzi per estratti:
Indirizzo: Storb, R Fred Hutchinson Canc Res Ctr, Div Clin Res, 1100 Fairview Ave N,D1-100,POB19024, Seattle, WA 98109 USA Fred Hutchinson Canc Res Ctr 1100 Fairview Ave N,D1-100,POB 19024 Seattle WA USA 98109
Citazione:
J.M. Zaucha et al., "Severe canine hereditary hemolytic anemia treated by nonmyeloablative marrow transplantation", BIOL BLOOD, 7(1), 2001, pp. 14-24

Abstract

Severe hemolytic anemia in Basenji dogs secondary to pyruvate kinase (PK) deficiency can be corrected by marrow allografts from healthy littermates after a conventional high-dose myeloablative conditioning regimen. The nonmyeloablative conditioning regimen used here, which consisted of a sublethal dose of 200 cGy total body irradiation before and immunosuppression with mycophenolate mofetil and cyclosporine after a dog leukocyte antigen DLA)identical littermate allograft, has been found to be effective in establishing stable mixed donor/host hematopoietic chimerism in normal dogs. We exploredthe feasibility of nonmyeloablative marrow allografts for the treatment ofcanine PK deficiency and studied the effect of stable allogeneic mixed hematopoietic chimerism on the natural course of the disease. Five affected dogs received transplants, of which 3 dogs had advanced liver cirrhosis and myelofibrosis. Both complications were presumed to be due to iron overload. All 5 dogs showed initial engraftment. Two rejected their grafts after 6 weeks but survived with complete autologous marrow recovery and return of thedisease, One dog died from liver failure on day 27 with 60% donor engraftment, Two dogs have shown sustained mixed donor/host chimerism for more thana year with 85% and 12% donor hematopoietic cells, respectively. Overall clinical response correlated with the degree of donor chimerism, The dog with the low degree of chimerism achieved partial resolution of hemolysis, butthe disease symptoms persisted as manifested by increasing iron overload resulting in progression of marrow and liver fibrosis, The dog with the highdegree of donor chimerism achieved almost complete resolution of hemolysiswith a decrease of marrow iron content and resolution of marrow fibrosis, These observations suggest that mixed hematopoietic chimerism can be relatively safely established in dogs with PK deficiency even in the presence of advanced liver cirrhosis, However, although effective in correcting or delaying the development of myelofibrosis, a low degree of mixed chimerism was not sufficient to prevent continued hemolysis of red blood cells of host origin. Complete donor chimerism appears necessary to achieve a long-term cure.

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Documento generato il 27/09/20 alle ore 00:44:28