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Titolo:
Osteogenic sarcoma associated with Diamond-Blackfan anemia: A report from the Diamond-Blackfan Anemia Registry
Autore:
Lipton, JM; Federman, N; Khabbaze, Y; Schwartz, CL; Hilliard, LM; Clark, JI; Vlachos, A;
Indirizzi:
Schneider Childrens Hosp, Long Isl Jewish Med Ctr, Albert Einstein Coll Med, Div Pediat Hematol Oncol & Stem Cell Transplantat, New Hyde Park, NY 11040 USA Schneider Childrens Hosp New Hyde Park NY USA 11040 de Park, NY 11040 USA Mt Sinai Sch Med, New York, NY USA Mt Sinai Sch Med New York NY USAMt Sinai Sch Med, New York, NY USA Johns Hopkins Hosp, Div Pediat Oncol, Baltimore, MD 21287 USA Johns Hopkins Hosp Baltimore MD USA 21287 Oncol, Baltimore, MD 21287 USA Univ Alabama, Sch Med, Div Pediat Hematol Oncol, Birmingham, AL USA Univ Alabama Birmingham AL USA Pediat Hematol Oncol, Birmingham, AL USA Loyola Univ, Cardinal Bernandin Canc Ctr, Div Oncol, Maywood, IL 60153 USALoyola Univ Maywood IL USA 60153 nc Ctr, Div Oncol, Maywood, IL 60153 USA
Titolo Testata:
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
fascicolo: 1, volume: 23, anno: 2001,
pagine: 39 - 44
SICI:
1077-4114(200101)23:1<39:OSAWDA>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
ROTHMUND-THOMSON SYNDROME; SOLID MALIGNANT-TUMORS; APLASTIC-ANEMIA; MARROW TRANSPLANTATION; GENE; OSTEOSARCOMA; SURVIVAL; LEUKEMIA; PROTEIN; CHILD;
Keywords:
Diamond-Blackfan anemia; malignancy; osteogenic sarcoma;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Lipton, JM Schneider Childrens Hosp, Long Isl Jewish Med Ctr, Albert Einstein Coll Med, Div Pediat Hematol Oncol & Stem Cell Transplantat, 269-01 76th Ave, New Hyde Park, NY 11040 USA Schneider Childrens Hosp 269-01 76th AveNew Hyde Park NY USA 11040
Citazione:
J.M. Lipton et al., "Osteogenic sarcoma associated with Diamond-Blackfan anemia: A report from the Diamond-Blackfan Anemia Registry", J PED H ONC, 23(1), 2001, pp. 39-44

Abstract

Purpose: Diamond-Blackfan anemia (DBA) is a congenital pure red cell aplasia, usually presenting in infancy or early childhood. A review of the literature strongly supports a predisposition to hematopoietic malignancy. Recently, solid tumors have been reported, some attributable to hemosiderosis and/or androgen therapy. Two cases of osteogenic sarcoma have also been documented. An analysis from the Diamond-Blackfan Anemia Registry was performed to evaluate the cancer risk in patients with DBA. Methods: The Diamond-Blackfan Anemia Registry of North America (DBAR) is acomprehensive database of patients with DBA enrolled, after informed consent, through outreach to pediatric hematologists and family groups. The patients and/or their families complete a detailed questionnaire, and a review of medical records and telephone interviews are performed to complete and clarify the information provided. Results: Of the 354 patients registered in the DEAR, there were six patients meeting the accepted diagnostic criteria for DBA who were found to have malignancies. Three patients had osteogenic sarcoma diagnosed, one with myelodysplastic syndrome, one with colon carcinoma, and one with a soft tissuesarcoma. Conclusion: There appears to be an association of osteogenic sarcoma with DBA. A young age at presentation may be a feature of DBA-associated osteogenic sarcoma. Because of the immaturity of the database, the actuarial risk for osteogenic sarcoma and other cancers in individuals with DBA cannot be ascertained. Speculation is made regarding the nature of the molecular defect leading to the association of DBA and osteogenic sarcoma.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/07/20 alle ore 13:02:55