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Diamond‐blackfan anemia and malignancy. A case report and a review of the literature
Author(s) -
van Dijken Pieter J.,
Verwijs Wim
Publication year - 1995
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19950801)76:3<517::aid-cncr2820760324>3.0.co;2-8
Subject(s) - medicine , diamond–blackfan anemia , malignancy , anemia , diamond , dermatology , pediatrics , pathology , metallurgy , genetics , ribosome , rna , gene , biology , materials science
Background . Diamond‐Blackfan anemia (DBA) is a congenital bone marrow failure syndrome involving the erythropoietic lineage. A preleukemic predisposition has been suggested but not proven. Methods . The development of Hodgkin's disease in a 15‐year‐old boy with DBA, in remission for 13 years after cessation of steroid therapy, is described. Review of the literature revealed 11 other cases of malignancy (10 hematologic) in DBA. Results . This patient, together with those described in the literature, shows that the incidence of hematological malignancy in DBA is increased (2.5% of all reported cases of DBA). Treatment was successful in three, including one after allogeneic bone marrow transplantation and our patient in whom recurrence of DBA complicated treatment. Conclusions . The incidence of hematologic malignancies in patients with DBA is increased. Treatment can be successful but may be complicated by recurrence of DBA. Bone marrow transplantation should be considered for patients with a suitable donor as part of treatment of patients with DBA and hematologic malignancy. Cancer 1995;76:517‐20.