Abstract
Neutropenia is a rare complication of Diamond-Blackfan syndrome (congenital hypoplastic anaemia). Three patients are reported: all had neutropenia as well as anaemia, and to investigate the cause of the neutropenia culture of bone marrow for granulocyte-macrophage colony forming cells (GMCFCs) was performed. Two cases had a low incidence of GMCFCs, but the third case had a high incidence. These findings suggest that myeloid precursors can be abnormal in Diamond-Blackfan syndrome and that the mechanism of neutropenia may, like that of anaemia, vary from patient to patient.
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Selected References
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- Diamond L. K., Wang W. C., Alter B. P. Congenital hypoplastic anemia. Adv Pediatr. 1976;22:349–378. [PubMed] [Google Scholar]
- Ershler W. B., Ross J., Finlay J. L., Shahidi N. T. Bone-marrow microenvironment defect in congenital hypoplastic anemia. N Engl J Med. 1980 Jun 12;302(24):1321–1327. doi: 10.1056/NEJM198006123022401. [DOI] [PubMed] [Google Scholar]
- Freedman M. H., Saunders E. F. Diamond-Blackfan syndrome: evidence against cell-mediated erythropoietic suppression. Blood. 1978 Jun;51(6):1125–1128. [PubMed] [Google Scholar]
- Halperin D. S., Freedman M. H. Diamond-blackfan anemia: etiology, pathophysiology, and treatment. Am J Pediatr Hematol Oncol. 1989 Winter;11(4):380–394. [PubMed] [Google Scholar]
- Kurtzman G. J., Cohen B., Meyers P., Amunullah A., Young N. S. Persistent B19 parvovirus infection as a cause of severe chronic anaemia in children with acute lymphocytic leukaemia. Lancet. 1988 Nov 19;2(8621):1159–1162. doi: 10.1016/s0140-6736(88)90233-4. [DOI] [PubMed] [Google Scholar]
- Smith M. A., Shah N. R., Lobel J. S., Cera P. J., Gary G. W., Anderson L. J. Severe anemia caused by human parvovirus in a leukemia patient on maintenance chemotherapy. Clin Pediatr (Phila) 1988 Aug;27(8):383–386. doi: 10.1177/000992288802700806. [DOI] [PubMed] [Google Scholar]
- Steinberg M. H., Coleman M. F., Pennebaker J. B. Diamond-Blackfan syndrome: evidence for T-cell mediated suppression of erythroid development and a serum blocking factor associated with remission. Br J Haematol. 1979 Jan;41(1):57–68. doi: 10.1111/j.1365-2141.1979.tb03681.x. [DOI] [PubMed] [Google Scholar]
- Tsai P. H., Arkin S., Lipton J. M. An intrinsic progenitor defect in Diamond-Blackfan anaemia. Br J Haematol. 1989 Sep;73(1):112–120. doi: 10.1111/j.1365-2141.1989.tb00229.x. [DOI] [PubMed] [Google Scholar]
- Wasser J. S., Yolken R., Miller D. R., Diamond L. Congenital hypoplastic anemia (Diamond-Blackfan syndrome) terminating in acute myelogenous leukemia. Blood. 1978 May;51(5):991–995. [PubMed] [Google Scholar]
