Abstract：
:Severe congenital neutropenia (CN) (Kostmann syndrome) is a hematologic disorder characterized by a maturation arrest of myelopoiesis at the promyelocyte/myelocyte stage of development. This arrest results in severe neutropenia leading to absolute neutrophil counts (ANC) below 0.2 x 10(9)/L associated with severe bacterial infections from early infancy. Data on over 300 patients with CN collected by the Severe Chronic Neutropenia International Registry (SCNIR) beginning in 1994 indicate that more than 90% of these patients respond to recombinant human granulocyte-colony stimulating factor (r-HuG-CSF) treatment with an ANC greater than 1. 0 x 10(9)/L. For patients who are refractory to r-HuG-CSF treatment and continue to have severe and often life-threatening bacterial infections, hematopoietic stem cell transplantation is the only currently available treatment. We report on a total of 11 patients with CN reported to the SCNIR who underwent transplantation for reasons other than malignant transformation between 1976 and 1998. Of these patients, 8 were nonresponders or showed only partial response to r-HuG-CSF treatment with ongoing infections. Results from these patients suggest that transplantation of stem cells from an HLA-identical sibling is beneficial for patients refractory to r-HuG-CSF. (Blood. 2000;95:1195-1198)
journal_name
Bloodjournal_title
Bloodauthors
Zeidler C,Welte K,Barak Y,Barriga F,Bolyard AA,Boxer L,Cornu G,Cowan MJ,Dale DC,Flood T,Freedman M,Gadner H,Mandel H,O'Reilly RJ,Ramenghi U,Reiter A,Skinner R,Vermylen C,Levine JEsubject
Has Abstractpub_date
2000-02-15 00:00:00pages
1195-8issue
4eissn
0006-4971issn
1528-0020journal_volume
95pub_type
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