Evaluation of proliferative activity of granulocytic progenitor cells from marrows of neutropenic patients was performed by utilizing in vitro culture methods measuring granulocytic colony-forming capacity (CFC) and the proportion of granulocytic progenitor cells in the DNA synthetic (S) phase of the cell cycle. Except for low CFC values found in five patients with myeloid hypoplasia, the remai...

A patient with acute leukemia in susations. Both aand /3-globulin UBBC tamed complete remission was studied were affected. Serum muramidase rose for 8 months. Periodic maintenance and fell out of phase with the granchemotherapy with cytosine arabinoside ulocyte and UBBC fluctuations, precedregularly induced granulocytopenia, with ing them by several days. These data granulocytosis upon marrow r...

OBJECTIVE Barth syndrome is a rare, X-linked recessive disorder that affects only boys. The cardinal characteristics include growth retardation, cardioskeletal myopathy, chronic or cyclic neutropenia, and 3-methylglutaconic aciduria. A preliminary study of five young boys with Barth syndrome suggested a distinct cognitive phenotype. METHODS The present study was designed to explore whether ad...

Canine cyclic neutropenia is an autosomal recessive disease in which the number of neutrophils, the primary blood phagocyte, oscillates between almost zero and normal values with two week frequency. We previously found that the causative mutation is an insertion of an extra adenine residue within a tract of nine A's in exon 21 of the 27 exon canine AP3B1 gene. In the course of identifying the m...

Several hematological diseases are characterised by oscillations of various blood cell populations. Two of these are a variant of chronic myelogenous leukemia (CML) and cyclical neutropenia (CN). These oscillations typically have long periods ranging from 20 to 60 days, despite the fact that the stem cell cycling time is thought to be of the order of 2-3 days. Clinical data from humans and labo...

x-linked agammaglobulinemia (xla) is a hereditary immunodeficiency, characterized by an early onset of recurrent bacterial infections, hypogammaglobulinemia and markedly reduced b lymphocytes number. in order to determine the association of neutropenia among iranian patients with xla, hospital records of 30 patients with confirmed xla in children medical center hospital, were reviewed. eight ou...