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At least 451 records · Page 25Linked to original sources
PubMed2004

Chediak-Higashi syndrome: a case report.

A 5-month-old Chinese male infant was referred to the University Hospital, Kuala Lumpur for persistent fever, generalised rash and abdominal distension. Clinically he was suspected to have haemophagocytic lymphohistiocytosis. Haematological findings including the presence of several abnormal giant granules in neutrophils and single large azurophilic granules in many lymphocytes and monocytes in the peripheral blood established the diagnosis of Chediak-Higashi syndrome. The patient responded to allogeneic bone marrow transplant. This paper discusses the characteristic features, clinical course and management of this rare disorder. We suggest that peripheral blood film examination for the abnormal giant granules in granulocytes is an essential investigation in all young children with frequent recurrent infections or who are suspected to have virus-associated haemophagocytic syndrome or familial haemophagocytic lymphohistiocytosis.

Bone Marrow Transplantation↗
PubMed1991

The hematopoietic and mature blood cells of the rat: their morphology and the kinetics of circulating leukocytes in control rats.

Although the morphology of the hematopoietic and circulating blood cells of the rat differs slightly from that of human blood cells, the basophil is the only human blood cell for which the rat does not have a readily recognizable counterpart. The morphological classification of the rat's marrow and peripheral blood cells as based on our experience is described with reference to the differing observations of previous investigators and with comparison to the human hematopoietic system. Examination of the bone marrow and peripheral blood demonstrated that control rats injected i.v. with 1% normal rat serum in sterile saline exhibit a moderate transient lymphopenia as the only significant hematologic fluctuation induced by the stresses of injection, general anesthesia, and tail bleeding. Morphologic quantitation of marrow and peripheral blood cells complements the insights obtained by the culture of marrow cells. An appreciation of the morphology of the rat's blood cells and of the reproducible and quantitative nature of marrow and peripheral blood smear examinations may lead to more in vivo investigations of hematopoiesis and leukocyte trafficking.

Animals↗
PubMed1991

The effects of interleukin 6 and interleukin 3 on early hematopoietic events in long-term cultures of human marrow.

Interleukin (IL)-6 and IL-3, both alone and in combination, stimulate hematopoietic cells in short-term in vitro assays and in vivo. To study their ability to influence hematopoiesis in a system that mimics many features of the marrow microenvironment, long-term cultures (LTC) were produced by co-cultivating normal human marrow cells on feeder layers of murine marrow-derived stromal cells (M2-10B4 cells) genetically engineered to produce human IL-6 and/or IL-3. Feeders stably producing 20 ng/ml IL-6 slightly increased the output of clonogenic progenitors in these LTC but did not change the production of mature (total nonadherent) cells as compared to control cultures. Feeders producing 50 ng/ml IL-3 increased both clonogenic progenitor output (approximately threefold) and the output of mature cells (six-fold) as compared to controls. Feeders producing both factors also increased the output of both progenitors and mature cells. At the time of the weekly half-medium change when primitive clonogenic progenitors in the adherent layer are quiescent, such progenitors were actively cycling in all cultures with factor-producing feeders, as shown by [3H]thymidine suicide assays. Similarly, three sequential daily additions of 20 ng/ml of IL-6 also stimulated the quiescent progenitors to enter S-phase 2 days later, although single doses of recombinant IL-6 as high as 100 ng/ml failed to do so. The combined presence of IL-6- and IL-3-producing feeders, but neither alone, was also able to enhance more than twofold the maintenance and early differentiation of cells capable of generating clonogenic cells for at least a further 5 weeks in secondary LTC. Thus, the provision of a continuous source of IL-6 or IL-3 to primitive hematopoietic cells even in the LTC system can enhance late events in the hierarchy of hematopoietic cell differentiation, but a combination of the two factors is required to stimulate early multipotent progenitors.

Animals↗
PubMed1991

Endoglin is expressed on a subpopulation of immature erythroid cells of normal human bone marrow.

A monoclonal antibody (1G2) was raised by immunization of a Balb/c mouse with the leukemic blasts from a patient suffering from chronic myelogenous leukemia blast crisis (CML-BC). Sequential immunoprecipitation of the protein from human umbilical vein endothelial cells with 1G2 and the endoglin-specific monoclonal antibody 44G4 indicated that both antibodies react with the same molecule, a homodimer of molecular mass 180,000. This protein was first identified on acute lymphoblastic leukemia and was shown to be primarily associated with endothelial cells. In addition, 1G2 and 44G4 identified the same subpopulation of human bone marrow mononuclear cells (BMMNC), as established by two colour immunofluorescence analysis. By cell sorting and colony assays it could be demonstrated that endoglin is not expressed on hemopoietic precursor cells (CFU-G, CFU-GM, CFU-GEMM, BFU-E). May-Grünwald-Giemsa staining of sorted BMMNC revealed that 1G2 recognized immature proerythroblasts and double-fluorescence analysis showed that endoglin is present on a subset of glycophorin A-positive BMMNC. 1G2 was not reactive on bone marrow B-cells (CD19, CD20), T-cells (CD3, CD7), natural killer cells (CD56), myeloid cells (CD13, CD14, CD15, CD33), and on CD34-positive cells. Endoglin contains an arginine-glycine-aspartic acid sequence, a feature generally associated with extracellular matrix proteins which interact with integrins. It is suggested that proerythroblasts may utilize endoglin to interact with integrins in cell-cell adhesion events in the stromal or hemopoietic compartment of the bone marrow.

Animals↗
PubMed1990

Effect of culture conditions on in vitro erythroid colony formation in myelodysplastic syndromes.

In vitro colony formation by erythroid progenitors from the bone marrow was studied in 42 patients with a myelodysplastic syndrome using both a standard assay for growing erythroid colonies and an assay designed for growing megakaryocyte colonies. In the standard assay 5 patients had normal numbers of erythroid burst-forming unit (BFU-E) colonies, 8 showed reduced numbers of colonies, and 29 patients had no colony formation. Six patients with markedly reduced numbers of erythroid colonies or no colonies at all in the standard assay showed normal or increased numbers of erythroid colonies in the megakaryocyte assay. In three of these patients the erythroid colony morphology was normal, whereas the other three showed abundant diffuse growth of erythroid subclusters with no normal colonies. In the other patients, the erythroid culture results were similar in both assays. These results indicate that the impairment of erythroid colony growth seen in most patients with a myelodysplastic syndrome is, at least in a number of patients, not due to reduced numbers of erythroid progenitors but to unusual milieu requirements of abnormal progenitors.

Bone Marrow↗