Treatment of cyclic neutropenia with very low doses of GM-CSF

Article Abstract:

Neutrophils and eosinophils are among the types of granule-containing white blood cells that develop from precursor stem cells in the bone marrow. Differentiation from stem cells into these different types of white cells is regulated by proteins, two of which, granulocyte colony-stimulating factor (G-CSF) and granulocyte macrophage-CSF (GM-CSF), have been identified and produced in the laboratory. In a condition known as cyclic neutropenia, the bone marrow periodically shuts down, resulting in low blood levels of neutrophils, and this is usually followed by intense blood cell production. The periods of severe neutropenia tend to recur about every three weeks and feature fever, ulceration of the mouth and throat, and infection. G-CSF has treated this disorder successfully, while doses of GM-CSF over 3 micrograms per kilogram of body weight per day (ug/kg/day) have increased levels of eosinophils but not neutrophils. A case is reported of a 30-year-old woman with cyclic neutropenia who was successfully treated with very low levels of GM-CSF. She had the disorder for five years, and suffered from great fatigue, mouth ulcers with resulting inability to eat, and fever for up to 10 days out of every 3 weeks. She had been hospitalized 11 times for high fever and complications of the disorder. She was treated with 0.3 ug/kg/day of GM-CSF, and after an initial unresponsive period, probably due to lack of cells, her condition responded in 10 days to the protein. After sharply peaking, neutrophil levels plateaued, and the patient had complete resolution of ulcers, fevers, and malaise. No significant adverse effects appeared. The report indicates that low-dose GM-CSF therapy may be very useful in neutropenic disorders. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Drug therapy, Neutropenia, Granulocyte-macrophage colony stimulating factor

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Association of sebaceous gland tumors and internal malignancy: the Muir-Torre syndrome

Article Abstract:

Muir-Torre syndrome (MTS) is a genetic disorder characterized by tumors of the sebaceous, or oil-secreting skin glands; internal malignancy; and keratoacanthomas, which are solid, round skin lesions filled with the tough protein keratin. Patients with MTS often develop several cancers of the abdominal organs. A history of internal organ cancer and sebaceous gland tumor are criteria for diagnosis of MTS. There have been 120 cases of MTS reported. The most common internal malignancies were colorectal cancer (51 percent) and cancer of the genital and urinary organs (25 percent) of cases. MTS-associated colorectal tumors developed near the splenic flexure, the bend at the junction of the transverse and descending portions of the colon, in 58 percent of cases. Almost 50 percent of MTS patients had more than one type of cancer. Forty-one percent of patients had a skin lesion before or at the same time as the internal cancer. The skin lesions appeared at about 53 years of age in most cases, whereas the internal organ cancers developed at about 50 years of age in most patients. The cancers tended to be slow-growing and the duration of follow-up has been more than 10 years for most patients. MTS patients with skin lesions should be assessed for internal malignancies, and relatives of MTS patients should also be evaluated for sebaceous gland tumors and internal cancers. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Colorectal cancer, Pelvic cancer

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A new familial immunodeficiency disorder characterized by severe neutropenia, a defective marrow release mechanism, and hypogammaglobulinemia

Article Abstract:

Inherited deficiencies of the immune system are rare. Two cases are described of sisters who developed a disorder characterized some of the following features: chronic infection of the lungs with papillomavirus and bacteria; low levels of immunoglobulin (Ig), an immune protein; neutropenia, an abnormal decrease in neutrophils (a type of white blood cell); physically distinct forms of neutrophils; and changes in granulopoiesis, the formation of granulocytes (a granular type of white blood cell). Previous cases of family members with abnormal neutrophils have been reported. The development of neutropenia and retention of neutrophils in the bone marrow has been referred to as myelokathexis. Neutropenia may result from defects in the ability of bone marrow to release the neutrophils, rather than abnormalities in the production and development of the cells. WHIM syndrome is a disorder characterized by the development of warts; hypogammaglobulinemia (decreased levels of Ig); infection; and myelokathexis. The two cases described in detail suggest a new immunodeficiency disorder consisting of WHIM syndrome and other known familial syndromes characterized by neutropenia and hypogammaglobulinemia. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Evaluation, Genetic aspects, Immunological deficiency syndromes, Immunologic deficiency syndromes, Familial diseases

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