Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor the clinical course, treatments, and disease outcomes for SCN patients. This report summarizes data for 853 patients, almost all treated with daily or alternate-day recombinant human granulocyte colony-stimulating factor (G-CSF or Filgrastim). G-CSF treatment increased the ANC overall from 0.34 x 10(9)/L +/- 0.018 pre-treatment to 3.70 x 10(9)/L +/- 0.18 during the first year of treatment. For most patients, the responses were durable with patients remaining on the same dose of G-CSF for many years. Long-term hematological observations showed stable mean leukocyte and neutrophil counts and gradually increasing hemoglobin levels. Thrombocytopenia developed in 4% of patients. As of January 1, 2000, myelodysplasia (MDS) or acute myelogenous leukemia (AML) has occurred in 35 of 387 patients with congenital neutropenia with a cumulative risk of 13% after 8 years of G-CSF treatment. This event occurred without a predictable relationship to the duration or dose of G-CSF treatment. No patients with cyclic or idiopathic neutropenia developed MDS or AML. Other important adverse events included hepatomegaly, osteoporosis, vasculitis, glomerulonephritis, and deaths in 4 of 14 cases requiring splenectomy. Growth and development and the outcome of pregnancy appeared to be unaffected by G-CSF treatment. These data indicate that congenital, cyclic, and idiopathic neutropenia can be effectively treated with long-term G-CSF. The risk of leukemia, osteoporosis, other potentially adverse events, and pregnancy outcome need to be further evaluated with continuing long-term observations.
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PMID:Severe chronic neutropenia: treatment and follow-up of patients in the Severe Chronic Neutropenia International Registry. 1255 10

Filgrastim and pegfilgrastim are granulocyte colony-stimulating factor (G-CSF) products, which have been part of the supportive treatment of cancer patients for years to increase the white blood cell count and absolute neutrophil count with the primary objective of preventing the appearance of neutropenic fever in patients at risk because of the very toxic chemotherapy. Pegfilgrastim is a glycosylated form of filgrastim with a prolonged duration of effect, a reduced renal clearance, and relatively fewer side effects. Rash in particular has been described rarely (less than 3.7% of cases). Various dermatologic complications have been associated with G-CSF therapy, with filgastrim more than pegfilgrastim. These complications include local skin reactions, folliculitis, vasculitis, and pyoderma gangrenosum as well as the more classic generalized allergic rash associated both with and without anaphylaxis. We present a patient with pancreatic cancer who developed facial rash related to the use of pegfilgrastim that led to discontinuation of the agent and we review the literature on this topic.
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PMID:Rash associated with the use of pegylated filgrastim in a patient with advanced pancreatic cancer. 1988 88