Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Familial benign chronic neutropenia is a rare anomaly which is transmitted as an autosomally dominant trait and is characterized by normal or somewhat low total leukocyte counts, consistent neutropenia, and, usually, relative monocytosis and lymphocytosis, sometimes with eosinophilia. Affected individuals have a normal life expectancy. Many are asymptomatic, but some have histories of tendency to develop furuncles and/or periodontal disease. A Danish family with familial benign chronic neutropenia is reported. Four family members were affected, of these one had repeated attacks of severe stomatitis, two had histories of tendencies to develop furuncles, and one was asymptomatic.
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PMID:[Familial benign chronic neutropenia in a Danish family]. 240 45

Clinical data in 23 consecutive patients with chronic idiopathic neutropenia are reported. During a long-term follow-up (4 to 11 years), none had leukemia or autoimmune diseases. In particular, in no case did serious recurrent infections develop despite severe neutropenia. Immunological studies showed the presence of antibodies to neutrophils in a unique case and of the marker make-up of K lymphocytes in other two with chronic T8 lymphocytosis and associated neutropenia. The immunological features of this syndrome is briefly discussed. Chronic idiopathic neutropenia, even in the presence of an immunological imbalance, is a benign haematological disorder and does not need any treatment.
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PMID:[Chronic idiopathic neutropenia: a benign hematological disorder]. 325 73

Chronic idiopathic neutropenia can occur in spite of the normocellular bone marrow in myeloid series with or without anti-neutrophilic antibody. We report a patient with chronic neutropenia and severe polyclonal hypergammaglobulinemia. The patient demonstrated a positive anti-neutrophil antibody by fluorocytometry, although granulocyte-specific anti-nuclear factor and anti-stem cell (CFU-GM) antibody were negative. Thus, neutropenia of this patient appeared to be due to the antibody-mediated destruction of neutrophils. Both neutropenia and hypergammaglobulinemia were normalized by the administration of prednisone.
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PMID:Chronic idiopathic neutropenia with hypergammaglobulinemia. 349 73

Chronic idiopathic neutropenia (CIN) is a disorder characterized by severe neutropenia and a maturational arrest of the neutrophil precursors in the bone marrow. The pathogenesis of this disorder has been obscure. We examined the production of endogenous G-CSF and the expression of G-CSFmRNA in a patient with adult type CIN. The G-CSF production by the patient's mononuclear cells was deficient in spite of the adequate accumulation of G-CSFmRNA. Our data suggests that the defect in the endogenous G-CSF production at the post-transcriptional level is likely to be an aetiological factor in CIN.
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PMID:Impaired G-CSF production at post-transcriptional level in a patient with chronic idiopathic neutropenia. 750 8

Chronic idiopathic neutropenia (CIN) is a disorder characterized by severe neutropenia and a maturational arrest of the neutrophil precursors in the bone marrow. We examined the effect of recombinant human granulocyte-colony stimulating factor (rhG-CSF) on the growth and maturation of the myeloid progenitor cells from a patient with CIN. The patient's marrow cells showed poor colony forming activity, but a normal differentiating capacity to the stimulation with rhG-CSF, although they displayed a normal colony forming capacity in the presence of GM-CSF. Our observation indicates the distinct effect of rhG-CSF on the growth and maturation of the myeloid progenitors from a CIN patient.
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PMID:Distinct effect of G-CSF on the growth and differentiation of myeloid progenitor cells from chronic idiopathic neutropenia. 754 73

Chronic idiopathic neutropenia is regarded as a benign disorder without risk of malignant transformation. We present two patients with chronic idiopathic neutropenia who showed disease progression to acute myeloid leukaemia. Sequence analysis of the granulocyte-colony stimulating factor receptor (G-CSFR) gene from leukaemic DNA did not reveal any mutations and microsatellite analysis provided no evidence of microsatellite instability or loss of constitutional heterozygosity. These case studies suggest that chronic idiopathic neutropenia may constitute a preleukaemic condition in some patients. Alterations of the G-CSFR or defective DNA mismatch repair do not appear to be involved in malignant transformation.
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PMID:Two case studies of chronic idiopathic neutropenia preceding acute myeloid leukaemia. 1023 15

Antineutrophil antibodies are well recognized causes of neutropenia, producing both quantitative and qualitative defects in neutrophils and increased risk for infection. In primary autoimmune neutropenia (AIN) of infancy, a moderate to severe neutropenia is the sole abnormality; it is rarely associated with serious infections and exhibits a self-limited course. Chronic idiopathic neutropenia of adults is characterized by occurrence in late childhood or adulthood, greater prevalence among females than among males, and rare spontaneous remission. Secondary AIN is more commonly seen in adults and underlying causes include collagen disorders, drugs, viruses and lymphoproliferative disorders. In most patients with AIN, antibodies recognize antigens located on the IgG Fc receptor type 3b but other target antigens have been recently identified in secondary AIN. Granulocyte colony-stimulating factor is a proven treatment in patients with AIN of all types and is now preferred to other possible therapies.
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PMID:Primary and secondary autoimmune neutropenia. 1620 50

The incidental discovery of neutropenia during routine blood counting represents a common problem for clinicians. However, there are no reported data of systematic evaluations of adults with incidental neutropenia. As such, this was the aim of the present study. Ninety-seven adults with incidental neutropenia were submitted to a clinical and laboratory approach including medical evaluation, complete blood count (CBC), serial CBC, direct and indirect antiglobulin test, bone marrow smear and biopsy, assessment of folate, vitamin B12 and iron status, serum liver enzymes, serum proteins, serological exams for hepatitis B and C virus, cytomegalovirus, mononucleosis, human immunodeficiency virus and toxoplasmosis, detection of lupus erythematosus cells, antinuclear and anti-DNA antibodies and rheumatoid factor, dosage of free thyroxin and thyrotropin, chest roentgenogram and abdominal echography. Chronic idiopathic neutropenia of adults was identified in 34.0% of the individuals, neutropenia due to exposure to chemical agents was seen in 16.5%, infectious diseases in 9.3%, autoimmune diseases in 9.3%, haematological diseases in 9.3%, thyroid disorders in 8.2%, ethnic neutropenia in 7.2%, drug-related neutropenia in 2.1%, cyclic neutropenia in 2.1% and iron deficiency in 2.1%. Recovery or improvement of the neutrophil count was seen upon treatment or recuperation from infectious, autoimmune, haematological and thyroid diseases and iron supplementation. We conclude that the evaluation of individuals with incidental neutropenia using a structured approach may make the identification of clinically silent diseases possible, and provide an opportunity for early treatment, avoiding complications of the diseases and consequences of neutropenia.
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PMID:Causes of incidental neutropenia in adulthood. 1680 47