Infantile genetic agranulocytosis

agranulocytosis /agran·u·lo·cy·to·sis/ (a-gran″u-lo-si-to´sis) a symptom complex characterized by decreased granulocytes and by lesions of the throat, other mucous membranes, gastrointestinal tract, and skin; most cases are complications of drug therapy, radiation, or exposure to chemicals.

a·gran·u·lo·cy·to·sis (

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An acute disease characterized by high fever, lesions of the mucous membranes and skin, and a sharp drop in circulating granular white blood cells.

Agranulocytosis

An acute condition marked by severe depression of the bone marrow, which produces white blood cells, and by prostration, chills, swollen neck, and sore throat sometimes with local ulceration. Aalso called agranulocytic angina or granulocytopenia.

Mentioned in: Antipsychotic Drugs, Central Nervous System Stimulants

agranulocytosis

[āgran′yoo͡lō′sītō′sis]

a severe reduction in the number of leukocytes (basophils, eosinophils, and neutrophils). Neutropenia results, whereby the body is severely depleted in its ability to defend itself. Fever, prostration, and bleeding ulcers of the rectum, mouth, and vagina may be present. The acute disease may be an adverse reaction to a medication or the result of the effect of radiation therapy or chemotherapy on bone marrow.

agranulocytosis [a-gran″u-lo-si-to´sis]

an acute disease in which there is a dramatic decrease in the production of granulocytes, so that a pronounced neutropenia evolves, leaving the body defenseless against bacterial invasion. A great majority of cases are caused by sensitization to drugs or chemicals that affect the bone marrow and depress the formation of granulocytes. Called also malignant or pernicious leukopenia and idiopathic or malignant neutropenia.

Symptoms. The first manifestations are usually produced by a severe infection and include high fever, chills, prostration, and ulcerations of mucous membranes such as in the mouth, rectum, or vagina. Laboratory tests reveal a profound leukopenia (low leukocyte count).

Treatment. Treatment is aimed at immediate withdrawal of the drug or chemical causing the disorder, and control of infection. In most cases control can be achieved by the administration of antibiotics. If the bone marrow is not irreparably damaged, the prognosis is good with proper treatment, and the patient will recover as the production of granulocytes resumes. Occasionally the leukocyte-producing tissues are damaged beyond repair and death ensues.

infantile genetic agranulocytosis a severe congenital condition of virtual absence of neutrophils from the blood; most patients die of infection before reaching adulthood. Called also congenital neutropenia and Kostmann's syndrome or neutropenia.

agranulocytosis (āgran´y

lōsītō´sis),

n a decrease in the number of granulocytes in peripheral blood resulting from bone marrow depression by drugs and chemicals or replacement by a neoplasm. Oral lesions are ulceronecrotic, involving the gingivae, tongue, buccal mucosa, or lips. Regional lymphadenopathy and lymphadenitis are prevalent.

Agranulocytosis.

agranulocytosis

a disease state characterized by a marked reduction in the granulocyte count in the blood and in the body's defenses against bacterial invasion. Called also granulocytopenia. See also feline panleukopenia.

agranulocytosis

Granulocytopenia, granulopenia Hematology A marked ↓ in PMNs < 500/mm3 Clinical Fever, malaise, mucocutaneous ulcers–throat, GI tract, skin Etiology Acquired due to adverse response to prescription drugs–chloramphenicol, clozapine, nitrous oxide, procainamide, sulfonamides, thiazide diuretics. See Infantile genetic agranulocytosis.

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agranulosis aleukia aleukocytosis angina pectoris Antipsychotic Drugs basophilic leukopenia captopril Central Nervous System Stimulants clozapine Clozaril congenital agranulocytosis cyclic neutropenia dipyrone granulocytopenia granulopenia infantile agranulocytosis Kostmann's syndrome leukocytopenia leukopenia	infant mortality infant mortality rate infant of chemically dependent mother infant of diabetic mother infant seat Infanticide infantile infantile acrodermatitis infantile acropustulosis infantile agranulocytosis infantile amnesia infantile arteritis infantile autism infantile celiac disease infantile cerebral ataxic paralysis infantile cerebral sphingolipidosis infantile cirrhosis infantile colic infantile cortical hyperostosis infantile digital fibromatosis infantile dwarf infantile eczema infantile encephalitis infantile fibromatosis infantile fibrosarcoma Infantile genetic agranulocytosis infantile hemiplegia infantile hydrocele infantile muscular atrophy infantile myofibromatosis infantile neuroaxonal dystrophy infantile osteomalacia infantile paralysis infantile pellagra infantile poliomyelitis infantile polyarteritis infantile progressive spinal muscular atrophy infantile purulent conjunctivitis infantile scurvy infantile sexuality Infantile Spasm infantile spasms infantile spinal muscular atrophy infantile spinal paralysis infantile uterus infantilism infantophagia infarct infarct extension infarct-avid scintigraphy infarctectomy	infantile cerebral ataxic paralysis infantile cerebral sphingolipidosis infantile cirrhosis Infantile colic Infantile colic Infantile colic Infantile Convulsions and Choreoathetosis infantile cortical hyperostosis infantile cortical hyperostosis infantile cortical hyperostosis Infantile diarrhea Infantile diarrhea Infantile diarrhea infantile digital fibromatosis infantile dwarf infantile dwarf infantile dwarf infantile dwarf infantile dwarf Infantile Dystrophy infantile eczema infantile eczema infantile encephalitis Infantile epileptic encephalopathy Infantile esotropia Infantile esotropia Infantile esotropia infantile fibromatosis infantile fibrosarcoma infantile fixation Infantile genetic agranulocytosis infantile genitalia infantile giantism infantile giantism infantile giantism infantile glaucoma infantile glaucoma infantile glaucoma infantile hemiplegia infantile hydrocele Infantile Hypercalcaemia/Williams-Beuren Syndrome Infantile hypercalcemia Infantile hypercalcemia infantile hypertrophic gastric stenosis infantile hypertrophic gastric stenosis infantile hypertrophic gastric stenosis Infantile Hypertrophic Pyloric Stenosis Infantile Hypotonia Infantile Hypotonia Infantile Hypotonia Infantile Idiopathic Nephrotic Syndrome infantile lobar emphysema infantile lobar emphysema infantile lobar emphysema infantile massive s infantile massive s infantile massive s infantile muscular atrophy Infantile myoclonic encephalopathy infantile myofibromatosis infantile neuroaxonal dystrophy

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