Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The hypereosinophilic syndrome is a multi-organ disease characterized by large counts of eosinophils in peripheral blood observed during at least six months without any evidence for other known causes of eosinophilia. Idiopathic hypereosinophilia is rare and is always diagnosed by exclusion of other disease. This work describes a man aged 21 years who was hospitalized at the Department of Dermatology because of skin manifestations resembling eczema and urticaria. High eosinophil counts in this patient necessitated a wide array of diagnostic tests. The hypereosinophilic syndrome was diagnosed after more than 12 months of follow-up and exclusion of other causes. Corticosteroids and hydroxycarbamide were administered as first-line therapy. Unfortunately, the patient was unresponsive to steroids. Improvement in peripheral eosinophilia after Glivec therapy correlated with improved clinical status.
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PMID:[The hypereosinophilic syndrome: case report]. 1747 38

Acquired agranulocytosis is a rare, life-threatening disorder. The few known causes/associations usually are readily identifiable (e.g., drug reaction, Felty syndrome, megaloblastosis, large granular lymphocytic leukemia, etc.). We report a novel association with mast cell disease. A 61-year-old morbidly obese man developed rheumatoid arthritis unresponsive to several medications. Agranulocytosis developed shortly after sulfasalazine was started but did not improve when the drug was soon stopped. Other symptoms across many systems developed including hives and presyncope. Marrow aspiration and biopsy showed only neutropenia. Serum tryptase was mildly elevated; urinary prostaglandin D2 was markedly elevated. Other causes were not found. Mast cell activation syndrome (MCAS) was diagnosed. Oral antihistamines, montelukast, and cromolyn were unhelpful; aspirin was initially felt contraindicated. Imatinib immediately increased neutrophils from 0% to 25% but did not help symptoms; subsequent addition of aspirin increased neutrophils further and abated symptoms. Different presentations of different MCAS patients reflect elaboration of different mediators likely consequent to different Kit mutations. Mast cells (MCs) help regulate adipocytes, and adipocytes can inhibit granulopoiesis; thus, a Kit-mutated MC clone may have directly and/or indirectly driven agranulocytosis. MCAS should be considered in otherwise idiopathic agranulocytosis presenting with comorbidities best explained by MC mediator release.
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PMID:Mast cell activation syndrome masquerading as agranulocytosis. 2233 92