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

We report our experience with 8 patients with Felty's syndrome who were treated with D-penicillamine for a mean of one year. Six of the 8 patients experienced improvement in their neutropenia. Cutaneous ulcers healed in 4 of 6, while recurrent infections cleared in 3 of 5 patients. The drug was withdrawn in 6 patients--lack of response in one, thrombocytopenia in one, urticaria in one, rash in one, and granulocytopenia in 2. One of the latter 2 patients developed pancytopenia and died. Although D-penicillamine is effective in treatment of Felty's syndrome, its side effects can be serious and potentially lethal. Its use should be limited to patients who have failed other treatments.
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PMID:D-penicillamine in Felty's syndrome. 405 91

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