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systemic mastocytosis

Also see mastocytosis. Pathology: - mast cell proliferation & organ infiltration Clinical manifestations: 1) 1/2 of patients may have no skin findings 2) weight loss 3) weakness 4) episodes of flushing (histamine release) 5) headache 6) diarrhea - malabsoprion 7) peptic ulcer 8) bath pruritus (itching after hot bath) 9) ascites 10) osteopenia Laboratory: 1) complete blood count: a) anemia b) leukocytosis c) eosinophilia 3) prostaglandins in urine Management: - treatment is primarily symptomatic with a goal of preventing mast cell activation [3] - prevention & treatment of anaphylaxis - pruritus & flushing - diarrhea & intestinal malabsorption - glucocorticoids to control malabsorption, bone pain, ascites, & to prvent anaphylaxis - epinephrine for acute anaphylaxis - histamine H1 receptor antagonists to control symptoms of anaphylaxis - diphenhydramine, hydroxyzine - aspirin may be useful if H1 blockers are not effective - ketotifen (mast cell stabilizer) - cromolyn is used for decreasing bone pain & headaches & for improving skin symptoms (flushing, pruritus) - H2 receptor blockers or proton pump inhibitor for peptic ulcer - psoralen ultraviolet A therapy for pruritus - aticholinergics for diarrhea - interferon alfa-2b may be useful in patients with osteopenia

General

mastocytosis

References

  1. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 718, 562-65
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 298
  3. Krishnan K eMedicine (Medscape): Mastocytosis, Systemic http://emedicine.medscape.com/article/203948-overview
  4. Medical Knowledge Self Assessment Program (MKSAP) 19 American College of Physicians, Philadelphia 2022