Cases reported "Mastocytosis"

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1/3. Anaesthetic management of systemic mastocytosis.

    Systemic mastocytosis is an uncommon disorder of mast cell proliferation in connective tissues. Mast cell degranulation may occur on exposure to various stimuli and drugs. The release of histamine, heparin and vasoactive substances such as prostaglandin d2 may cause severe hypotension and other anaphylactoid manifestations. Anaesthetic management should include perioperative stabilization of mast cells and avoidance of the use of histamine-releasing drugs. Intradermal skin testing is useful in predicting the sensitivity to drugs that may be used during anaesthesia. We present a patient with systemic mastocytosis who underwent uneventful cholecystectomy.
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keywords = anaesthesia
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2/3. Histamine levels and cardiovascular responses during splenectomy and splenorenal shunt formation in a patient with systemic mastocytosis.

    Systemic mastocytosis is a rare disorder characterised by the infiltration of many tissues by abnormal numbers of mast cells. Life-threatening episodes of bronchospasm and hypotension are common in response to a variety of triggers which cause mast cell degranulation. We present the case history of a patient with severe mast cell disease who successfully underwent splenectomy and portocaval anastomosis. Peri-operative therapy was directed towards mast cell stabilisation, and histamine-releasing drugs were avoided. Cardiovascular data, together with blood histamine levels and thrombin times, indicated that mast cell degranulation did not occur. A review of the current literature concerning mast cell disease as it relates to anaesthesia is presented and suggestions for the safe management of such cases are given.
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keywords = anaesthesia
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3/3. Systemic mastocytosis presenting as profound cardiovascular collapse during anaesthesia.

    mastocytosis is a rare disorder with serious anaesthetic implications. Anaesthetic management is hazardous since trauma, stress, extremes of temperature and drugs may precipitate intra-operative mast cell degranulation. Release of histamine and other mast cell mediators can lead to profound cardiovascular collapse and even death. We present a case report of a patient with mastocytosis who suffered cardiac arrest during anaesthesia. Anaphylactoid/anaphylactic shock may be delayed and lack supporting signs of histamine release such as cutaneous flushing and bronchospasm.
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ranking = 5
keywords = anaesthesia
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