A patient with a mast cell disorder may release enough heparin to develop clinical bleeding.


Clinical features:

(1) mast cell disorder (localized or systemic)

(2) clinical bleeding disorder following mast cell activation

(3) prolonged aPTT or evidence of increased serum heparin

(4) absence of a bleeding disorder in the absence of mast cell activation


Protamine sulfate therapy may reverse the heparin effect. However, protamine sulfate may also activate mast cells, making the bleeding worse.


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