CIRCULATORY SHOCK AND COAGULATION DISORDE RS IN SYSTEMIC MASTOCYTOSIS

A 41-year-old man was admitted in circulatory shock of unknown aetiolo gy (systolic pressure 50 mm Hg) and marked reddening of the upper part of the body as well as maculopapular rash over the whole body. After 1500 ml of colloidal solution had been infused the blood pressure rose to a level at which the patient's condition was no longer at risk. He reported having had a similar attack of flushing and circulatory coll apse during the last few years, each time after drinking 3-4 l of beer . Laboratory test showed thromboplastin time 56%, partial thromboplast in time 130 s and thrombin time > 180 s. Three hours after admission t he coagulation times had further deteriorated, but had become normal w ithin 20 hours. After rest and after a provocation (hot bath) the seru m concentrations were: heparin 0.21 U/ml and 0.85 U/ml; histamine 1.9 mu g/ml and 2.3 mu g/ml; serotonin 23 mu g/ml and 38 mu g/ml. Histolog ical examination of an iliac crest bone marrow biopsy revealed dense c ollections of mast cells, as seen in systemic mastocytosis. A skin bio psy was diagnostic of urticaria pigmentosa.