Production of 5-lipoxygenase pathway metabolites by peripheral leucocytes in capillary leak syndrome (Clarkson disease).
Periodic systemic capillary leak syndrome (Clarkson disease) is characterized by unexplained attacks of a marked increase in capillary permeability. As leukotrienes, derived from arachidonic acid via the 5-lipoxygenase pathway, enhance capillary permeability, we studied arachidonate metabolism in leucocytes of a patient with capillary leak syndrome. Leucocyte-platelet suspensions, prepared from blood collected from the patient during asymptomatic periods (n = 11) produced greater amounts of 5-hydroxyeicosatetraenoic acid (5-HETE) than control suspensions (P less than 0.05). Peripheral leucocytes, collected during attacks (n = 3) and studied without addition of A23187 released LTB4 in vitro but not sulphidopeptides leukotrienes. This result was never observed with leucocytes from control subjects or from the patient out of a crisis. These results suggest that in the patient, peripheral leucocytes could be stimulated by an unknown, as yet to be determined, endogenous factor to produce more 5-HETE and LTB4. Whether LTB4 plays a pathogenic role in the capillary leakage remains to be determined.
D000328:Adult; D001094:Arachidonate 5-Lipoxygenase / Q000378:metabolism*; D001091:Arachidonate Lipoxygenases / Q000378:metabolism*; D000001:Calcimycin / Q000494:pharmacology; D002199:Capillary Permeability; D002851:Chromatography, High Pressure Liquid; D005260:Female; D006801:Humans; D006893:Hydroxyeicosatetraenoic Acids / Q000378:metabolism*; D007022:Hypotension / Q000378:metabolism*; D007962:Leukocytes / Q000378:metabolism*; D007975:Leukotriene B4 / Q000378:metabolism; D008297:Male; D011863:Radioimmunoassay; D013189:SRS-A / Q000378:metabolism
D006893:Hydroxyeicosatetraenoic Acids; D013189:SRS-A; D007975:Leukotriene B4; D000001:Calcimycin; D001091:Arachidonate Lipoxygenases; D001094:Arachidonate 5-Lipoxygenase