Production of 5-lipoxygenase pathway metabolites by peripheral leucocytes in capillary leak syndrome (Clarkson disease)

• Published in: European journal of clinical investigation Vol. 17; no. 1; p. 53
• Main Authors: Rondeau, E, Sraer, J, Bens, M, Doleris, L M, Lacave, R, Sraer, J D
• Format: Journal Article
• Language: English
• Published: England 01.02.1987
• Subjects: Adult; Arachidonate 5-Lipoxygenase - metabolism; Arachidonate Lipoxygenases - metabolism; Calcimycin - pharmacology; Capillary Permeability; Chromatography, High Pressure Liquid; Female; Humans; Hydroxyeicosatetraenoic Acids - metabolism; Hypotension - metabolism; Leukocytes - metabolism; Leukotriene B4 - metabolism; Male; Radioimmunoassay; SRS-A - metabolism
• ISSN: 0014-2972
• DOI: 10.1111/j.1365-2362.1987.tb01225.x

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.