Characterization of platelets from normal mink and mink with the Chediak‐Higashi syndrome

Abstract

Bleeding times of mink with the Chediak-Higashi (CH) syndrome was markedly prolonged. Platelet counts were normal but there was an impaired platelet aggregation response to collagen. The metabolic adenine nucleotide pool of platelets from normal and CH mink was labeled with 14C-adenine and the platelets were gel-filtered. Gel-filtered platelets (GFP) from CH mink contained only 37.9% of the adenosine triphosphate (ATP) and 9.6% of the adenosine diphosphate (ADP) found in normal platelets and the ATP/ADP ratio was similar to the 14C-ATP/14C-ADP ratio. Platelet content of Ca2+, Mg2+, and in particular 5-hydroxytryptamine was decreased. When GFP were incubated with thrombin to induce maximal secretion, only negligible amounts of ATP and ADP were released. The specific activity of the extracellular nucleotides approximated that within the platelet. These findings suggest that the stored nucleotide pool in CH platelets is virtually absent and that the abnormalities in platelet function may be due, in part, to the essential absence of secretable ADP and serotonin. The release of Ca2+ and Mg2+ by CH platelets was 56% and 27.8% of normal, respectively.

Evaluation of the platelet storage pool deficiency in the feline counterpart of the chediak‐higashi syndrome

“…Whole blood coagulation times were determined by the Lee-White method as described previously [3]. Platelet-rich plasma (PRP) was obtained from citrated blood (0.1 1 M sodium citrate, 1 part citrate to 9 parts blood) by centrifugation (160g for 12 min) after allowing the blood to sit at room temperature for 30 minutes.…”

“…Associated with the Chediak-Higashi (CH) syndrome is a hemorrhagic tendency that appears to be, in part, due t o a thrombopathy similar t o that of human patients with platelet storage pool deficiency (SPD) [l- [ 2 ,3 ,7 ,9 , l o ] , Mg2+, and/or Ca2+ [2,3,7, 111, that are associated with a decreased number of dense bodies or storage vesicles [2,5,13, 141. Numerous defects of platelet function are observed in SPD [15] and CH [ 1 , 3 , 4 , 7 , 8 ] platelets including a markedly impaired collagen induced aggregation response.…”

“…The arachidonate pathway for platelet activation appears normal in some SPD patients but not in others [17-211. Platelets from CH mink do not form less MDA in response to arachidonate and thrombin than do normal mink platelets [3]. An accompanying defect in the metabolic nucleotide pool has not been reported in SPD platelets.…”

“…An accompanying defect in the metabolic nucleotide pool has not been reported in SPD platelets. Platelets from CH cattle and mink, however, have a reduced level of metabolic ATP [2,3].A feline counterpart of the CH syndrome has recently been identified in the Persian breed of cats [22]. The condition is recessively transmitted [23], granule abnormalities have been observed in leukocytes and kidney tubule cells [22], and enlarged melanin granules are present in hair, skin, and pigmented ocular structures [24].…”

“…Whole blood coagulation times were determined by the Lee-White method as described previously [3]. Platelet-rich plasma (PRP) was obtained from citrated blood (0.1 1 M sodium citrate, 1 part citrate to 9 parts blood) by centrifugation (160g for 12 min) after allowing the blood to sit at room temperature for 30 minutes.Bleeding times of thiamyal sodium anesthetized cats were obtained by a modified Ivy technique as described previously [3].The six normal and four CH cats used in this study were from the colony of cats mainChediak-Higashi Syndrome -Cats …”