Thromb Haemost 1992; 67(06): 708-712
DOI: 10.1055/s-0038-1648527
Original Articles
Schattauer GmbH Stuttgart

Prolonged Bleeding Time of Chediak-Higashi Cats Corrected by Platelet Transfusion

B E Cowles
1   The Department of Veterinary and Comparative Anatomy, Pharmacology and Physiology, Washington State University, Pullman, WA, USA
,
K M Meyers
1   The Department of Veterinary and Comparative Anatomy, Pharmacology and Physiology, Washington State University, Pullman, WA, USA
,
K J Wardrop
1   The Department of Veterinary and Comparative Anatomy, Pharmacology and Physiology, Washington State University, Pullman, WA, USA
,
M Menard
2   The Department of Veterinary Clinical Medicine and Surgery, Washington State University, Pullman, WA, USA
,
D Sylvester
3   College of Pharmacy, Washington State University, Pullman, WA, USA
› Author Affiliations
Further Information

Publication History

Received 28 August 1991

Accepted after revision 20 December 1991

Publication Date:
03 July 2018 (online)

Summary

Cats with the Chediak-Higashi syndrome (CHS) have a platelet storage pool deficiency (SPD). Ten CHS cats were transfused with a concentrate of 51Cr-labeled platelets prepared from normal donor cats. Gne hour after transfusion, the donor platelet count in CHS recipient cats was 40,000-60,000/μl. Bleeding time before transfusion was 9.1 ± 3.0 min. When donor platelet count in CHS cats was 50,000/μl, bleeding time was 1.7 ± 0.2 min. Bleeding time of normal cats was 1.4 ± 0.3 min. Bleeding time increased to 3.3 ± 0.2 min and to 5.3 ± 0.2 min when the platelet count was 30,000/μl, and 15,000/μl, respectively. The close inverse relationship between bleeding time and number of donor platelets in CHS cats (r = —0.92), suggests that prolonged bleeding time is due to a platelet abnormality, that platelet transfusion can effectively correct prolonged bleeding time in an animal model of platelet SPD and that CHS cats may be an appropriate animal model to evaluate hemostatic capabilities of transfused platelets.

 
  • References

  • 1 Hardisty RM, Hutton RA. Bleeding tendency associated with “new” abnormality of platelet behavior. Lancet 1967; 1: 983-985
  • 2 Weiss HJ, Chervenick PA, Zalusky R, Factor A. A familial defect in platelet function associated with impaired release of adenosine diphosphate. N Engl J Med 1969; 281: 1264-1270
  • 3 Caen JP, Sultan Y, Larrieu MJ. A familial platelet disease. Lancet 1968; 27: 203-204
  • 4 Logan LJ, Rapaport SI, Maher I. Albinism and abnormal platelet function. N Engl J Med 1971; 284: 1340-1350
  • 5 Holmsen H, Weiss HJ. Hereditary defect in the platelet release reaction caused by a deficiency in the storage pool of platelet adenine nucleotides. Br J Haematol 1970; 19: 643-649
  • 6 Lages B, Holmsen H, Weiss HJ, Dangelmaier C. Thrombin and ionophore A23187-induced dense granule secretion in storage pool deficient platelets: Evidence for impaired nucleotide storage as the primary dense granule defect. Blood 1983; 61: 154-162
  • 7 Weiss HJ, Rosove MH, Lages BA, Kaplan KL. Acquired storage pool deficiency with increased platelet-associated IgG report of five cases. Am J Med 1980; 69: 711-717
  • 8 Weiss HJ. Congenital disorders of platelet function. Semin Hematol 1980; 17: 228-241
  • 9 Blume RS, Wolff SM. The Chediak-Higashi syndrome: Studies in four patients and a review of the literature. Medicine 1972; 51: 247-280
  • 10 Leader RW, Padgett GA, Gorham JR. Studies of abnormal leukocyte bodies in mink. Blood 1963; 22: 477-484
  • 11 Padgett GA, Leader RW, Gorham JR. Familial occurrence of the Chediak-Higashi syndrome in mink and cattle. Genetics 1964; 49: 505-512
  • 12 Lutzner MA, Lowrie CT, Jordan HW. Giant granules in leukocytes of the beige mouse. J Hered 1967; 58: 299-300
  • 13 Kramer JW, Davis WC, Prieur DJ. The Chediak-Higashi syndrome of cats. Lab Invest 1977; 36: 554-562
  • 14 Sjaastad OV, Blom AK, Stormorken H, Nes N. Adenine nucleotides, serotonin, and aggregation properties of platelets of blue foxes (Alopex lagopus) with the Chediak-Higashi Syndrome. Am J Med Genet 1990; 35: 373-378
  • 15 Nishimura M, Inoue M, Nishikawa T, Miyamoto M, Kobayashi T, Kitamura Y. Beige Rat: A new animal model of Chediak-Higashi syndrome. Blood 1989; 74: 270-273
  • 16 Taylor RF, Farrell RK. Light and electron microscopy of peripheral blood neutrophils in a killer whale affected with Chediak-Higashi syndrome. Fed Proc 1973; 32: 822
  • 17 Meyers KM, Holmsen H, Seachord CL, Hopkins GE, Borchard RE, Padgett GA. Storage pool deficiency in platelets from Chediak-Higashi cattle. Am J Physiol 1979; 237: R239-48
  • 18 Meyers KM, Holmsen H, Seachord CL, Hopkins G, Gorham J. Characterization of platelets from normal mink and mink with the Chediak-Higashi syndrome. Am J Hematol 1979; 7: 137-146
  • 19 Meyers KM, Seachord CL, Holmsen H, Prieur DJ. Evaluation of the platelet storage pool deficiency in the feline counterpart of the Chediak-Higashi syndrome. Am J Hematol 1981; 11: 241-253
  • 20 Aptiz-Castro R, Cruz MR, Ledzman E, Merino F, Ramerez-Duque C, Dangelmeier C, Holmsen H. The storage pool deficiency in platelets from humans with the Chediak-Higashi syndrome: A study of six patients. Br J Haematol 1985; 59: 471-483
  • 21 Boxer GJ, Holmsen H, Robkin L, Bank NU, Boxer LA, Baehner RL. Abnormal platelet functions in the Chediak-Higashi syndrome. Br J Haematol 1977; 35: 521-533
  • 22 Holland JM. Serotonin deficiency and prolonged bleeding time in beige mice. Proc Soc Exp Biol Med 1976; 151: 32-39
  • 23 Ito M, Sato A, Tanabe F, Ishida E, Takami Y, Shigeta S. The thiol proteinase inhibitors improve the abnormal rapid down-regulation of protein kinase C and the impaired natural killer cell activity in (Chediak-Higashi syndrome) beige mouse. Biochem Biophys Res Commun 1989; 160: 433-440
  • 24 Sato A, Tanabe F, Ito M, Shigeta S. Thiol proteinase inhibitors reverse the increased protein kinase C down-regulation and concava-lin A cap formation in polymorphonuclear leukocytes from Chediak-Higashi syndrome (beige) mouse. J Leuko Biol 1990; 48: 377-381
  • 25 Pyrzwansky KB, Schliwa M, Boxer LA. Microtubule organization of unstimulated and stimulated adherent human neutrophils in Chediak-Higashi syndrome. Blood 1985; 66: 1398-1403
  • 26 Boxer LA, Albertini DE, Baehner RL, Oliver JM. Correction of leukocyte functions in the Chediak-Higashi syndrome correctable by ascorbate. N Engl J Med 1976; 43: 207-213
  • 27 Haak RA, Ingraham LM, Baechner RL, Boxer LA. Membrane fluidity in human and Chediak-Higashi leukocytes. J Clin Invest 1979; 64: 138-143
  • 28 Ingerman CM, Smith JB, Shapiro S, Sedar A, Silver MJ. Hereditary abnormality of platelet aggregation attributable to nucleotide storage pool deficiency. Blood 1978; 52: 332-344
  • 29 Pareti FI, Day HJ, Mills DCB. Nucleotide and serotonin metabolism in platelets with defective secondary aggregation. Blood 1974; 44: 789-800
  • 30 Witkop CJ, Quevedo WC, Fitzpatrick TB. Albinism. In: The Metabolic Basis of Inherited Disease. Stanbury JB, Wyngaarden JB, Fredrickson DS. (eds) McGraw-Hill Book Company; New York: 1978. 14th edn. pp 283-316
  • 31 Theuring F, Fiedler J. Fatal bleeding following tooth extraction. Hermansky-Pudlak syndrome. Dtsch Stomatol 1973; 23: 52
  • 32 Nieuwenhuis HK, Sixma JJ. l-Deamino-8-D-arginine vasopressin (Desmopressin) shortens the bleeding time in storage pool deficiency. Ann Intern Med 1988; 108: 65-67
  • 33 Mannucci PM, Vicente V, Vianello L, Cattaneo M, Alberca I, Coccato MP, Faioni E, Mari D. Controlled trial of desmopressin in liver cirrhosis and other conditions associated with a prolonged bleeding time. Blood 1986; 67: 1148-1153
  • 34 Kobrinsky NL, Israels ED, Gerrard JM, Cheang MS, Watson CM, Bishop AJ, Schroeder ML. Shortening of bleeding time by l-deamino-8-d-arginine vasopressin in various bleeding disorders. Lancet 1984; 1: 1145-1148
  • 35 Gerritsen SW, Akkerman JW, Sixma JJ. Correction of the bleeding time in patients with storage pool deficiency by infusion of cryo-precipitate. Br J Haematol 1978; 40: 153-160
  • 36 Tschopp TB, Zucker MB. Hereditary defect in platelet function in rats. Blood 1972; 40: 217-226
  • 37 McGarry MP, Novak EK, Reddington M, Swank RT. Effects of mixed chimeric bone marrow repopulation on platelet storage pool-associated bleeding defects in mouse mutants. Exp Hematol 1990; 18: 1174-1179
  • 38 Wilkerson MJ, Wardrop KJ, Giger U, Meyers KM. Two cat colonies with A and B blood types and a clinical transfusion reaction. Feline Pract 1991; 19: 22-26
  • 39 Parker MT, Collier LL, Kier AB, Johnson GS. Oral mucosal bleeding times of normal cats and cats with the Chediak-Higashi syndrome or Hagman Trait (factor XII deficiency). Vet Clin Pathol 1987; 17: 9-13
  • 40 Ott L. An introduction to statistical methods and data analysis. PES-Kent Publishing Company; Boston, MA: 1988. 3 170-300
  • 41 Harker LA, Finch CA. Thrombokinetics in man. J Clin Invest 1969; 48: 963-975
  • 42 Slichter SJ. Poststorage platelet viability in thrombocytopenic recipients is reliably measured by radiochromium-labeled platelet recovery and survival measurements in normal volunteers. Transfusion 1986; 26: 8-13
  • 43 Slichter SJ, Deeg HJ, Kennedy MS. Prevention of platelet alloimmunization in dogs with systemic cyclosporine and by UV-irradiation or cyclosporine-loading of donor platelets. Blood 1987; 69: 414-418
  • 44 Jacobs RM, Boyce JT, Kociba GJ. Flow cytometric and radioisotopic determinations of platelet survival time in normal cats and feline leukemia virus-infected cats. Cytometry 1986; 7: 64-69
  • 45 Mannucci PM, Canciani MT, Rota L, Donovan BS. esponse of factor VIH/von Willebrand factor to DDAVP in healthy subjects and patients with haemophilia A and von Willebrand’s Disease. Br J Haematol 1981; 47: 283-293
  • 46 Mannucci PM, Aberg M, Milsson IM, Robertson B. Mechanism of plasminogen activator and factor VIII increase after vasoactive drugs. Br J Haematol 1975; 30: 81-93
  • 47 Novak EK, McGarry MP, Swank RT. Correction of symptoms of platelet storage pool deficiency in animal models for Chediak-Higashi syndrome and Hermansky-Pudlak syndrome. Blood 1985; 66: 1196-1201
  • 48 Meyers KM, Seachord CL, Holmsen H, Prieur DS. Evaluation of the storage pool deficiency in the feline counterpart of the Chediak-Higashi syndrome. Am J Hematol 1981; 11: 241-247
  • 49 Tschopp TB. Aggregation of cats in vitro. Thromb Diath Haemorrh 1970; 23: 601-608
  • 50 Rosenblum WI, Nelson GH, Cockrell CS, Ellis EF. Some properties of mouse platelets. Thromb Res 1983; 30: 347-353
  • 51 Drummond AH. Interaction of blood platelets with biogenic amines: uptake, stimulation, and receptor binding. In: Platelets in Biology and Pathology I. North-Holland; New York: 1976. pp 203-239
  • 52 Meyers KM. Species differences. In: Platelet Responses and Metabolism I. Holmsen H. (ed) CRC Press; Boca Raton, CA: 1986. pp 209-234
  • 53 Shanwell A, Gulliksson H, Berg BK, Jansson BA, Svensson LA. Evaluation of platelets prepared by apheresis and stored for 5 days. Transfusion 1989; 29: 783-788