Summary
Clinical and laboratory studies of two siblings, both suffering from gray platelet syndrome (GPS) are described. The patients had a mild bleeding disorder, their platelets were blue-gray in panoptic stains, and α-granules were markedly reduced, as shown by electron microscopy. The platelet content of platelet factor 4 and that of β-thromboglobulin were significantly reduced (3%–7% of normal). Platelet count was decreased (33–150×109/l) and small platelets were increased in platelet volume distribution. Bleeding time was prolonged on most occasions. Bone marrow aspiration was performed in one patient and revealed increased reticulin fibers, however, megakaryocyte count was normal. The mean platelet survival was 4.8 days using 111indium-labelled platelets. In this patient, platelet-associated IgG was within the normal range. Prednisone therapy failed to increase platelet count. Dental surgery was performed under cover of desmopressin and no bleeding complication occurred; however, no improvement of bleeding time was observed. The patient delivered a healthy male infant without hemorrhaging while under concurrent platelet transfusion therapy.
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References
Boughton BJ, Allington MJ, King A (1978) Platelet and plasma β-thromboglobulin in myeloproliferative syndromes and secondary thrombocytosis. Br J Haematol 40: 125–132
Breton-Gorius J, Vainchenker W, Nurden A, Levy-Toledano S, Caen J (1981) Defective α-granule production in megakaryocytes from gray platelet syndrome. Ultrastructural studies of bone marrow cells and megakaryocytes growing in culture from blood precursors. Am J Pathol 102: 10–19
Burck HC (1973) Histologische Technik. Leitfaden für die Herstellung mikroskopischer Präparate in Unterricht und Praxis. 3rd edn. Georg Thieme Verlag, Stuttgart, p 120
Castro-Malaspina H, Rabellino EM, Yen A, Nachman RL, Moore MAS (1981) Human megakaryocyte stimulation of proliferative of bone marrow fibroblasts. Blood 57: 781–787
Castro-Malaspina H, Moore MAS (1982) Pathophysiological mechanisms operating in the development of myelofibrosis: Role of megakaryocytes. Nouv Rev Fr Hematol 24: 221–226
Coller PS, Hultin MB, Nurden AT, Rosa JP, Lane BP (1983) Isolated α-granule deficiency (gray platelet syndrome) with slight increase in bone marrow reticulin and possible glycoprotein and/or protease defect (Abstract). Thromb Haemostas 50: 211
George JN, Nurden AT, Phillips DR (1984) Molecular defects in interactions of platelets with the vessel wall. N Engl J Med 311: 1084–1098
Gerrard JM, Phillips DR, Rao GHR, Plow EF, Walz DA, Ross R, Harker LA, White JG (1980) Biochemical studies of two patients with the gray platelet syndrome. Selective deficiency of platelet alpha granules. J Clin Invest 66: 102–109
Groopman JE (1980) The pathogenesis of myelofibrosis in myeloproliferative disorders. Ann Int Med 92: 857–858
Hardisty RM (1983) Hereditary disorders of platelet function. Clin Haematol 12: 153–173
Harker LA, Malpass TW, Branson HE, Hessel EA, Slichter SJ (1980) Mechanism of abnormal bleeding in patients undergoing cardiopulmonary bypass: Acquired transient platelet dysfunction associated with selective α-granule release. Blood 56: 824–834
Hellstern P, Dumitrescu E, Podolsak B, Dreher B, Meiser RJ, Wenzel E (1980) Typische Veränderungen der Größenverteilung zirkulierender Thrombozyten als Folge medikamentöser und transfusionsmedizinischer Maßnahmen. In: v. Voss H, Göbel U (eds) Praktische Anwendung der Thrombozytenfunktionsdiagnostik, 2. Symposion „Gerinnungsstörungen im Kindesalter“, Georg Thieme Verlag, Stuttgart, New York
Hellstern P, Miyashita C, Köhler M, Wenzel E, Holl W (1982) Thrombin-induced platelet malondialdehyde (MDA) formation in normal subjects and in women taking oral contraceptives. Thromb Haemostas 48: 325–326
Hiti-Harper J, Wohl H, Harper E (1978) Platelet factor 4: An inhibitor of collagenase. Science 199: 991–992
Kaplan DR, Chao FC, Stiles CD, Antoniades HN, Scher CD (1979) Platelet α-granules contain a growth factor for fibroblasts. Blood 53: 1043–1052
Krzywanek HJ, Grun H, Breddin K (1977) Thrombozytenfunktionstests. In: Englhardt A, Lommel H, Róka L, Ohler WGA (eds) Diagnostik hämorrhagischer Diathesen, Verlag Chemie, Weinheim, New York, pp 131–158
Kurstjens R, Bolt C, Vossen M, Haanen C (1968) Familial thrombopathic thrombocytopenia. Br J Haematol 15: 305–317
Levy-Toledano S, Caen JP, Breton-Gorius J, Rendu F, Cywiner-Golenzer C, Dupuy E, Legrand Y, Maclouf J (1981) Gray platelet syndrome: α-granule-deficiency. Its influence on platelet function. J Lab Clin Med 98: 831–848
Libánská J, Falcào L, Gautier A, Ammon J, Spahr A, Vainer H, Caen J (1975) Thrombocytopénie thrombocytopathique hypogranulaire héréditaire. Etude ultrastructurale d'une mégacaryocytopathie. Nouv Rev Fr Hematol 15: 165–182
Mielke CH, Kaneshiro MM, Maher IA, Weiner JM, Rapaport SI (1969) The standardized normal Ivy bleeding time and its prolongation by aspirin. Blood 34: 204–215
Morgenstern E (1982) Coated membranes in blood platelets. Europ J Cell Biol 26: 315–318
Mueller-Eckhardt C, Schultz G, Sauer KH, Dienst C, Mahn I (1978) Studies on the platelet radioactive anti-immunoglobulin test. J Immunol Meth 19: 1–11
Mueller-Eckhardt C, Kayser W, Mersch-Baumert K, Mueller-Eckhardt G, Breidenbach M, Kugel HG, Graubner M (1980) The clinical significance of platelet-associated IgG: A study on 298 patients with various disorders. Br J Haematol 46: 123–131
Nurden AT, Kunicki TJ, Dupuis D, Soria C, Caen JP (1982) Specific protein and glycoprotein deficiencies in platelets isolated from two patients with the gray platelet syndrome. Blood 59: 709–718
Pumphrey CW, Dawes J (1983) Platelet alpha granule depletion: Findings in patients with prosthetic heart valves and following cardiopulmonary bypass surgery. Thromb Res 30: 257–264
Raccuglia G (1971) Gray platelet syndrome. A variety of qualitative platelet disorder. Am J Med 51: 818–828
Reimers HJ, Packham MA, Kinlough-Rathbone RL, Mustard JF (1973) Effect of repeated treatment of rabbit platelets with low concentrations of thrombin on their function, metabolism and survival. Br J Haematol 25: 675–689
Rendu F, Nurden AT, Lebret M, Caen JP (1979) Relationship between mepacrine-labelled dense body number, platelet capacity to accumulate 14C-5-HT and platelet density in the Bernard-Soulier and Hermansky-Pudlak syndromes. Thromb Haemostas 42: 694–704
Suzuki S, Mori K, Sugai S, Akutsu J, Sakai H (1983) Electronmicroscopic studies on platelet in congenital platelet function disorders (2) Gray platelet syndrome (Abstract). Thromb Haemostas 50: 369
White JG, Gerrard JM (1976) Ultrastructural features of abnormal blood platelets. A review. Am J Pathol 83: 590–614
White JG (1979) Ultrastructural studies of the gray platelet syndrome. Am J Pathol 95: 445–462
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Köhler, M., Hellstern, P., Morgenstern, E. et al. Gray platelet syndrome: Selective α-granule deficiency and thrombocytopenia due to increased platelet turnover. Blut 50, 331–340 (1985). https://doi.org/10.1007/BF00320926
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DOI: https://doi.org/10.1007/BF00320926