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Oculocutaneous Albinism, Platelet Storage Pool Disease, and Progressive Lupus Nephritis

Oculocutaneous Albinism, Platelet Storage Pool Disease, and Progressive Lupus Nephritis Abstract • Systemic lupus erythematosus (SLE) was seen in a patient with oculocutaneous albinism and platelet storage pool disease (Hermansky Pudlak syndrome). Although the patient had severely compromised platelet function, lupus nephritis developed nonetheless, which progressed to end-stage renal disease. The role of platelets in the pathogenesis of the nephritic process is discussed, and it is concluded that release of platelet granular constituents, particularly serotonin, is not required for the microvascular damage of SLE. (Arch Intern Med 1983;143:809-811) References 1. Cameron JS: Glomerulonephritis: Current problems and understanding. J Lab Clin Med 1982;99:755-787. 2. Miller K, Michael AF: Coagulation and the kidney , in McIntosh RM, Guggenheim SJ, Schrier RW (eds): Kidney Disease: Hematologic and Vascular Problems . New York, John Wiley & Sons, 1977, pp 3-28. 3. Kasai N, Parbtani A, Cameron JS, et al: Platelet-aggregating immune complexes and intraplatelet serotonin in idiopathic glomerulonephritis and systemic lupus. Clin Exp Immunol 1981;43:64-72. 4. Kant KS, Pollak VE, Weiss MA, et al: Glomerular thrombosis in systemic lupus erythematosus: Prevalence and significance. Medicine 1981; 60:71-86.Crossref 5. George CR, Slichter SJ, Quadracci LJ, et al: A kinetic evaluation of hemostasis in renal disease. N Engl J Med 1974;291:1111-1115.Crossref 6. Clark WV, Lewis ML, Cameron JS, et al: Intrarenal platelet consumption in the diffuse proliferative nephritis of systemic lupus erythematosus. Clin Sci Suppl 1975;49:247-252. 7. Parbtani A, Frampton G, Cameron JS: Platelet and plasma serotonin concentrations in glomerulonephritis: II. Clin Nephrol 1980;14:112-123. 8. Parbtani A, Frampton G, Yewdall V, et al: Platelet and plasma serotonin in glomerulonephritis: III. The nephritis of systemic lupus erythematosus. Clin Nephrol 1980;14:164-172. 9. Anti-platelet agents in nephrology, editorial. Lancet 1981;1:426-427. 10. Mosher DB, Fitzpatrick TB, Ortonne JP: Abnormalities of pigmentation , in Fitzpatrick TB, Eisen AZ, Wolff K, et al (eds): Dermatology in General Medicine , ed 2. New York, McGraw-Hill Book Co, 1979, pp 568-620. 11. Witkop CJ Jr, Quevedo WC Jr, Fitzpatrick TB: Albinism , in Stanbury JB, Wyngaarden JB, Frederickson DS (eds): Metabolic Basis of Inherited Disease . New York, McGraw-Hill Book Co, 1978, pp 283-313. 12. Green D, Ts'ao CH, Cohen I, et al: Haemorrhagic thrombocytopathy associated with dilatation of the platelet-membrane complex. Br J Haematol 1981;48:585-600. 13. Weiss HJ: Congenital disorders of platelet function. Semin Hematol 1980;17:228-241. 14. Cronlund M, Hardin J, Burton J, et al: Fibrinopeptide A in plasma of normal subjects and patients with disseminated intravascular coagulation and systemic lupus erythematosus. J Clin Invest 1976;58:142-151.Crossref 15. Hardin JA, Cronlund M, Haber E, et al: Activation of blood clotting in patients with systemic lupus erythematosus: Relationship to disease activity. Am J Med 1978;65:430-436.Crossref 16. Weiss HJ, Rosove MH, Lages BA, et al: Acquired storage pool deficiency with increased platelet-associated IgG: Report of five cases. Am J Med 1980;69:711-717.Crossref 17. Meyerhoff J, Dorsch CA: Decreased platelet serotonin levels in systemic lupus erythematosus. Arthritis Rheum 1981;24:1495-1500.Crossref 18. Hermansky F, Pudlak P: Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: Report of two cases with histochemical studies. Blood 1959;14:162-169. 19. Logan LJ, Rapaport SI, Maher I: Albinism and abnormal platelet function. N Engl J Med 1971;284:1340-1345.Crossref 20. Weiss HJ, Tschopp TB, Rogers J, et al: Studies of platelet 5-hydroxytryptamine (serotonin) in storage pool disease and albinism. J Clin Invest 1974;54:421-432.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Oculocutaneous Albinism, Platelet Storage Pool Disease, and Progressive Lupus Nephritis

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Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1983.00350040199030
Publisher site
See Article on Publisher Site

Abstract

Abstract • Systemic lupus erythematosus (SLE) was seen in a patient with oculocutaneous albinism and platelet storage pool disease (Hermansky Pudlak syndrome). Although the patient had severely compromised platelet function, lupus nephritis developed nonetheless, which progressed to end-stage renal disease. The role of platelets in the pathogenesis of the nephritic process is discussed, and it is concluded that release of platelet granular constituents, particularly serotonin, is not required for the microvascular damage of SLE. (Arch Intern Med 1983;143:809-811) References 1. Cameron JS: Glomerulonephritis: Current problems and understanding. J Lab Clin Med 1982;99:755-787. 2. Miller K, Michael AF: Coagulation and the kidney , in McIntosh RM, Guggenheim SJ, Schrier RW (eds): Kidney Disease: Hematologic and Vascular Problems . New York, John Wiley & Sons, 1977, pp 3-28. 3. Kasai N, Parbtani A, Cameron JS, et al: Platelet-aggregating immune complexes and intraplatelet serotonin in idiopathic glomerulonephritis and systemic lupus. Clin Exp Immunol 1981;43:64-72. 4. Kant KS, Pollak VE, Weiss MA, et al: Glomerular thrombosis in systemic lupus erythematosus: Prevalence and significance. Medicine 1981; 60:71-86.Crossref 5. George CR, Slichter SJ, Quadracci LJ, et al: A kinetic evaluation of hemostasis in renal disease. N Engl J Med 1974;291:1111-1115.Crossref 6. Clark WV, Lewis ML, Cameron JS, et al: Intrarenal platelet consumption in the diffuse proliferative nephritis of systemic lupus erythematosus. Clin Sci Suppl 1975;49:247-252. 7. Parbtani A, Frampton G, Cameron JS: Platelet and plasma serotonin concentrations in glomerulonephritis: II. Clin Nephrol 1980;14:112-123. 8. Parbtani A, Frampton G, Yewdall V, et al: Platelet and plasma serotonin in glomerulonephritis: III. The nephritis of systemic lupus erythematosus. Clin Nephrol 1980;14:164-172. 9. Anti-platelet agents in nephrology, editorial. Lancet 1981;1:426-427. 10. Mosher DB, Fitzpatrick TB, Ortonne JP: Abnormalities of pigmentation , in Fitzpatrick TB, Eisen AZ, Wolff K, et al (eds): Dermatology in General Medicine , ed 2. New York, McGraw-Hill Book Co, 1979, pp 568-620. 11. Witkop CJ Jr, Quevedo WC Jr, Fitzpatrick TB: Albinism , in Stanbury JB, Wyngaarden JB, Frederickson DS (eds): Metabolic Basis of Inherited Disease . New York, McGraw-Hill Book Co, 1978, pp 283-313. 12. Green D, Ts'ao CH, Cohen I, et al: Haemorrhagic thrombocytopathy associated with dilatation of the platelet-membrane complex. Br J Haematol 1981;48:585-600. 13. Weiss HJ: Congenital disorders of platelet function. Semin Hematol 1980;17:228-241. 14. Cronlund M, Hardin J, Burton J, et al: Fibrinopeptide A in plasma of normal subjects and patients with disseminated intravascular coagulation and systemic lupus erythematosus. J Clin Invest 1976;58:142-151.Crossref 15. Hardin JA, Cronlund M, Haber E, et al: Activation of blood clotting in patients with systemic lupus erythematosus: Relationship to disease activity. Am J Med 1978;65:430-436.Crossref 16. Weiss HJ, Rosove MH, Lages BA, et al: Acquired storage pool deficiency with increased platelet-associated IgG: Report of five cases. Am J Med 1980;69:711-717.Crossref 17. Meyerhoff J, Dorsch CA: Decreased platelet serotonin levels in systemic lupus erythematosus. Arthritis Rheum 1981;24:1495-1500.Crossref 18. Hermansky F, Pudlak P: Albinism associated with hemorrhagic diathesis and unusual pigmented reticular cells in the bone marrow: Report of two cases with histochemical studies. Blood 1959;14:162-169. 19. Logan LJ, Rapaport SI, Maher I: Albinism and abnormal platelet function. N Engl J Med 1971;284:1340-1345.Crossref 20. Weiss HJ, Tschopp TB, Rogers J, et al: Studies of platelet 5-hydroxytryptamine (serotonin) in storage pool disease and albinism. J Clin Invest 1974;54:421-432.Crossref

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Apr 1, 1983

References