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April 1983

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

Author Affiliations

From the Departments of Medicine and Pathology, Northwestern Memorial Hospital and Northwestern University Medical School, Chicago (Drs Bomalaski and Carone), and the Atherosclerosis Program of the Rehabilitation Institute of Chicago (Dr Green).

Arch Intern Med. 1983;143(4):809-811. doi:10.1001/archinte.1983.00350040199030

• 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)