Oculocutaneous albinism, platelet storage pool disease, and progressive lupus nephritis.


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… (More)


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