MCN is an uncommon histological class of lupus nephritis. Typically, patients present with heavy proteinuria, and transient renal dysfunction is common. The prognosis of MCN in SLE appears to be good because of its rapid response to glucocorticoids. Relapses of proteinuria may be reduced by the use of maintenance immunosuppression. Alkylating agents, calcineurin inhibitors, mycophenolate mofetil, and rituximab can be considered in glucocorticoid-dependent or refractory cases of SLE-related MCN.

A 10-year-old Chinese boy who had a history of congenital thrombocytopathy presented with severe iron deficiency anemia secondary to chronic gastric inflammation and duodenal ulcerations. Subtle oculocutaneous albinism led to the finding of diminished dense bodies in the platelets under electron microscopy, hence the diagnosis of Hermansky-Pudlak syndrome (HPS). Biopsies from the stomach and duodenum revealed a lymphocytic infiltration in the submucosa, but H pylori infection was absent. The gastroduodenitis responded to the treatment with omeprazole while iron deficiency anemia was corrected by oral iron therapy. HPS is a rare cause of congenital bleeding disorder with multisystemic manifestations. Upper gastrointestinal involvement is rare and should be distinguished from a mere manifestation of the bleeding diathesis.

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