Microscopic polyangiitis (MPA) is associated with renal dysfunction, in most cases, and occasionally with pulmonary hemorrhage. However, massive intestinal bleeding is a rare manifestation. We report a case of MPA in a man who developed arterial bleeding in the small intestine. A 74-year-old man was admitted after enduring a fever for 4 weeks. Laboratory examination revealed leucocytosis with neutrophil predominance, and renal dysfunction was noted. He did not respond to treatment with antibiotics, and the worsened renal function soon required hemodialysis. On the seventh hospital day, he suddenly developed massive melena. A colonoscopic examination suggested bleeding in the small intestine, and abdominal angiography demonstrated that the bleeding originated in a branch of the ileal artery. Hemostasis was achieved by transarterial embolization (TAE), but the patient suffered a massive pulmonary hemorrhage 2 days after the TAE. Although he responded well to a combination treatment with corticosteroid and cyclophosphamide, recurrence of pulmonary hemorrhage led to death, on the 87th hospital day. MPA in this patient was associated with three serious complications; deteriorating renal function, massive melena, and pulmonary hemorrhage. Of the various manifestations associated with MPA, arterial bleeding in the gastrointestinal tract, although rare, should be considered as one of the serious complications in MPA.
Hemophagocytic syndrome (HPS) is a clinicopathological condition characterized by activation of macrophages or histiocytes with prominent hemophagocytosis in bone marrow and other reticuloendothelial systems (1). (Fig. 1A, B) and bone marrow (Fig. 1C), though no neoplastic cells were observed. Erythrophagocytosis was also observed in Kupffer cells in the liver (Fig. 1D)
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