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Comments, Opinions, and Brief Case Reports
January 28, 2002

Pulmonary Renal Syndrome and Thrombotic Thrombocytopenic Purpura in a Patient With Giant Cavernous Hemangioma of the Leg

Author Affiliations

Copyright 2002 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2002

Arch Intern Med. 2002;162(2):222-223. doi:

The microangiopathic hemolytic disorders cover a wide spectrum of clinical disorders and have a variety of underlying and triggering causes. Early diagnosis and treatment are essential to minimize organ damage and improve prognosis.

A 60-year-old man with giant congenital cavernous hemangioma of the leg (Figure 1, A) was admitted for recurrent gastrointestinal bleeding for 1 year. On admission his platelet count and coagulation profile were normal. Celiac angiogram, red blood cell scintiscan (Figure 1, B) and laparotomy led to the uneventful resection of 3 bleeding ileal segments. One week after the third surgery, he developed acute pallor, jaundice, fever, and oliguria. Investigations showed a low hemoglobin level (6.2 g/dL) and platelet count (12 × 103/µL), absent haptoglobin, a grossly elevated lactate dehydrogenase level (3560 U/L; normal, 197-401 U/L), and an elevated creatinine level (9.6 mg/dL [850 µmol/L]; normal, 0.9-1.4 mg/dL [76-128 µmol/L]), with a normal coagulation profile. A peripheral blood film showed microangiopathic schistocytes with polychromasia, and a clinical diagnosis of thrombotic thrombocytopenic purpura/hemolytic uremia syndrome (TTP/HUS) was made. A magnetic resonance image of the leg showed no bleeding or thrombosis. Screening test results for infective and autoimmune causes (antineutrophil cytoplasmic antibodies, antiglomerular basement membrane, antinuclear factor, anticardiolipin antibodies) were negative. He underwent daily plasmapheresis (8 L) with cryosupernatant replacement. By day 10 there was clinical and biochemical improvement (hemoglobin level, 7.5 g/dL; platelet count, 65 × 103/µL; lactate dehydrogenase level, 685 U/L; and creatinine level, 6.3 mg/dL [560 µmol/L]) with good urine output, and plasmapheresis was reduced to alternate-day treatment. However, his condition deteriorated with respiratory distress, and bronchoscopic lavage and lung function test results confirmed pulmonary hemorrhage.