Abstract
Renal thrombotic microangiopathy (TMA) is an uncommon vascular complication of severe hypertension. Until now, its clinical-pathological characteristics and renal survival have been unclear. Twenty-one patients with biopsy-proven renal TMA and with severe or malignant hypertension were retrospectively studied. All the patients exhibited severe hypertension, with systolic blood pressure at 200–280 mmHg and diastolic pressure at 110–180 mmHg. No patients had hemorrhagic manifestations. Elevated lactate dehydrogenase and thrombocytopenia were found in 6 and 5 patients, respectively. Significant proteinuria (>3 g/day) was present in 2 patients and microscopic hematuria in 18 patients. All patients presented with renal insufficiency (creatinine 3.1±2.1 mg/dL). The level of von Willebrand factor:antigen (vWF:Ag) in patients was not significantly higher than that in the healthy subjects, while the ADAMTS13 (a disintegrin and metalloprotease, with thrombospondin-1–like domains) activity was not significantly lower than that in the healthy subjects. Renal histology showed a TMA involving preglomerular arterioles and/or interlobular arteries characterized by fibrin deposits and vascular wall sclerosis. Fibrin glomerular microthrombi were not observed in these patients. Four patients required hemodialysis upon admission for severe acute renal failure. On follow-up, 3 patients had recovered normal renal function and 14 had mild renal insufficiency (creatinine 1.8±0.3 mg/dL), while 4 patients still required persistent hemodialysis. In conclusion, compared with patients having hemolytic uremic syndrome/thrombocytopenic purpura, our patients showed a low incidence of thrombocytopenia and better renal outcome.
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Moake JL : Thrombotic microangiopathies. N Engl J Med 2002; 347: 589–600.
Liapis H : Thrombotic microangiopathy involving the kidney: a histopathologic perspective. Hippokratia 2003; 7: 152–158.
Vaughan CJ, Delanty N : Hypertensive emergencies. Lancet 2000; 356: 411–417.
Khanna A, McCullough PA : Malignant hypertension presenting as hemolysis, thrombocytopenia, and renal failure. Rev Cardiovasc Med 2003; 4: 255–259.
Egan JA, Bandarenko N, Hay SN, et al: Differentiating thrombotic microangiopathies induced by severe hypertension from anemia and thrombocytopenia seen in thrombotic thrombocytopenia purpura. J Clin Apher 2004; 19: 125–129.
Galbusera M, Benigni A, Paris S, et al: Unrecognized pattern of von Willebrand factor abnormalities in hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. J Am Soc Nephrol 1999; 10: 1234–1241.
Rick ME, Molls S, Taylor MA : Clinical use of rapid collagen binding assay for von Willebrand factor cleaving protease in patients with thrombotic thrombocytopenic purpura. Thromb Haemost 2002; 88: 598–604.
Kadiri S, Olutade BO : The clinical presentation of malignant hypertension in Nigerians. J Hum Hypertens 1991; 5: 339–343.
Espinosa G, Bucciarelli S, Cervera R, et al: Thrombotic microangiopathic haemolytic anemia and antiphospholipid antibodies. Ann Rheum Dis 2004; 63: 730–736.
Gurkan E, Baslamisli, Guvenc B, et al: Thrombotic thrombocytopenic purpura in southern Turkey: a single-center experience of 29 cases. Clin Lab Haematol 2005; 27: 121–125.
Hollenbeck M, Kutkuhn B, Aul C, et al: Haemolytic-uraemic syndrome and thrombotic-thrombocytopenic purpura in adults: clinical findings and prognostic factors for death and end-stage renal disease. Nephrol Dial Transplant 1998; 13: 76–81.
Bridoux F, Vrtovsnik F, Noel C, et al: Renal thrombotic microangiopathy in systemic lupus erythematosus: clinical correlations and long-term renal survival. Nephrol Dial Transplant 1998; 13: 298–304.
Farrugia E, Torres VE, Gastineau D, et al: Lupus anticoagulant in systemic lupus erythematosus: a clinical and renal pathological study. Am J Kidney Dis 1992; 20: 463–471.
Ono H, Ono Y : Nephrosclerosis and hypertension. Med Clin North Am 1997; 81: 1273–1288.
Morel-Maroger L, Kanfer A, Solez K, et al: Prognostic importance of vascular lesions in acute renal failure with microangiopathic hemolytic anemia (hemolytic-uremic syndrome). Clinicopathologic study in 20 adults. Kidney Int 1979; 15: 548–558.
Schieppati A, Ruggenenti P, Cornejo RP, et al: Renal function at hospital admission as a prognostic factor in adult hemolytic uremic syndrome. The Italian Registry of Haemolytic Uremic Syndrome. J Am Soc Nephrol 1992; 2: 1640–1644.
Conlon PJ, Howell DN, Macik G, Kovalik EC, Smith SR : The renal manifestation and outcome of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in adults. Nephrol Dial Transplant 1995; 10: 1189–1193.
Tsai HM, Chandler WL, Sarode R, et al: von Willebrand factor and von Willebrand factor–cleaving metalloprotease activity in Escherichia coli O157: H7–associated hemolytic uremic syndrome. Pediatr Res 2001; 49: 653–659.
Moore JC, Hayward CPM, Warkentin TE, et al: Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood 2001; 98: 1842–1846.
Mannucci PM, Canciani MT, Forza I, Lussana F, Lattuada A, Rossi E : Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood 2001; 98: 2730–2735.
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Zhang, B., Xing, C., Yu, X. et al. Renal Thrombotic Microangiopathies Induced by Severe Hypertension. Hypertens Res 31, 479–483 (2008). https://doi.org/10.1291/hypres.31.479
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DOI: https://doi.org/10.1291/hypres.31.479
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