성인 혈전성 미세혈관병증에 관한 임상적고찰

Abstract

Objectives: Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are rare clinical entities characterized by platelet thrombi occluding the microvasculature. These two entities have been linked to similar precipitating factors, leading many to favor the notion that they actually represent different clinical manifestations of same disease that is currently referred to as thrombotic microangiopathy. In the early reports, the mortality from thrombotic microangiopathy was nearly 100%. Since the advent of plasma therapy patient's survival has been improved greatly.

Methods: From January 1989 to August 1993, 12 adult patients with thrombotic microangiopathy were evaluated with respect to etiology, clinical and laboratory data at the time of admission and response to therapy.

Results: They ranged in age from 22 to 59 years old (mean age 36.3 years) with female preponderance (Female: male=5:l) In three patients, thrombotic microangiopahy was associated diarrhea, whereas in nine, it was associated with another disease (2 with lupus erythematosus, 2 with cancer, 2 with cyclosporine A, each 1 with pregnancy, poststreptococcal glomerulonephritis and mitomycin). Five cases had prodromal illness characterized by abdominal pain or bloody diarrhea, six had seizure. Mean laboratory values at presentation were follows, Hb 5.7g/dl, PLT 48,750/mm3, WBC 20,728/mm3, BUN 117 mg/dl, creatinine 7.8 mg/dl, bilirubin 3.2 mg/dl, AST 447.0 U/L, ALT 338.0 U/L. LDH 670.5 U/L, uric acid 10.9 mg/dl, all cases had proteinuria and eight cases with microscopic hematuria.

Eight (67%) patients were treated with plasma manipulation, i.e, plasma infusion, plasma pheresis, or both; seven (58%) patients received blood transfusions in forms of whole blood or packed red cells because of severe anemia. Other forms of treatment were steroids (9 patients) and intravenous infusion of r-globulin (2 patients). Eight (67%) patients required hemodialysis during the acute phase of the disease. Of the 12 patients, six (50%) had recovered a normal renal function, one had persistant renal failure. Five (42%) patients died due to either underlying disease or CNS insufficiency.

Conclusion: Early diagnosis and introduction of plasmapheresis and plasma infusion seems to increase the survival rate. Key Words: Thrombotic microangiopathy, Hemolytic uremic syndrome, Thrombotic thrombocytopenic purpura