Svetlana Stankovikj


Introduction:  Thrombotic thrombocytopenic purpura (TTP) is a syndrome that consists of the pentad of thrombocytopenia, microangiopathic hemolytic anemia, neurologic abnormalities, fever and renal disease.  Moskowitz was the first who described this syndrome in 1925, finding hyaline thrombi in many organs. The micro thrombi cause tissue ischemia, platelet consumption, and microangiopathic hemolytic anemia. Brain involvement is common and leads to stroke, seizure, confusion, and headache. Renal injury occurs in a minority of patients and it is usually modest.

Case report:  We present a 57-year old male who came to our hospital because of weakness, prostration and darkening of his urine, occurring several days before admission. On physical examination we found icteric coloring of his skin and conjunctiva, big hematoma on his right lower leg and he had neurological abnormalities presented as mild headache, disorientation and aphasia. Laboratory tests revealed anemia and thrombocytopenia and the examination of peripheral blood smear showed presence of schistocytes.  Direct and indirect antiglobulin test (Coombs) was negative. Emergency treatment was started with plasmapheresis on daily basis, immunosuppressive treatment with high-dose methyl prednisolone and transfusions of red blood cells. The laboratory results and the clinical condition improved within two weeks.

Conclusion: TTP is a medical condition that can be fatal if emergency treatment with plasma pheresis is not initiated presently after suspected diagnosis.


thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), plasma exchange

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George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014; 371(7):654-66.

Moschowitz E. An acute febrile pleochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. 1925. Mt Sinai J Med. 2003; 70(5):352-5.

Zheng XL, Salder JE. Pathogenesis of thrombotic microangiopathies. Annu Rev Pathol. 2008; 3:249-77.

Lotta LA, Garagiola I, Palla R, Cairo A, Peyvandi F. ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura. Hum Mutat. 2010; 31(1):11-9.

Esparza-Gordillo J, Jorge EG, Garrido CA, Carreras L, Lopez-Trascasa M, Sanchez-Corral P, et al. Insights into hemolytic uremic syndrome: segregation of three independent predisposition factors in a large, multiple affected pedigree. Mol Immunol. 2006;43(11):1769–75.

Booth KK, Terrell DR, Vesely SK, George Jn. Systemic infections mimicking thrombotic thrombocytopenic purpura. Am J Hematol. 2011; 86(9):743-51.

Ariceta G, Besbas N, Johnson S, Karpman D, Landau D, Licht C et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome. Pediatr Nephrol. 2009;24(4):687–96.

George JN. How I treat patients with thrombotic thrombocytopenic purpura. Blood. 2010; 116(20):4060-9.

Michael M, Chanet V, Dechartres A, Morin AS, Piette JC, Cirasino L et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009; 114(15):3167-72.

Zheng XL. ADAMTS13 testing: why bother. Blood. 2010; 115(8):1475-6.

Peyvandi F, Lavoretano S, Palla R, Feys HB, Vanhoorelbeke K, Battaglioli T et al.ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica. 2008; 93(2):232-9.

Ruggenenti P, Galbusera M, Cornejo RP, Bellavita P, Remuzzi G. Thrombotic thrombocytopenic purpura: evidence that infusion rather than removal of plasma induces remission of the disease. Am J Kidney Dis. 1993; 21(3):314-8.



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