Semin Thromb Hemost 2005; 31(6): 673-680
DOI: 10.1055/s-2005-925473
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

The Clinical Spectrum of Thrombotic Thrombocytopenic Purpura

Hau C. Kwaan1 , 2 , Lisa N. Boggio2
  • 1Professor of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
  • 2Division of Hematology/Oncology, Northwestern University Feinberg School of Medicine, Chicago, Illinois
Further Information

Publication History

Publication Date:
30 December 2005 (online)

ABSTRACT

The clinical syndrome of fever, neurologic abnormalities, renal impairment with laboratory findings of thrombocytopenic and microangiopathic hemolytic anemia is seen in thrombotic thrombocytopenic purpura (TTP) and a variety of disorders associated with thrombotic microangiopathy (TMA). With improved understanding of the pathogenesis of the perturbed metabolic pathway of von Willebrand factor in TTP, the classic Moschcowitz syndrome, now more accurately referred to as idiopathic TTP, can be distinguished from other TMAs. The distinguishing features are useful not only in providing an accurate diagnosis but also help to determine the best therapeutic strategy.

REFERENCES

  • 1 Symmers W C. Thrombotic microangiopathic haemolytic anaemia.  Br J Haematol. 1952;  2 897-903
  • 2 Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease.  Proc NY Pathol Soc. 1924;  24 21-24
  • 3 Baehr G, Klemperer P, Schlifrin A. An acute febrile anaemia and thrombotic purpura with diffuse platelet thrombosis of capillaries and arterioles.  Trans Assoc Am Physicians. 1936;  51 43-58
  • 4 Asada Y, Sumiyoshi A, Hayashi T, Suzumiya J, Kaketani K. Immunohistocytochemistry of vascular lesion in thrombocytopenic thrombocytopenic purpura with special reference to factor VIII related antigen.  Thromb Res. 1985;  38 469-479
  • 5 Ridolfi R L, Bell W R. Thrombotic thrombocytopenic purpura: a report of 25 cases and a review of the literature.  Medicine (Baltimore). 1981;  60 413-428
  • 6 Hosler G A, Cusumano A M, Hutchins G M. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases.  Arch Pathol Lab Med. 2003;  127 834-839
  • 7 Shidham V B, Chang C C, Schidham G et al.. Colon biopsies for evaluation of acute graft-versus-host disease (AGVHD) in allogenic bone marrow transplant patients.  BMC Gastroenterol. 2003;  3 5-XX
  • 8 Blecher T E, Roper A B. Early diagnosis of thrombotic microangiopathy by paraffin sections of aspirated bone marrow.  Arch Dis Child. 1967;  42 158-162
  • 9 Petitt R M. Thrombotic thrombocytopenic purpura: A thirty year review.  Semin Thromb Hemost. 1980;  6 350-355
  • 10 Rossi E. Thrombotic thrombocytopenic purpura. In: Wu K Pathophysiology and Management of Thromboembolic Disorders. Littleton, MA; 1984: 219-231
  • 11 Kwaan H C. The pathogenesis of thrombotic thrombocytopenic purpura.  Semin Thromb Hemost. 1979;  5 184-198
  • 12 Torok T J, Holman R C, Chorba T I. Increasing mortality from thrombotic thrombocytopenic purpura in the United States: analysis of national mortality data, 1969-1991.  Am J Hematol. 1995;  50 84-90
  • 13 Miller D P, Kaye J A, Shea K et al.. Incidence of thrombotic thrombocytopenic purpura/ hemolytic uremic syndrome.  Epidemiology. 2004;  15 208-215
  • 14 Terrell D R, Williams L A, Vesely S K et al.. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients and patients with severe ADAMTS-13 deficiency.  J Thromb Haemost. 2005;  3 1432-1436
  • 15 Kennedy S S, Zacharski L R, Beck J R. Thrombotic thrombocytopenic purpura: analysis of 48 unselected cases.  Semin Thromb Hemost. 1980;  6 341-349
  • 16 Raife T J, Lentz S R, Atkinson B S, Vesely S K, Hessner M J. Factor V Leiden: a genetic risk factor for thrombotic microangiopathy in patients with normal von Willebrand factor-cleaving protease activity.  Blood. 2002;  99 437-442
  • 17 Hymes K B, Karpatian S. Immunodeficiency virus infection and thrombotic microangiopathy.  Semin Hematol. 1997;  34 117-125
  • 18 Novitzky N, Thomson J, Abrahams L, du Toit C, McDonald A. Thrombotic thrombocytopenic purpura in patients with retroviral infection is highly responsive to plasma infusion therapy.  Br J Haematol. 2005;  128 373-379
  • 19 Sahud M A, Claster S, Liu L et al.. Von Willebrand factor-cleaving protease inhibitor in a patient with human immunodeficiency syndrome-associated thrombotic thrombocytopenic purpura.  Br J Haematol. 2002;  116 909-911
  • 20 Gruszecki A C, Wehrli G, Ragland B D et al.. Management of a patient with HIV infection-induced anemia and thrombocytopenia who presented with thrombotic thrombocytopenic purpura.  Am J Hematol. 2002;  69 228-231
  • 21 Nicol K K, Shelton B J, Knovich M A, Owen J. Overweight individuals are at increased risk for thrombotic thrombocytopenic purpura.  Am J Hematol. 2003;  74 170-174
  • 22 Ducloy-Bouthers A S, Caron C, Subtil D et al.. Thrombotic thrombocytopenic purpura and biological monitoring of six pregnancies.  Eur J Obstet Gynecol Reprod Biol. 2003;  111 146-152
  • 23 Amorosi E L, Ultmann J E. Thrombotic thrombocytopenic purpura: Report of 16 cases and review of the literature.  Medicine (Baltimore). 1966;  45 139-159
  • 24 Lukes R J, Rath C E, Steussy C N et al.. Thrombotic thrombocytopenic purpura: Clinical and pathologic findings in 49 cases.  Blood. 1961;  17 366 , (abst)
  • 25 Bukowski R. Thrombotic thrombocytopenic purpura: a review.  Prog Hemost Thromb. 1982;  6 287-337
  • 26 Kwaan H C, Domer P H. Fatal thrombotic thrombocytopenic purpura despite plasmaphersis. A case report of autopsy findings.  Haemostasis. 1989;  19 344-347
  • 27 Park S A, Lee T K, Sung K B, Park S K. Extensive brain stem lesions in thrombocytopenic thrombocytopenic purpura: repeat magnetic resonance findings.  J Neurol. 2005;  15 79-81
  • 28 D'Aprile P, Farchi G, Pagliarulo R, Carella A. Thrombotic thrombocytopenic purpura: MR demonstration of reversible brain abnormalities.  AJNR Am J Neuroradiol. 1994;  15 19-20
  • 29 Blum A S, Drislane F W. Nonconvulsive status epilepticus in thrombotic thrombocytopenic purpura.  Neuroimaging. 1996;  47 1079-1081
  • 30 Garrett W T, Chang C W, Bleck T P. Altered mental status in thrombotic thrombocytopenic purpura is secondary to non-convulsive status epilepticus.  Ann Neurol. 1996;  40 245-246
  • 31 Mecozzi G, Milano A D, De Carlo M et al.. Intravascular hemolysis in patients with new-generation prosthetic heart valves: a prospective study.  J Thorac Cardiovasc Surg. 2002;  123 550-556
  • 32 Zomas A, Saso R, Powles R et al.. Red cell fragmentation (schistocytosis) after bone marrow transplantation.  Bone Marrow Transplant. 1998;  22 777-780
  • 33 Atkins J N, Muss H B. Schistocytes in erthroleukemia.  Am J Med Sci. 1985;  289 110-113
  • 34 Burns E R, Lou Y, Pathak A. Morphologic diagnosis of thrombotic thrombocytopenic purpura.  Am J Hematol. 2004;  75 18-21
  • 35 Daram S R, Philipneri M, Puri N, Bastani B. Thrombotic thrombocytopenic purpura without schistocytes on the peripheral blood smear.  South Med J. 2005;  98 392-395
  • 36 Fava S, Galizia A C. Thrombotic thrombocytopenic purpura-like syndrome in the absence of schistocytes.  Br J Haematol. 1995;  89 643-644
  • 37 Brilliant S E, Lester P A, Ohno A K et al.. Hemolytic-uremic syndrome without evidence of microangiopathic hemolytic anemia on peripheral blood smear.  South Med J. 1996;  89 342-345
  • 38 Lee J-L, Lee J-H, Kim M-K et al.. A case of bone marrow necrosis with thrombotic thrombocytopenic purpura as a manifestation of occult colon cancer.  Jpn J Clin Oncol. 2004;  34 476-480
  • 39 Remuzzi G, Galbusera M, Salvsdori M et al.. Bilateral nephrectomy stopped disease progression in plasma-resistant hemolytic uremic syndrome with neurological signs and coma.  Kidney Int. 1996;  49 282-286
  • 40 Kwaan H C. Clinicopathologic features of thrombotic thrombocytopenic purpura.  Semin Hematol. 1987;  24 71-81
  • 41 Muniz A E, Barbee R W. Thrombotic thrombocytopenic purpura (TTP) presenting as pancreatitis.  J Emerg Med. 2003;  24 407-411
  • 42 Singh R, Saunders B, Scopelitis E. Pancreatitis leading to thrombotic thrombocytopenic purpura in systemic lupus erythematosus: a case report and review of literature.  Lupus. 2003;  12 136-139
  • 43 Talwalkar J A, Ruymann F W, Marcoux P, Farraye F A. Recurrent thrombotic thrombocytopenic purpura (TTP) as a complication of acute relapsing pancreatitis.  Dig Dis Sci. 2002;  47 1096-1099
  • 44 Varadarajulu S, Ramsey W H, Israel R H. Thrombotic thrombocytopenic purpura in acute pancreatitis.  J Clin Gastroenterol. 2000;  31 243-245
  • 45 Vergara M, Modolell I, Puig-Divi V, Guarner L, Malagelada J R. Acute pancreatitis as triggering factor for thrombotic thrombocytopenic purpura.  Am J Gastroenterol. 1998;  93 2215-2218
  • 46 Berlin I, Sinclair C L, Richman L J et al.. Thrombotic thrombocytopenic purpura.  Va Med Monthly. 1957;  84 293-295
  • 47 Chung E B, Gomez-Acebo J. Thrombotic thrombocytopenic purpura: a report of two cases with some unusual features.  Georgetown Med Bull. 1960;  14 137-141
  • 48 Comess O H, Oyamada A. Uremic and disseminated platelet-cell thrombosis.  Arch Intern Med. 1952;  89 802-811
  • 49 Geisinger K R, Solomon A R. Sudden cardiac death in thrombotic thrombocytopenic purpura.  Arch Pathol Lab Med. 1979;  103 599 , (letter)
  • 50 James T N, Monto R W. Pathology of the cardiac conduction system in thrombotic thrombocytopenic purpura.  Ann Intern Med. 1966;  65 37-43
  • 51 McCrae K R, Cines D B. Thrombotic microangiopathy during pregnancy.  Semin Hematol. 1997;  34 148-158
  • 52 Vesely S K, Li X, McMinn J R, Terrell D R, George J N. Pregnancy outcomes after recovery from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.  Transfus Pract. 2004;  44 1149-1158
  • 53 Dashe J S, Ramin S M, Cunningham F G. The long term consequences of thrombotic microangiopathy (thrombotic thrombocytopenic purpura and hemolytic uremic syndrome) in pregnancy.  Obstet Gynecol. 1998;  91 662-668
  • 54 Veyradier A, Obert B, Houllier A, Meyer D, Gima J-P. Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases.  Blood. 2001;  98 1765-1772
  • 55 Zheng X L, Kaufman R M, Goodnough L T, Sadler J E. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura.  Blood. 2004;  103 4043-4049
  • 56 George J N, Li X, McMinn J R et al.. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic HPC transplantation: a diagnostic dilemma.  Transfusion. 2004;  44 294-304

Hau C KwaanM.D. 

Professor, Division of Hematology/Oncology, Northwestern University Medical School, VA Lakeside Medical Center

333 East Huron Street, Chicago, IL 60611

Email: h-kwaan@northwestern.edu

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