T.T.P.

This young woman is suffering from a hemolytic anemia with fragmented RBC's in the absence of D.I.C. She also has thrombocytopenia, fever, mental status changes and renal dysfunction, which is essentially pathognomonic of thrombotic thrombocytopenic purpura. The etiology of TTP is unknown, though immunologic and primary vascuolopathic phenomena have been associated with this disorder. Pathologically, arteriolar hyalinization, which is also seen in D.I.C., may be noted. Plasma exchange decreases mortality rates from 90% (1) to approximately 10-30% (2,3). Despite successful treatment with plasmapheresis, it is estimated that approximately 36% have a recurrence over a 10 year period (4). Glucocorticoids, antiplatelet agents, splenectomy, and vincristine have been of benefit to subsets of patients, but each is less effective and probably associated with a greater risk than therapeutic plasmapheresis.

References:

  1. Moschowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. Arch Intern Med. 1925;36:89-93.
  2. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survial in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. N Engl J Med. 1991;325:398-403.
  3. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991; 325:393-7.
  4. Shumak KH, Rock GA, Nair RC and the Canadian Apheresis Group. Late Relapses in patients successfully treated for thrombotic thrombocytopenic purpura. Ann Intern Med. 1995;122:569-72.
  5. Thompson, Blood 80: 1890, 1992.

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