Correspondence

More about Thrombotic Thrombocytopenic Purpura

N Engl J Med 1998; 338:548February 19, 1998

Article

To the Editor:

Kupfer and Tessler (Oct. 23 issue)1 report a possible association of ticlopidine with fatal thrombotic thrombocytopenic purpura in two patients, but they imply that platelet transfusion is standard therapy for thrombotic thrombocytopenic purpura. In fact, the report illustrates the potential hazard of platelet transfusion for the condition. Platelet transfusions have been temporally associated with severe exacerbations of thrombotic thrombocytopenic purpura,2,3 including rapid deterioration and death, as occurred in one of the women described by Kupfer and Tessler, who died within six hours after platelet transfusion. The most likely mechanism for the adverse effect appears to be exacerbation of the microcirculatory thrombotic process.4 Although it is often difficult to determine the exact cause of bad outcomes in patients with complicated illnesses, platelet transfusion should generally be avoided in patients with thrombotic thrombocytopenic purpura.

John V.L. Sheffield, M.D.
Ed Merrens, M.D.
Harborview Medical Center, Seattle, WA 98104

4 References

1. 1

   Kupfer Y, Tessler S. Ticlopidine and thrombotic thrombocytopenic purpura. N Engl J Med 1997;337:1245-1245
   Full Text | Web of Science | Medline

2. 2

   Harkness DR, Byrnes JJ, Lian EC, Williams WD, Hensley GT. Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA 1981;246:1931-1933
   CrossRef | Web of Science | Medline

3. 3

   Gordon LI, Kwaan HC, Rossi EC. Deleterious effects of platelet transfusions and recovery thrombocytosis in patients with thrombotic microangiopathy. Semin Hematol 1987;24:194-201
   Web of Science | Medline

4. 4

   Byrnes JJ, Moake JL. Thrombotic thrombocytopenic purpura and the haemolytic-uraemic syndrome: evolving concepts of pathogenesis and therapy. Clin Haematol 1986;15:413-442
   Medline

Author/Editor Response

The authors reply:

To the Editor: The purpose of our report was to alert the medical community to the association between ticlopidine (Ticlid) and fatal thrombotic thrombocytopenic purpura in young women and not to discuss the treatment of thrombotic thrombocytopenic purpura. We agree with Drs. Sheffield and Merrens that platelet transfusions generally have no role in the treatment of thrombotic thrombocytopenic purpura. Indeed, a recent analysis concluded that there is increased mortality among patients with thrombotic thrombocytopenic purpura who are receiving platelets.1 This increase may be due to worsening organ failure secondary to increased microvascular thrombi formation induced by circulating platelet aggregates.

We agree that plasmapheresis is the appropriate therapy for patients with thrombotic thrombocytopenic purpura. Unfortunately, at the time that our patients were being prepared for plasmapheresis, they were in severe hemorrhagic shock, were bleeding from multiple sites, and had severe thrombocytopenia. The surgeons were afraid to insert the wide-bore vascular catheter that is required for plasmapheresis without platelet transfusions. Platelets were not transfused before this. Despite our attempts at plasmapheresis, the patients died.

Yizhak Kupfer, M.D.
Sidney Tessler, M.D.
953 49th St., Brooklyn, NY 11219

1 References

1. 1

   McCarthy LJ, Danielson CF, Graves V, et al. Do platelet transfusions to patients with TTP influence their survival? Blood 1994;84:Suppl 1:669a-669a abstract.
   Web of Science

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1. 1

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