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Plasmapheresis should utilize FFP or cryosupernatant (depleted of vWF, fibrinogen, factor XIII)
If a delay in institution of plasma exchange is unavoidable for technical reasons, appropriate to initiate therapy
with plasma infusion
Plasmapheresis should continue for a few days after the platelet count, serum LDH , blood smear, and
neurologic status have normalized. Plasma exchange should then be tapered over a period of 1 to 2 weeks. 1/3
of patients will have exacerbation of disease.
Plasmapheresis most common complication bacteremia, catheter problems, allergic response, hypotension,
citrate toxicity (paresthesias, tetany)
Contraindication of platelet transfusions: 55pts with TTP, 13/25 (52%) died in those who received platelets vs.
2/30 (7%) dying in those who did not receive platelets.
Glucocorticoids: before era of plasma exchange, 10% response rate. Appropriate in initial management
Adjunctive therapy with possible but unproven benefit (steroids, dipyridamole, ASA, azathioprine, vincristine)
Salvage splenectomy of unclear benefit but may be most helpful in preventing relapses
If poor response to daily plasma exchanges, increase number of exchanges, add prednisone, try infusion of
cryosupernatant of plasma, consider splenectomy
(UCSF experience: Daily plasma exchanges (60-80 ml/kg/d), replaced with FFP. Median # of exchanges for
TTP was 9)
Outcomes: order of recovery: neuro sx -> LDH -> platelet ct -> renal function
* 78% overall survival (no difference in HIV patients) with plasma exchange but only 50% with plasma infusion
* Those with previous splenectomy had 100% response rate
* Relapse most common within first 60 days after tx
* In a series of 319 pts, 64% experienced a relapse, most of whom (84%) had a single relapse. No predictors of
patients likely to relapse.
* Pts who have had TTP should have indefinite regular monitoring of blood parameters.
* Relapse did not confer a state of resistence as the number of plasmapheresis txs required did not differ from the
number needed for the first remission.
Sources: 1) Bill Plauth. Cover Sheet: Thrombotic Thrombocytopenic Purpura 2) Thompson, CE. Thrombotic Microangiopathies in the 1980s:
Clinical Features, Response to Treatment, and the Impact of the Human Immunodeficiency Virus Epidemic. Blood. October 1992; 80 (8): 18901895. 3) Kwaan, HC. Management of Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome. Seminars in Hematology. April
1997; 34(2): 159-166. 4) Bell, WR. Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome Relapse: Frequency, Pathogenesis, and
Meaning. Seminars in Hematology. April 1997; 34 (2). 5) UpToDate 2002 on TTP 6) Ferrara, et al. Vincristine in the treatment of TTP. Annals
of hem. Jan 2002.