Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura

Abstract

The current established treatment of thrombotic thrombocytopenic purpura (TTP) is plasma exchange with fresh frozen plasma (FEP). With this treatment, there is a 49% response after seven exchanges and a 78% survival at 1 month. Although the exact cause of TTP is unknown, the presence of von Willebrand factor (VWF) multimers has been implicated in the disease. Accordingly, it has been suggested that cryosupernatant (plasma from which cryoprecipitate has been removed), which is relatively deficient in VWF multimers, might be an effective replacement fluid during plasma exchange. Patients from six centers were treated by plasma exchange with cryosupernatant. 18 patients who had failed a first course (average 7.7 exchanges) of plasma exchange with FFP. received a further seven exchanges with cryosupernatant. Subsequently, 40 previously untreated patients were exchanged with cryosupernatant. Of the 18 previously treated patients, 11 responded (defined as an increase in platelet count to > 150 x 10(9) /1 and no neurological events) after seven exchanges and 15 (83%) of the patients were alive at 1 month. The response rate in the 40 previously untreated patients was 75% at the end of seven exchanges and 95% of the patients were alive at 1 month. These values are significantly different (P < 0.05) from those reported in our earlier study and in other patients concurrently treated at the same centres with FFP when cryosupernatant was not available. Some patients who have failed to respond to plasma exchange with FFP replacement will respond to further exchange with cryosupernatant. Cryosupernatant replacement may be more effective as first-line treatment of TTP than FFP.

High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study

“…Fresh-frozen plasma was used as replacement fluid in the first procedure, while cryosupernatant (Rock et al, 1996) was used in the following exchanges. Cryosupernatant was used based on a pilot study by the Canadian Apheresis Group suggesting that this replacement fluid was more effective than fresh frozen plasma [7].…”

Transplantation-associated thrombotic microangiopathy: twenty-two years later

“…40 While response rates as high as 75% have been reported in classical TTP, 41 response rates in TA-TMA have been considerably lower and long-term survival rates have been disappointing (see Table 3). Overall, just under half of patients in nine reported case series have achieved a complete or partial response to plasma exchange according to a variety of response criteria.…”

Current Concepts in Thrombotic Thrombocytopenic Purpura

“…Because large VWF multimers are involved in causing platelet aggregation, cryosupernatant of fresh frozen plasma has been advocated as a more effective alternative of fresh frozen plasma (67,68). Cryosupernatant plasma contains the same amount of ADAMTS13 as fresh frozen plasma, and is not expected to be more effective in raising the ADAMTS13 levels.…”