Therapeutic cytapheresis: too many platelets, too many white blood cells.

Myeloproliferative disorders may present with thrombocytosis and/or leukocytosis with symptoms ranging from minor to life-threatening. Therapeutic cytapheresis (TC), the removal of excess and/or abnormal cells such as platelets and white blood cells, can be used to rapidly reduce cells in the symptomatic patient. The patient's clinical status determines the need for TC as symptoms may not correlate to laboratory values. TC does not alter the underlying disease process but serves as a bridge until drug therapy is initiated. Because of low frequency of procedures performed and the often urgent need for treatment, clinical data for the use of TC are difficult to collect and analyze. American Society for Apheresis Clinical Applications Committee has described indications for TC for treatment of disease entities. These publications provide guidelines for these less commonly performed procedures. Maintaining staff education, competency, and proficiency for these urgent procedures is an ongoing challenge for the majority of therapeutic apheresis programs.

Twice-daily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006.

BACKGROUND: Twice-daily plasma exchange has been used for patients with thrombotic thrombocytopenic purpura (TTP) who are unresponsive to treatment with daily plasma exchange for many years but with no clear evidence of effectiveness. STUDY DESIGN AND METHODS: The 18 years' experience of The Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry, 1989 through 2006, with twice-daily plasma exchange for 31 episodes of TTP in 28 patients is reported. A definite response to twice-daily plasma exchange was defined a priori as a platelet (PLT) count increase after twice-daily plasma exchange on two separate occasions during the treatment of a single episode of TTP, with no change of other treatments. A possible response was defined as a PLT count increase after initiation of twice-daily plasma exchange only once with or without change of other treatments. RESULTS: A definite response to twice-daily plasma exchange occurred in 3 episodes (three patients), 27 episodes had a possible response, and 1 episode had no response. The three patients with a definite response had ADAMTS13 activities of 5, 6, and 12 percent and all had an inhibitor; the patient with no response was subsequently determined to have Rocky Mountain spotted fever. CONCLUSION: Twice-daily plasma exchange was typically considered in acutely ill patients who had initially responded but then severe thrombocytopenia recurred, often with new neurologic abnormalities, while continuing daily plasma exchange. In three patients, twice-daily plasma exchange appeared to be beneficial. In most patients, a benefit of twice-daily plasma exchange could not be clearly documented because other treatments were initiated or intensified.

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry: a community service.

The Oklahoma Thrombotic ThrombocytopenicPurpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry has enrolled all 335 consecutive patients from central, western and southeastern Oklahoma who had their first episode of clinically diagnosed TTP or HUS from 1989 through 2005; follow-up is complete for 333 patients. Identifying all patients is possible since plasma exchange is the essential treatment for all adults and some children with these disorders and since the Oklahoma Blood Institute (OBI) is the sole provider of plasma exchange services for this region. Patients in the Oklahoma TTP-HUS Registry have come from 46 counties and have been treated in 16 hospitals; 227 physicians have provided the principal care for these patients. A support group formed in 1996 has met 3 times each year, averaging 17 former patients plus 16 additional family members and friends. Registry data are an important source of information for physicians and their patients, providing a complete community perspective of these uncommon disorders.

A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): The six-year experience of the Oklahoma TTP-HUS Study Group.

A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), named The Oklahoma TTP-HUS Study Group, has been a successful program for 6 years. This group has met 3 times each year with an average attendance of 16 former patients; in addition, an average of 14 family members and friends have attended each meeting. Eighty-four percent of patients who attended a meeting were women as compared to 68% women among those who did not attend (P = 0.016). Twenty-three percent of patients who attended a meeting have relapsed as compared to 8% among those who did not attend (P = 0.006). There was also a difference in the rates of attendance among the different clinical categories of patients (P < 0.001). A survey of former patients, as well as their families and friends, documented that interest in learning more about TTP-HUS and talking with other people who have had TTP-HUS were principal motivations for attending the meetings. The meetings of The Oklahoma TTP-HUS Study Group have been successful not only for support of former patients but also for research. Long-term patient follow-up has been facilitated and the group discussions have revealed previously unreported persistent problems with cognitive ability and endurance. A survey of member centers of the American Association of Blood Banks and America's Blood Centers revealed no similar programs for patients who have recovered from TTP-HUS. The absence of a support group for TTP-HUS in the national survey contrasts to the 274 patient support groups and related wellness/education classes, including some for rare disorders, currently active in the Oklahoma City metropolitan area. The experience of The Oklahoma TTP-HUS Study Group suggests that it serves a previously unmet need.