Thrombotic Thrombocytopenic Purpura (TTP)

3/01/00 (Doyle)

Group: Wednesday Residents

RE: A 31 year old female with headache, back pain, and low urine output progressing to anuria.

Question: What are the criteria for a diagnosis of thrombotic thrombocytopenic purpura?

<3> Link Directly to Fulltext Article at Science Direct

Unique Identifier: 20088364

Authors: Lara PN Jr. Coe TL. Zhou H. Fernando L. Holland PV. Wun T.

Institution: Division of Hematology-Oncology, University of California Davis School of Medicine, UC Davis Cancer Center, Sacramento 95817, USA.

Abstract: PURPOSE: Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are uncommon disorders that are generally fatal if left untreated. Plasma exchange therapy is associated with high response rates and improved short-term survival, but most previous studies have been limited by small numbers of patients or short duration of follow-up. METHODS: We performed a retrospective cohort analysis in 126 consecutive patients with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, most of whom were treated principally with plasma exchange at the Sacramento Medical Foundation Blood (Center and the University of California Davis Medical Center between 1978 and 1998. We measured the effect of therapeutic plasma exchange on 30-day mortality, response rate, and overall survival, and determined which factors were associated with 30-day mortality and relapse. RESULTS: The overall 30-day mortality was 10% of the 122 patients who received plasma exchange as their principal treatment (a median of 9 exchanges and a mean cumulative infused volume of 43 +/- 77 L fresh frozen plasma); 56% were complete responders and 21% were partial responders. The relapse rate was 13%. The estimated 2-year survival was about 60%; among patients without serious underlying comorbid conditions, the estimated 2-year survival was about 80%. Each unit increase in clinical severity score (on a 0 to 8 scale) was associated with a 2.2-fold (95% confidence interval [CI]: 1.3 to 3.9) increase in the odds of 30-day mortality. Patients who were febrile at presentation were substantially less likely to suffer a relapse (odds ratio = 0.2; 95% CI: 0.03 to 0.9). CONCLUSION: Plasma exchange therapy produced high response and survival rates in this large cohort of patients with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. The Clinical Severity Score may be useful in predicting 30-day mortality, whereas fever at onset was associated with a lesser risk of relapse. Prospective studies should stratify patients according to these prognostic factors.

Link Directly to Fulltext article in Ovid

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Unique Identifier: 94056462

Authors: Lau DH. Wun T.

Institution: University of California Davis Cancer Center, Sacramento.

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Unique Identifier: 86212394

Authors: Eknoyan G. Riggs SA.

Abstract: A retrospective review of the records of 15 patients with thrombotic thrombocytopenic purpura (TTP) was performed to determine the spectrum of renal involvement that occurs in this disease. All cases exhibited some evidence of renal involvement, the most common manifestation of which was an abnormal urinalysis. Twelve cases (80%) had some degree of elevation of the serum urea nitrogen (SUN) or creatinine level at some time during the course of their disease. Renal involvement could be categorized into three types depending on the severity of TTP. In those cases presenting as an acute devastating illness, renal insufficiency, when present, was severe and a dominant component of the disease. In those that pursued a more protracted course, with subsequent acute exacerbations of TTP, renal insufficiency was variable and less severe than in the first group. In those presenting with a mild form of TTP, renal involvement consisted primarily of an abnormal urine sediment and azotemia that corrected rapidly following fluid replacement. A review of the literature beginning with 1966, when renal disease was established as part of the features that characterize TTP, provided 216 cases in whom sufficient data on renal involvement were recorded. Of the 168 cases where urinalysis was reported, hematuria was noted in 78% of the cases, proteinuria in 75, pyuria in 31, and cylindriuria in 24%. Of the 181 cases where the admission SUN was reported, it was higher than 20 mg/dl in 69% and greater than 60 mg/dl in 17% of the cases. The level of SUN was a significant determinant of the final prognosis of these patients.