VolumeVol. 53 • Number No. 1 • January - March 2015 Back

Original Paper

​​​​​​​Clinical and Laboratory Profile of Patients with Thrombotic Thrombocytopenic Purpura Seen at the University of Sto. Tomas Hospital: A Single Center Experience

Authors: Pamela Rose L. Mancio, Ma. Rosario Irene D. Castillo

DOI: 10.65564/pjim.c7508b0111

Date of Publication: March 2015


Abstract

Background: Thrombotic thrombocytopenic purpura (TTP) is considered a rare disease. In the Philippines, there are currently no local registry for such rare disease, thus clinical features that may be unique to the country is difficult to characterize.

Objective: To characterize the TTP patients seen in our institution based on the demographic, clinical and laboratory profile of these patients.

Results: A total of eight patients were described in this report. Median age was 38 years (range, 20- 63) with a female predominance. All patients had neurologic symptoms and microangiopathic hemolytic anemia. Minor neurologic symptoms (confusion, headache and dizziness) were more frequently observed. Renal dysfunction (serum creatinine >1.4 mg/dL), fever (>38.0°C), and thrombocytopenia were observed in four patients (50%), seven patients (87.5%), and six patients (75%), respectively. Patients underwent median of three sessions of Plasma Exchange (range, two to 38). Of the six patients who received therapeutic plasma exchange, five were discharged alive.

Conclusion: Fifty percent of the patients in this report presented with the classic pentad. This feature should be kept in mind as TTP is a true hematologic emergency. A high index of suspicion, prompt evaluation and treatment of patients are necessary because multi-organ thrombosis may not be reversible, if treatment is not urgently administered. Therapeutic Plasma Exchange is the management of choice and should be done immediately once TTP is recognized.

Keywords: Thrombotic Thrombocytopenic Purpura, Therapeutic Plasma Exchange


References

  1. Tsai HM. Pathophysiology of TTP. Int J Hematol 2010; 91:1-19.
  2. Harrison’s Principles of Internal Medicine 18th ed.
  3. Miller DP, Kaye JA, Shea K, et al: Incidence of thrombotic thrombocytopenic purpura/Hemolytic uremic syndrome. Epidemiology 15:208, 2004
  4. Rose M, Eldor A. High incidence of relapses in thrombotic thrombocytopenic purpura: clinical study of 38 patients. Am J Med 1987;83:437-44.
  5. J.W. Kim, I. Kim, S.S. Yoon, K.S. Han, S. Park, B.K. Kim. Clinical Outcome And Risk Factor Analyses In Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Patients Who Underwent Therapeutic Plasma Exchange. Haematologica 2009; 94[suppl.2]:316 abs. 0779.
  6. Marie Scully, Helen Yarranton, Ri Liesner, et al. Regional UK TTP Registry: correlation with laboratory ADAMTS 13 analysis and clinical features. British Journal of Haematology Volume 142, Issue 5, pages 819–826, September 2008
  7. Christopher D. Hillyer, Beth H. Shaz, James C. Zimring and Thomas C. Abshire, ed. Transfusion Medicine and Hemostasis.
  8. Petri Salven2, Arto Orpana and Heikki Joensuu. Leukocytes and Platelets of Patients with Cancer Contain High Levels of Vascular Endothelial Growth Factor. Clin Cancer Res March 1999 5; 487
  9. Han-Mou Tsai. The Kidney in Thrombotic Thrombocytopenic Purpura. Minerva Med. 2007 December ; 98(6): 731–737.
  10. Ridolfi RL, Bell MD. Thrombotic thrombocytopenic purpura: report of 25 cases and review of the literature. Medicine (Baltimore) 1981; 60: 413-28
  11. James N. George, MD. How I treat Thrombotic thrombocytopenic purpura. Blood. 2000;96:1223-1229
  12. Gail Rock, Camillo Porta, Enrico Bobbio-Pallavicini. Thrombotic thrombocytopenic purpura treatment in year 2000. Haematologica 2000; 85:410-419.
  13. Rock GA, Shumak KH, Buskard NA, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991; 325:393-7.