Outcome and relapse risks of thrombotic thrombocytopaenic purpura: an Egyptian experience
- Noha M El-Husseiny1,
- Hady Goubran2,
- Hala M Fahmy1,
- Nehad M Tawfik1,
- Heba Moustafa1,
- Sherif N Amin3,
- Magdy El-Ekiaby4
- 1The Clinical Haematology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University, Cairo, Egypt
- 2Saskatoon Cancer Centre, Saskatoon, Canada
- 3Department of Clinical Pathology, Faculty of Medicine, Cairo University, Cairo, Egypt
- 4Shabrawishy Blood bank, Shabrawishy Hospital, Cairo, Egypt
- Correspondence to Dr Noha M El-Husseiny, The Clinical Haematology Unit, Department of Internal Medicine, Faculty of Medicine, Cairo University, Cairo, Egypt;
Contributors All authors contributed to manuscript writing and preparation. NMEH: prepared the manuscript and did all the statistical work. HG: prepared the data and participated in preparation of the manuscript. HMF:prepared the data on the patients and participated in preparation of the manuscript. NMT: participated in manuscript preparation and data collection. HM: participated in manuscript preparation and data management. SNA and MEE did all the laboratory work-up of the patients.
- Received 13 August 2011
- Accepted 4 February 2012
- Published Online First 20 March 2012
Background Thrombotic thrombocytopaenic purpura (TTP) is a rare life-threatening disease. Plasma exchange has signiﬁcantly decreased the mortality from this disease, which still tends to recur in a substantial proportion of patients. This study describes the clinical spectrum and response to treatment and explores the risks of relapse in a cohort of patients.
Methods Patients treated for TTP at the Clinical Haematology Unit, Cairo University, Egypt, between 2000 and 2008 were identiﬁed. Complete demographic, clinical history and full clinical examination, laboratory, treatment modalities and duration, and outcome data were collected and analysed. The follow-up duration was 24 months.
Results 30 patients; 13 men (43%) and 17 women (57%) with a median age of 42 years were treated for 46 episodes of TTP. The median duration of disease onset to diagnosis for the ﬁrst episode was 7 days. Twenty-three patients (76.66%) were diagnosed as idiopathic primary and seven patients (23.33%) were secondary TTP. Four patients died during the ﬁrst 24 h. Of the 26 patients, 22 (85.6%) achieved remission with an average of 7.55 plasma exchange sessions, Another nine patients had 25 relapses (mean 2.7). Splenectomy was performed in three patients (11.5%). The 24-month overall survival was 80%. The initial low platelet count and high LDH were the only two statistically signiﬁcant relapse predictors.
Conclusions The current results conform to the reported literature on the outcome of TTP. The very early mortality due to late referral highlights the need of education about the disease among primary healthcare providers.
Competing interests None.
Patient consent Obtained.
Ethics approval Ethics committee approval for the study was obtained from the Faculty of Medicine ethical committee board.
Provenance and peer review Not commissioned; externally peer reviewed.