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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front. Med.    2016, Vol. 10 Issue (4) : 430-436     DOI: 10.1007/s11684-016-0492-5
RESEARCH ARTICLE |
Diagnosis and management of acquired thrombotic thrombocytopenic purpura in southeast China: a single center experience of 60 cases
Xinping Zhou1,Xingnong Ye1,2,Yanling Ren1,Chen Mei1,Liya Ma1,Jiansong Huang1,Weilai Xu1,Juying Wei1,Li Ye1,Wenyuan Mai1,Wenbin Qian1,Haitao Meng1,Jie Jin1,Hongyan Tong1()
1. Department of Hematology, the First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310003, China
2. Department of Hematology, the Forth Affiliated Hospital, College of Medicine, Zhejiang University, Yiwu 322000, China
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Abstract  

Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening thrombotic microangiopathy. This study aimed to provide a profile of the diagnosis and management of patients with acquired TTP collected in 10 years in a single center in southeast China. A total of 60 patients diagnosed with acute acquired TTP from March 2005 to August 2015 were enrolled. Among the 60 patients, 52 patients presented with their first episodes, and eight patients had two or more episodes. The median age at presentation was 49 (range, 17 to 78) years with a female predominance (male:female ratio, 1:1.60). ADAMTS 13 activity were analyzed in 43 patients, among whom 33 (76.7%) patients had a baseline level of<5%. Mortality was 30%. Plasma exchange (PEX) was performed in 62 of 69 (89.9%) episodes. Corticosteroids were administered in 54 of 69 (78.3%) episodes. Other immunosuppressants (e.g., vincristine, cyclosporine, and cyclosporin) were used in 7 of 69 (10.1%) episodes. Rituximab was documented in 4 patients with refractory/relapsed TTP for 5 episodes, showing encouraging results. In conclusion, the diagnosis of TTP depended on a comprehensive analysis of clinical data. Plasma ADAMTS13 activity assay helped confirm a diagnosis. PEX was the mainstay of the therapy, and rituximab can be used in relapsed/refractory disease.

Keywords thrombotic thrombocytopenic purpura      ADAMTS 13      plasma exchange     
Corresponding Authors: Hongyan Tong   
Just Accepted Date: 07 November 2016   Online First Date: 23 November 2016    Issue Date: 01 December 2016
URL:  
http://academic.hep.com.cn/fmd/EN/10.1007/s11684-016-0492-5     OR     http://academic.hep.com.cn/fmd/EN/Y2016/V10/I4/430
Clinical manifestation Number of patients (%)
Fever 40 (66.67)
Hemorrhagic manifestations 18 (30)
Cutaneous 13 (21.67)
Cenorrhagia 2 (3.33)
Gastrointestinal 2 (3.33)
Central nervous system 1(1.6)
Hematuresis 4 (6.67)
Neurological manifestations 46 (76.66)
Headache 7 (11.67)
Cognitive change 9 (15)
Focal deficit 5 (10.67)
Syncope 2 (3.33)
Visual disturbance 3 (5)
Seizure 10 (16.67)
Stroke 1 (1.6)
Coma 16 (26.66)
Other 2 (3.33)
Renal manifestations 10 (16.67)
Gastrointestinal manifestations 14 (23.33)
Nausea/vomiting 10 (16.67)
Abdominal pain 3 (5)
Diarrhea 2 (3.33)
MAHA 55 (91.7)
Thrombocytopenia 60 (100)
Tab.1  Clinical manifestations of cohort of 60 patients
Clinical features Survivor group (n = 42) Death group (n = 18) P value
Fever 25 15 0.073
Hemorrhagic manifestations 8 10 0.005*
Neurological manifestations 30 16 0.192
Coma 9 7 0.207
Renal manifestations 2 8 0.001*
Gastrointestinal manifestations 9 5 0.736
MAHA 37 18 0.309
Pentad 0 6 0.000*
Tab.2  Clinical manifestations between patients with different outcomes
Treatment Efficacy in episodes of initial onset [n (%)] Efficacy in episodes of relapsed disease [n (%)]
Total number
(n = 60)
Improved Died Total number
(n = 9)
Improved Died
PEX/PI 12 4 (33.33) 8 (66.67) 1 1 (100) 0 (0)
Glucocorticoid 3 0 (0) 3 (100) 0 0 (0) 0 (0)
PEX/PI+ glucocorticoid 37 33 (89.19) 4 (10.81) 3 3 (100) 0 (0)
PEX/PI+ glucocorticoid+ immunosuppressor 6 4 (66.67) 2 (33.33) 1 1 (100) 0 (0)
PEX/PI+ glucocorticoid+ rituximab 1 1 (100) 0 (0) 3 3 (100) 0 (0)
Rituximab 0 0 (0) 0 (0) 1 1 (100) 0 (0)
Untreated 1 0 (0) 1 (100) 0 0 (0) 0 (0)
Tab.3  Treatments and outcomes of 60 patients
Clinical features Survivor group (n = 42) Death group (n = 18) P value
Age (year) 45.9±15.81 55.1±15.18 0.043
Lactate dehydrogenase (U/L) 1166.2±802.6 1734.4±926.7 0.030*
Hemoglobin (g/L) 71.4±19.6 72.2±11.0 0.886
Platelet (×109/L) 17.0±9.1 11.2±5.6 0.021*
Total bilirubin (mmol/ L) 66.1±40.5 80.1±26.7 0.396
Creatinine (mmol/L) 103.7±60.5 171.7±224.5 0.459
Urea nitrogen (mmol/ L) 11.2±5.4 15.06±9.8 0.458
Alkaline phosphatase (U/L) 32.4±20.2 52.8±6.9 0.021*
Tab.4  Comparison of clinical features between patients with different outcomes
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