The clinical studies of 51 patients with thrombotic thrombocytopenic purpura / 中华血液学杂志
Chinese Journal of Hematology
; (12): 147-151, 2014.
Article
ي Zh
| WPRIM
| ID: wpr-295690
المكتبة المسؤولة:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To comprehensively analyze the clinical characteristics, treatment strategies and outcome of patients with thrombotic thrombocytopenic purpura (TTP).</p><p><b>METHODS</b>A retrospective survey of 51 TTP patients confirmed in our database. Relevant statistical analyzes were performed by GraphPad Prism 5 software.</p><p><b>RESULTS</b>51 cases of patients with acquired TTP were identified as idiopathic TTP. In our study, only 18 cases (35.29%) had typical pentalogy of TTP, where thrombocytopenia (100.00%), microangiopathic hemolytic anemia (92.16%) and neurologic abnormalities (88.24%) were more common than fever (72.55%) and renal abnormalities (70.59%). Plasma ADAMTS13 activity was detected in 37 patients with TTP with ADAMTS13 deficiency confirmed in 31 patients (83.78%). Plasma exchange with response of 72.3% was still the preferred strategy in TTP with individuation. Among 36 survival TTP patients, 8 patients (22.22%) relapsed. 15 patients (29.41%) died in our study. The mean ages of responders and deaths were of (37.5±14.5) and (50.1±18.9) respectively; whereas total bilirubin level of responders and deaths were of (43.3±23.5)μmol/L and (63.7±39.7) μmol/L respectively, the differences were statistically significant. Conversely, body temperature, WBC, HGB, PLT, serum creatinine and LDH showed no significant differences (P>0.05).</p><p><b>CONCLUSION</b>The diagnosis of TTP was based on comprehensive analysis of clinical manifestations. Plasma ADAMTS13 activity test had a higher clinical practical value. The therapeutic alliance with corticosteroids, immunosuppressive agents and Rituximab significantly improved its outcome. The age and high total bilirubin level at onset were associated with less sensitive to plasmapheresis and poor prognosis.</p>
النص الكامل:
1
الفهرس:
WPRIM
الموضوع الرئيسي:
Plasma Exchange
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Purpura, Thrombotic Thrombocytopenic
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Therapeutics
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Blood
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Retrospective Studies
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Diagnosis
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ADAM Proteins
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ADAMTS13 Protein
نوع الدراسة:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
المحددات:
Adolescent
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Adult
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Aged
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Aged80
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Female
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Humans
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Male
اللغة:
Zh
مجلة:
Chinese Journal of Hematology
السنة:
2014
نوع:
Article