Abstract
Background: Thrombotic thrombocytopenic purpura (TTP) is an urgent, life-threatening hematologic disorder. It can result in a high fatality rate without early identification and prompt plasma exchange (PE) therapy. We report our experience of clinical manifestations, laboratory abnormalities, diagnosis, treatment and outcome of patients with TTP at our institution. Patients and Methods: Medical records of 13 patients diagnosed with TTP at our institution from 1997 to 2009 were reviewed. Relevant information including age, gender, clinical presentation, predisposing factors, laboratory abnormalities, treatment, complications, and outcome of each patient were analyzed. Results: The median age of the patients was 47 years with a range of 14-78. Five of the 13 (38%) patients presented with the full "pentad" of all clinical features. The median hemoglobin and platelet count at diagnosis was 8.3 g/dL and 9×10 3/μL, respectively. The median time from onset of symptoms to diagnosis was 14 days. Nine of the 13 patients (70%) had the sporadic or idiopathic form of TTP. The other cases of TTP were associated with ticlopidine, breast cancer and autoimmune diseases. The median number of PE was 11 (range: 6-37). Nine of 13 patients (70%) were treated successfully with the combination of PE and corticosteroids, and the remaining 4 patients who failed to respond to this therapy eventually died of multi-organ failure. Conclusion: Our study showed 38% of such patients presented with the full pentad of clinical features but the median time from symptom onset to diagnosis and initiation of therapy was 14 days for the entire cohort. Treatment with a combination of corticosteroids and PE resulted in a 70% success rate in achieving long term remission.
| Original language | English |
|---|---|
| Pages (from-to) | 121-127 |
| Number of pages | 7 |
| Journal | Journal of Medical Sciences (Taiwan) |
| Volume | 32 |
| Issue number | 3 |
| Publication status | Published - Jun 20 2012 |
| Externally published | Yes |
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Keywords
- Plasma exchange
- Thrombocytopenia
- Thrombotic thrombocytopenic purpura
ASJC Scopus subject areas
- Medicine(all)
Cite this
Diagnosis and treatment of thrombotic thrombocytopenic purpura : A single institution experience. / Chang, Ping Ying; Ho, Ching Liang; Chao, Tsu Yi; Kao, Woei Yau; Dai, Ming Shen; Chiueh, Tzong Shi; Wan, Hsiang Lin; Chen, Yeu Chin.
In: Journal of Medical Sciences (Taiwan), Vol. 32, No. 3, 20.06.2012, p. 121-127.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Diagnosis and treatment of thrombotic thrombocytopenic purpura
T2 - A single institution experience
AU - Chang, Ping Ying
AU - Ho, Ching Liang
AU - Chao, Tsu Yi
AU - Kao, Woei Yau
AU - Dai, Ming Shen
AU - Chiueh, Tzong Shi
AU - Wan, Hsiang Lin
AU - Chen, Yeu Chin
PY - 2012/6/20
Y1 - 2012/6/20
N2 - Background: Thrombotic thrombocytopenic purpura (TTP) is an urgent, life-threatening hematologic disorder. It can result in a high fatality rate without early identification and prompt plasma exchange (PE) therapy. We report our experience of clinical manifestations, laboratory abnormalities, diagnosis, treatment and outcome of patients with TTP at our institution. Patients and Methods: Medical records of 13 patients diagnosed with TTP at our institution from 1997 to 2009 were reviewed. Relevant information including age, gender, clinical presentation, predisposing factors, laboratory abnormalities, treatment, complications, and outcome of each patient were analyzed. Results: The median age of the patients was 47 years with a range of 14-78. Five of the 13 (38%) patients presented with the full "pentad" of all clinical features. The median hemoglobin and platelet count at diagnosis was 8.3 g/dL and 9×10 3/μL, respectively. The median time from onset of symptoms to diagnosis was 14 days. Nine of the 13 patients (70%) had the sporadic or idiopathic form of TTP. The other cases of TTP were associated with ticlopidine, breast cancer and autoimmune diseases. The median number of PE was 11 (range: 6-37). Nine of 13 patients (70%) were treated successfully with the combination of PE and corticosteroids, and the remaining 4 patients who failed to respond to this therapy eventually died of multi-organ failure. Conclusion: Our study showed 38% of such patients presented with the full pentad of clinical features but the median time from symptom onset to diagnosis and initiation of therapy was 14 days for the entire cohort. Treatment with a combination of corticosteroids and PE resulted in a 70% success rate in achieving long term remission.
AB - Background: Thrombotic thrombocytopenic purpura (TTP) is an urgent, life-threatening hematologic disorder. It can result in a high fatality rate without early identification and prompt plasma exchange (PE) therapy. We report our experience of clinical manifestations, laboratory abnormalities, diagnosis, treatment and outcome of patients with TTP at our institution. Patients and Methods: Medical records of 13 patients diagnosed with TTP at our institution from 1997 to 2009 were reviewed. Relevant information including age, gender, clinical presentation, predisposing factors, laboratory abnormalities, treatment, complications, and outcome of each patient were analyzed. Results: The median age of the patients was 47 years with a range of 14-78. Five of the 13 (38%) patients presented with the full "pentad" of all clinical features. The median hemoglobin and platelet count at diagnosis was 8.3 g/dL and 9×10 3/μL, respectively. The median time from onset of symptoms to diagnosis was 14 days. Nine of the 13 patients (70%) had the sporadic or idiopathic form of TTP. The other cases of TTP were associated with ticlopidine, breast cancer and autoimmune diseases. The median number of PE was 11 (range: 6-37). Nine of 13 patients (70%) were treated successfully with the combination of PE and corticosteroids, and the remaining 4 patients who failed to respond to this therapy eventually died of multi-organ failure. Conclusion: Our study showed 38% of such patients presented with the full pentad of clinical features but the median time from symptom onset to diagnosis and initiation of therapy was 14 days for the entire cohort. Treatment with a combination of corticosteroids and PE resulted in a 70% success rate in achieving long term remission.
KW - Plasma exchange
KW - Thrombocytopenia
KW - Thrombotic thrombocytopenic purpura
UR - http://www.scopus.com/inward/record.url?scp=84863920444&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84863920444&partnerID=8YFLogxK
M3 - Article
AN - SCOPUS:84863920444
VL - 32
SP - 121
EP - 127
JO - Journal of Medical Sciences
JF - Journal of Medical Sciences
SN - 1011-4564
IS - 3
ER -