Diagnosis and treatment of thrombotic thrombocytopenic purpura: A single institution experience

Ping Ying Chang, Ching Liang Ho, Tsu Yi Chao, Woei Yau Kao, Ming Shen Dai, Tzong Shi Chiueh, Hsiang Lin Wan, Yeu Chin Chen

Research output: Contribution to journalArticle

1 Citation (Scopus)

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 languageEnglish
Pages (from-to)121-127
Number of pages7
JournalJournal of Medical Sciences (Taiwan)
Volume32
Issue number3
Publication statusPublished - Jun 20 2012
Externally publishedYes

Fingerprint

Thrombotic Thrombocytopenic Purpura
Plasma Exchange
Therapeutics
Adrenal Cortex Hormones
Ticlopidine
Breast Diseases
Clinical Laboratory Techniques
Platelet Count
Causality
Autoimmune Diseases
Medical Records
Hemoglobins
Breast Neoplasms

Keywords

  • Plasma exchange
  • Thrombocytopenia
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Chang, P. Y., Ho, C. L., Chao, T. Y., Kao, W. Y., Dai, M. S., Chiueh, T. S., ... Chen, Y. C. (2012). Diagnosis and treatment of thrombotic thrombocytopenic purpura: A single institution experience. Journal of Medical Sciences (Taiwan), 32(3), 121-127.

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 journalArticle

Chang, PY, Ho, CL, Chao, TY, Kao, WY, Dai, MS, Chiueh, TS, Wan, HL & Chen, YC 2012, 'Diagnosis and treatment of thrombotic thrombocytopenic purpura: A single institution experience', Journal of Medical Sciences (Taiwan), vol. 32, no. 3, pp. 121-127.
Chang, Ping Ying ; Ho, Ching Liang ; Chao, Tsu Yi ; Kao, Woei Yau ; Dai, Ming Shen ; Chiueh, Tzong Shi ; Wan, Hsiang Lin ; Chen, Yeu Chin. / Diagnosis and treatment of thrombotic thrombocytopenic purpura : A single institution experience. In: Journal of Medical Sciences (Taiwan). 2012 ; Vol. 32, No. 3. pp. 121-127.
@article{e6aaefe1cdcb41b79585c42a017a07be,
title = "Diagnosis and treatment of thrombotic thrombocytopenic purpura: A single institution experience",
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.",
keywords = "Plasma exchange, Thrombocytopenia, Thrombotic thrombocytopenic purpura",
author = "Chang, {Ping Ying} and Ho, {Ching Liang} and Chao, {Tsu Yi} and Kao, {Woei Yau} and Dai, {Ming Shen} and Chiueh, {Tzong Shi} and Wan, {Hsiang Lin} and Chen, {Yeu Chin}",
year = "2012",
month = "6",
day = "20",
language = "English",
volume = "32",
pages = "121--127",
journal = "Journal of Medical Sciences",
issn = "1011-4564",
publisher = "國防醫學院",
number = "3",

}

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 -