Abstract
We report a child with thrombotic thrombocytopenic purpura (TTP) secondary to systemic lupus erythematosus. The diagnosis was confirmed by low ADAMTS13 activity (<5%) along with the presence of a low titer inhibitor. Her clinical course was complicated by systemic lupus erythematosus, immunosuppressant therapy, and septic shock. She responded to plasma exchange and ADAMTS13 activity levels recovered. This case illustrates the heterogeneity of TTP and the difficulty of making a diagnosis of TTP. ADAMTS13 activity assay can be useful in the differential diagnosis of diseases with clinical features of thrombotic microangiopathy in pediatric patients. However, treatment needs to be decided carefully case-by-case.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 221-223 |
| Number of pages | 3 |
| Journal | Journal of Pediatric Hematology/Oncology |
| Volume | 33 |
| Issue number | 3 |
| DOIs | |
| State | Published - Apr 2011 |
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Keywords
- ADAMTS 13
- systemic lupus erythematosus
- thrombotic thrombocytopenic purpura
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Oncology
- Hematology
Cite this
Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus. / Thampi, Sheila; Salmi, Darren; Imashuku, Shinsaku; Ducore, Jonathan M; Satake, Noriko.
In: Journal of Pediatric Hematology/Oncology, Vol. 33, No. 3, 04.2011, p. 221-223.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus
AU - Thampi, Sheila
AU - Salmi, Darren
AU - Imashuku, Shinsaku
AU - Ducore, Jonathan M
AU - Satake, Noriko
PY - 2011/4
Y1 - 2011/4
N2 - We report a child with thrombotic thrombocytopenic purpura (TTP) secondary to systemic lupus erythematosus. The diagnosis was confirmed by low ADAMTS13 activity (<5%) along with the presence of a low titer inhibitor. Her clinical course was complicated by systemic lupus erythematosus, immunosuppressant therapy, and septic shock. She responded to plasma exchange and ADAMTS13 activity levels recovered. This case illustrates the heterogeneity of TTP and the difficulty of making a diagnosis of TTP. ADAMTS13 activity assay can be useful in the differential diagnosis of diseases with clinical features of thrombotic microangiopathy in pediatric patients. However, treatment needs to be decided carefully case-by-case.
AB - We report a child with thrombotic thrombocytopenic purpura (TTP) secondary to systemic lupus erythematosus. The diagnosis was confirmed by low ADAMTS13 activity (<5%) along with the presence of a low titer inhibitor. Her clinical course was complicated by systemic lupus erythematosus, immunosuppressant therapy, and septic shock. She responded to plasma exchange and ADAMTS13 activity levels recovered. This case illustrates the heterogeneity of TTP and the difficulty of making a diagnosis of TTP. ADAMTS13 activity assay can be useful in the differential diagnosis of diseases with clinical features of thrombotic microangiopathy in pediatric patients. However, treatment needs to be decided carefully case-by-case.
KW - ADAMTS 13
KW - systemic lupus erythematosus
KW - thrombotic thrombocytopenic purpura
UR - http://www.scopus.com/inward/record.url?scp=79954416420&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79954416420&partnerID=8YFLogxK
U2 - 10.1097/MPH.0b013e318208425d
DO - 10.1097/MPH.0b013e318208425d
M3 - Article
VL - 33
SP - 221
EP - 223
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
SN - 1077-4114
IS - 3
ER -