Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus

Sheila Thampi; Darren Salmi; Shinsaku Imashuku; Jonathan M Ducore; Noriko Satake

doi:10.1097/MPH.0b013e318208425d

Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus

Sheila Thampi, Darren Salmi, Shinsaku Imashuku, Jonathan M Ducore, Noriko Satake

Pediatric Hematology and Oncology

Research output: Contribution to journal › Article

5 Scopus citations

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	https://doi.org/10.1097/MPH.0b013e318208425d
State	Published - Apr 2011

Keywords

ADAMTS 13
systemic lupus erythematosus
thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Oncology
Hematology

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Thampi, S., Salmi, D., Imashuku, S., Ducore, J. M., & Satake, N. (2011). Thrombotic thrombocytopenic purpura in a child with systemic lupus erythematosus. Journal of Pediatric Hematology/Oncology, 33(3), 221-223. https://doi.org/10.1097/MPH.0b013e318208425d

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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.",

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