1. Systemic lupus erythematosus presenting as thrombotic thrombocytopaenic purpura in a child: a diagnostic challenge.
- Author
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Orbe Jaramillo IA, De Lucas Collantes C, Martínez de Azagra A, and Sebastián E
- Subjects
- ADAMTS13 Protein deficiency, ADAMTS13 Protein genetics, Biopsy, Child, Diagnosis, Differential, Female, Glucocorticoids therapeutic use, Humans, Kidney pathology, Lupus Nephritis blood, Lupus Nephritis complications, Lupus Nephritis therapy, Mycophenolic Acid therapeutic use, Purpura, Thrombotic Thrombocytopenic blood, Purpura, Thrombotic Thrombocytopenic diagnosis, Purpura, Thrombotic Thrombocytopenic therapy, Sequence Analysis, DNA, Treatment Outcome, ADAMTS13 Protein analysis, Lupus Nephritis diagnosis, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic etiology
- Abstract
Thrombotic thrombocytopaenic purpura (TTP) is a life-threatening thrombotic microangiopathy characterised by microangiopathic haemolytic anaemia, thrombocytopaenia and organ ischaemia. TTP is caused by a severe functional deficiency of ADAMTS13 activity. We describe a 10-year-old girl presenting anaemia and thrombocytopaenia with schistocytes. Urine protein to creatinine ratio was within nephrotic range. ADAMTS13 activity was 0%, and no anti-ADAMTS13 antibodies were found. A renal biopsy showed deposits of IgG, C3 and C1q in the capillary membrane, compatible with class V lupus nephritis. Therapeutic plasma exchange (TPE) was performed in conjunction with therapy consisting of steroids and mycophenolate mofetil. After 11 months of follow-up, the patient remains in remission with normal ADAMTS13 activity. Although acquired TTP is a rare finding in children, differential diagnosis of thrombotic microangiopathy should include ADAMTS13 and the assay should be performed early. TTP treatment is based on TPE, although the underlying disease must be ruled out to optimise treatment and prevent relapse., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)
- Published
- 2020
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