Open AccessSystemic lupus erythematosus presenting as thrombotic thrombocytopaenic purpura in a child: a diagnostic challenge
Author(s) -
Irene Alejandra Orbe Jaramillo,
Carmen de Lucas Collantes,
Amelia Martínez de Azagra,
Elena Sebastián
Publication year - 2020
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2019-232002
Subject(s) - thrombotic microangiopathy , medicine , adamts13 , schistocyte , thrombotic thrombocytopenic purpura , lupus nephritis , microangiopathic hemolytic anemia , renal biopsy , gastroenterology , microangiopathy , nephrotic syndrome , immunology , renal function , disease , platelet , endocrinology , diabetes mellitus
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.