A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13

Pediatric Hematology and Oncology
Atsushi SatoMasue Imaizumi

Abstract

Although acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP) is rare in children, the authors present the case of a 9-month-old boy with ai-TTP showing severe deficiency of ADAMTS13 activity by its inhibitory IgG-autoantibody (4.8 Bethesda units/mL). Plasma exchange therapy was clinically effective but transient. Deficient activity of ADAMTS13 with the presence of its inhibitor persisted for 7 months after the initial diagnosis. However, other laboratory findings improved gradually with steroid (pulse) therapy. The hitherto insufficiently characterized clinical settings of ai-TTP during early childhood underscore the importance of measuring ADAMTS13 activity and its inhibitors for differential diagnosis in patients with thrombocytopenia of unknown etiology.

References

Feb 15, 2002·International Journal of Hematology·Yoshihiro FujimuraKoiti Titani
Apr 8, 2003·Journal of Pediatric Hematology/oncology·Terzah M HortonDonald H Mahoney
Jan 17, 2004·Seminars in Hematology·Reinhard SchneppenheimTobias Obser
Nov 25, 2004·Hematology·J Evan SadlerJames N George
Apr 2, 2005·British Journal of Haematology·Koichi KokameToshiyuki Miyata
Feb 2, 2006·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Yugo ShibagakiToshiro Fujita
Apr 26, 2006·Vox Sanguinis·S FontanaB Mansouri Taleghani
Aug 2, 2007·Hematology/oncology Clinics of North America·Han-Mou Tsai
Nov 21, 2007·Hematology·Pier Mannuccio Mannucci, Flora Peyvandi

❮ Previous
Next ❯

Citations

Feb 21, 2013·Current Opinion in Pediatrics·Chantal LoiratAgnès Veyradier
Jan 24, 2012·La Presse médicale·Paul Coppo, Agnès Veyradier

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Related Papers

Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis
Vickie McDonaldMarie Scully
Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
Jérôme HarambatOlivier Brissaud
Pediatric Blood & Cancer
Naomi P MoskowitzMark Weinblatt
© 2021 Meta ULC. All rights reserved