Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
Users Online: 73
About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Advertise Login 
Year : 2022  |  Volume : 11  |  Issue : 4  |  Page : 224-226

Congenital thrombotic thrombocytopenic purpura simulating alloimmune thrombocytopenia

1 Department of Pediatrics, Almana General Hospital, Al Ahsa, Saudi Arabia
2 Department of Pathology, Maternity and Children Hospital, Al Ahsa, Saudi Arabia

Correspondence Address:
Muneer Hassan Albagshi
Department of Pediatrics, Almana General Hospital, Al Ahsa
Saudi Arabia
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.jcn_63_22

Rights and Permissions

Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathic caused by severely reduced activity of the von Willebrand factor-cleaving protease A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS-13), which leads to small-vessel platelet-rich thrombi, thrombocytopenia, and microangiopathic hemolytic anemia (MHA) in the absence of neutralizing antibodies. We describe a case of cTTP in a female neonate presenting initially on the 1st day of life with asymptomatic thrombocytopenia thought to be alloimmune thrombocytopenia, then progressed to severe neonatal jaundice, pallor, and MHA, in whom ADAMTS-13 levels <5%, and the anti-ADAMTS-13 antibody titer was negative confirming the diagnosis of cTTP. The patient initially received intravenous immunoglobulin infusion before documenting MHA. She received fresh frozen plasma infusions which successfully reversed the MHA (by supplying ADAMTS-13) and prevented organ damage in this patient.

Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)

 Article Access Statistics
    PDF Downloaded58    
    Comments [Add]    

Recommend this journal