Parahaemophilia: A Rare Case Report
DOI:
https://doi.org/10.3329/jbcps.v38i4.48985Keywords:
Autosomal Recessive Disorder, FFPFresh Frozen Plasma, Parahaemophilia, RICDRare InheritedCoagulation DisorderAbstract
Parahaemophilia or Owren’s diseaseis a rare haemorrhagic disorder occurs due to congenital and frequently familial deficiency of Factor V.It is characterized by epistaxis, bruising, mucosal bleeding, soft tissue bleeding and haemarthrosis. We report a case of 6 year old female patient with overlapping features with other haemorrhagic disorder. With the complaints of recurrent episodic per rectal bleeding, patient was evaluated at different hospitals in Chattagram and was diagnosed as a case of Haemophilia B and treated accordingly. As her condition was not improved expectedly, she was referred to Armed Forces Institute of Pathology (AFIP) for further evaluation. The lab tests showed prolongation of prothrombin time (PT) and activated partial thromboplastin time (APTT), with normal bleeding time (BT) and thrombin time (TT). Coagulation factors assay revealed a significant decrease of factor V, 1% of normal range. Other coagulation factors are normal. She was treated with FFP and recovered four weeks after treatment.
J Bangladesh Coll Phys Surg 2020; 38(4): 205-208
Downloads
38
59
Downloads
Published
How to Cite
Issue
Section
License
Submission of a manuscript for publication implies the transfer of the copyright from the author to the publisher upon acceptance. Accepted manuscripts become the permanent property of the Journal of Bangladesh College of Physicians and Surgeons and may not be reproduced by any means in whole or in part without the written consent of the publisher.
No part of the materials published in this journal may be reproduced, stored in a retrieval system or transmitted in any form or by any means electronic, mechanical, photocopying, recording or otherwise without the prior written permission of the publisher. Reprints of any article in the Journal will be available from the publisher.