Abstract :
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.
Key word: Autosomal Recessive Disorder, FFPFresh
F rozen Plasma, Parahaemophilia, RICDRare
InheritedCoagulation Disorder.