Parahemophilia

Alternative names
Factor V deficiency; Owren’s disease

Definition
Factor V deficiency is an inherited abnormal blood coagulation disorder caused by a deficiency of the plasma protein Factor V.

Causes, incidence, and risk factors

Normal blood coagulation is a complex process involving as many as 20 different plasma proteins, which are known as blood coagulation factors. A series of complex chemical reactions using these factors takes place very rapidly to form an insoluble protein called fibrin that stops bleeding. In this disorder, bleeding ranges from mild to severe.

When certain coagulation factors are deficient or missing, the chain reaction does not take place normally. Factor V is rare, and can be caused by inheriting a defective Factor V gene or by acquiring an antibody that interferes with normal Factor V function.

An inhibitor of Factor V can be acquired after giving birth, after surgery, with automimmune diseases and certain cancers, in patients being treated wtih a certain type of fibrin glue, and from unknown sources.

The disease is similar to hemophilia, except bleeding into joints is less common. In the inherited form of Factor V deficiency, a family history of a bleeding disorder is a risk factor.

Excessive bleeding with menstrual periods and after delivery occurs frequently. A family history of a bleeding disorder is a risk factor. Men and women are affected equally. About 1 person per 1 million has the disorder.

Symptoms

     
  • Bleeding into the skin  
  • Excessive bruising  
  • Nose bleeds  
  • Bleeding of the gums  
  • Excessive menstrual bleeding  
  • Prolonged or excessive loss of blood with surgery or trauma  
  • Umbilical stump bleeding

Signs and tests

     
  • Factor V assay showing decreased activity  
  • Slightly prolonged bleeding time (in some people)  
  • Prolonged partial thromboplastin time  
  • Prolonged prothrombin time  
  • Normal thrombin time

Treatment
Fresh plasma or fresh frozen plasma infusions will correct the deficiency temporarily and should be given daily during a bleeding episode or after surgery.

Support Groups
The stress of illness can often be helped by joining a support group where members share common experiences and problems. See hemophilia - support group.

Expectations (prognosis)
The probable outcome is good with diagnosis and proper treatment.

Complications
Severe hemorrhage could occur.

Calling your health care provider
Go to the emergency room or call the local emergency number (such as 911) if you have an unexplained or prolonged loss of blood.

Prevention
This is an inherited disorder; there is no known prevention.

Johns Hopkins patient information

Last revised: December 3, 2012
by Gevorg A. Poghosian, Ph.D.

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All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.