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Hemorrhagiparous Thrombocytic Dystrophy



Hemorrhagiparous Thrombocytic Dystrophy Causes


The person experiences prolonged bleeding due to the absence of the glycoprotein Ib-IX-V complex that causes platelets to stick. A child may also inherit the abnormal gene that causes this syndrome from each parent. However, the disease is rarely inherited from just one parent who carries the abnormal gene.


Hemorrhagiparous Thrombocytic Dystrophy Definition


Hemorrhagiparous thrombocytic dystrophy is commonly called Bernard-Soulier syndrome named after Dr. Jean Bernard and Jean Pierre Soulier. It is a severe bleeding disorder caused by a deficiency of glycoprotein Ib (GpIb), which is important in clot formation.


Hemorrhagiparous Thrombocytic Dystrophy Diagnosis


Physicians must be aware of the patient's medical history particularly about bleeding and bleeding illness in the family. The patient undergoes a physical exam and laboratory tests, and examined for signs of bleeding. The disorder is usually confirmed in a specialist laboratory with a specialization in this illness.


Hemorrhagiparous Thrombocytic Dystrophy Symptoms and Signs


The patient suffers from a prolonged bleeding time, and has a low platelet count and large platelets.


Hemorrhagiparous Thrombocytic Dystrophy Treatment


Patients should be aware of certain medications to avoid such as aspirin, which can interfere with platelet function. Dental hygiene is also a vital issue. Heavy periods during a woman's menstruation cycle can result in low iron, which must be treated. Severe bleeding episodes usually require blood or platelets transfusion.


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