Glanzmann Thrombasthenia - DR GHULAM YASEEN

Glanzmann Thrombasthenia

Glanzmann Thrombasthenia

Overview

Your blood contains small cell particles named platelets. Whenever you are injured or cut a vein, and bleeding start from that injured part, then these platelets rush towards the place of injury to stop bleeding. Platelets stick together and send signals to other platelets to come at the site of injury. They form a plug at the bleeding point; due to which bleeding stops shortly. This is the normal procedure of blood clotting.

Glanzmann Thrombasthenia is a platelets function disorder in which blood doesn’t clot after a cut or injury. This is caused by an abnormality in the genes for glycoproteins IIb/IIIa.These genes are fibrinogen receptors which help to stick platelets together to stop bleeding. Due to the absence of these fibrinogen receptors, platelets don’t stick together and it becomes very difficult to stop bleeding from an injury Site.

In 1000,000 people, there is 1 person having this disease worldwide. This disease is found in both men and women. Children are diagnosed early in life, usually in the first five years of life. Because in these years they experience severe bleeding episodes from gums during teething or losing baby teeth. Some children bleed frequently than normal after vaccination. They may experience epistaxis.

Sign and symptoms

Most common symptoms of Glanzmann thrombasthenia are the following:

  • Bleeding from nose and gums
  • Heavy menstruation in females
  • Excessive bleeding after trauma or surgery
  • Heavy menstruation in women
  • Bleeding from nose or gums
  • Easy bruises
  • Small purple spots on the skin due to capillary damage (petechiae)

Rare symptoms of GT included the following:

  • Bleeding in the head and brain, that may cause headache, loss of consciousness, drowsiness, and pain in the neck.
  • Bleeding in the gastrointestinal tract that may cause bloody vomiting or bloody stools.
  • Bleeding in the joints leaving swelling and immobility of joint.

When to visit the doctor

If your child bleeds for a long time on slight injury or bleeds from nose more often, without any cause then visit a doctor. If you see purple spots on your child’s body and If he bleeds a lot after vaccination or upon losing baby teeth, then it’s an emergency condition; you may hurry to visit a doctor.

Causes

  • Glanzmann thrombasthenia is an autosomal recessive disorder, which means that both parents may carry these abnormal genes but they may not have the disease.
  • Acquired Glanzmann Thrombasthenia is associated with lymphoproliferative and autoimmune diseases, characterized by impaired functions of platelets.

 

Prevention

You can prevent your next generation from Glanzmann thrombasthenia, for that you need a genetic counselor before you marry. Women with GT should discuss menstrual bleeding and any sort of issues of planned pregnancy to the hematologist or gynecologist.

Risk factors

To stop bleeding, when other measurements don’t work, then you have to go for platelets transfusion. Platelets are derived from 1-2 healthy individuals. There are risks for infections due to transfusion. Patients may show transfusion reaction after receiving platelets.

Due to prolonged bleeding, you lose a huge amount of blood, that causes anemia. So, you have to look after your Hb level and general health if you have Glanzman’s thrombasthenia for better survival.

 

Treatment at Saher Homeo Clinic

Here, we treat every individual at the genetic level. Every individual seeks treatment on the basis of symptoms and health condition. We have a number of completely cured cases. See their stories here

 

 

 

 

 

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