What is Galnzmann’s disease or Glanzmann Thrombasthenia
It is an abnormality of the platelets. It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa), which is a receptor for fibrinogen.
It is a congenital hemorrhagic disorder, means a disorder, which is present from the time of birth.
Platelets are small blood cells that are the first responders in the case of a cut or other bleeding injury. They normally clump together to form a plug in the wound and stop bleeding.
Without enough glycoprotein IIb/IIIa, platelets won’t be able to stick together, or clot, properly. People who have Glanzmann’s disease have difficulty getting their blood to clot. Glanzmann’s disease can be a serious issue during surgeries or in the case of major injuries because a person could lose large quantities of blood.
Bleeding disorders are collectively called as Raktapitta in Ayurveda.
The intake of Ushna (warm), Theekshna (penetrative), Kadu (hot), Amla (sour) Lavana (salty)and Vidahi ( causing internal burning sensation) foods in excess leads to the vitiation of Pitta and Blood (Rakta) and the vitiated blood together with Pitta flows out from the circulating paths and results in excessive bleeding through nose, eyes, ears, mouth, penis, vagina or anus and even through the hair follicles.
Ayurveda texts specifically mention that the garbha charya of mother while conceiving a baby or during the Maithuna ( sexual intercourse) can even lead to some serious disorders in the new born-which can be remembered here as Glanzmann’s disease is a congenital disorder and there is no specific etiology for it other than the mutation of the gene. And also, Raktapitta is bleeding without trauma or internal injury. Even Sushrutacharya does not mention trauma in the etiology of Raktapitta.
Mutations in the ITGA2B or ITGB3 gene cause Glanzmann thrombasthenia. These genes provide instructions for making the two parts (subunits) of a receptor protein called integrin alphaIIb/beta3 (αIIbβ3). This protein is abundant on the surface of platelets. Platelets are small cell fragments that circulate in the blood and are an essential component of blood clots. During clot formation, integrin αIIbβ3 helps platelets bind together. Blood clots protect the body after injury by sealing off damaged blood vessels and preventing further blood loss.
ITGA2B or ITGB3 gene mutations result in a shortage (deficiency) of functional integrin αIIbβ3. As a result, platelets cannot clump together to form a blood clot, leading to prolonged bleeding.
Three types of Glanzmann thrombasthenia have been classified according to the amount of integrin αIIbβ3 that is available. People with type I (the most common type) have less than 5 percent of normal integrin αIIbβ3 levels, people with type II have between 5 and 20 percent of normal integrin αIIbβ3 levels, and people with the variant type have adequate integrin αIIbβ3 levels but produce only nonfunctional integrin.
Some people with Glanzmann thrombasthenia do not have an identified mutation in either the ITGA2B or ITGB3 gene; the cause of the disorder in these individuals is unknown.
This condition is autosomal recessive. That means both parents of the victim must carry the defective gene or genes for Glanzmann’s in order for him or her to inherit the disease. If there is a family history of Glanzmann’s disease or related disorders, there is an increased risk of inheriting the disorder or passing it on to the children.
Doctors and scientists are still researching what exactly causes Glanzmann’s disease and how it can best be treated.
Symptoms of Glanzmann’s Disease:
Glanzmann’s disease may cause severe or continual bleeding, even from a minor injury. People who have the disease may also experience:
- frequent nosebleeds
- bruising easily
- red or purple spots on the skin caused by bleeding underneath the skin (petechiae)
- swelling caused by bleeding within tissues (hematoma)
- bleeding gums
- heavy menstrual bleeding
- bleeding during or after surgery
The severity and frequency of the bleeding episodes in Glanzmann thrombasthenia can vary greatly among affected individuals, even in the same family. Spontaneous bleeding tends to become less frequent with age.
Together with the excessive bleeding from different body outlets,, the patient will exhibit the following symptoms:
1 Sirogurutwa (heaviness of head)
2. Aruchi (tastelessness)
3. Seethecha (desiring cold)
4. Dhoomaka (feels like he body is exposed to extreme hot weather)
5. Amlaka (sour burps)
6. Chardhi (vomiting)
7. Kasam (cough)
8. Swasam ( breathing difficulty)
9. Bhramam (vertigo)
11. Loha lohitha matsya aama gandha aasyatwam( Mouth tastes like Iron, Blood, fish, undigested food etc.)
12.Swarakshayam (loss of voice)
13. Rakta hardira harithavarnatha nayanadishu(reddish-yellowish or greenish discoloration of eyes etc.)
13. Neelalohitha peethavarna avivechanam ( Unable to distinguish blue, red and yellow colours)
14. Swapne thadvarna darshitwam( dreaming of blue red or yellow cours)
Diagnosing Glanzmann’s Disease:
The following simple blood tests help to diagnose Glanzmann’s disease:
- platelet aggregation tests: to see how well the platelets clot
- complete blood count: to determine the number of blood platelets the patient has
- prothrombin time (PT): to determine how long it takes for the blood to clot
- partial thromboplastin time (PTT): another test to see how long it takes for the blood to clot
Glanzmann thrombasthenia is estimated to affect 1 in one million individuals worldwide but may be more common in certain groups, including those of Romani ethnicity, particularly people within the French Manouche community.
Glanzmann disease is a lifelong condition, and there is no cure.
Ayurveda has a specific prognosis for diseases along with the treatment line.
Ayurveda specifies that only those bleeding disorders( Raktapitta) which occur through the nose, eyes, ears, and mouth which occurs to Balavan( healthy individuals with good immunity)can be cured.
Adhoga raktaputta(excessive bleeding through anus, penis or vagina)can be just maintained with medicines and good diet or exercise.
Raktapitta which occurs through all parts of the body and which occurs in Mandagni( people with extremely less digestive capacity, cannot be cured.)
Glanzmann disease is an inherited condition. There is no known prevention.
Modern medicine does not have a specific treatment for this disorder. Platelet transfusions may be given to people who are having severe bleeding.
Ayurvedic treatment line:
It is improper to arrest the emission of blood immediately at the outset of the disease if the patient is healthier with a compatible immune system,such a procedure can bring an attack of Pandu(anemia), Grahani, Kushta(dermatological disorders), Gulma or Jwara (fever) or Pleeha(spleen enlargement).
- Strong patients with the compatible immune system, if the blood makes a downward course, should be treated with Emetic (Vamana) medicines and if blood makes an upward course should be treated with purgatives ( Virechana).
Virechana can be induced with a combination of Draksha, Yashtimadhu, Kashamrya, and Sugar. Vamana can be induced with an emetic compound consisting of Yashti-madhu and Honey.
- A weak patient should be treated in both cases, with Samana dravyas.
- Langhana(fasting) should be first used in the case of an excessive blood emission, in a strong patient with unimpaired digestion and compatible immune system. APeya prepared with a small amount of rice should be administered after Langhana.
- Priyangu, Lodhra, Gairika, Utpala, Sauvarna Gairika, Kaliyaka, Chandana , Sugar, Ashvagandha, Ambudha, Yshtimadhu, Mrinala and Saougandhika taken in equal parts should be pounded together and mixed with copious quantities of milk, honey and clarified butter and inserted into the rectum (Nirooham). Then the patient should be sprinkled with cold water and should have his food with milk and after that, Ghrutham (clarified butter) duly cooked with Yasti madhu should be used for Anuvasana vasthi. These are extremely used in Adhoga (downward) Raktapitta. If the patient is strong enough, vomiting can be induced after the cessation of bleeding.
- The above-mentioned drugs can be used as Uttaravasthi in case of bleeding from the urethra.
- Bleeding can be arrested by external surgical measures like Sandhanam ligation of the bleeding artery),Dahanam ( cauterization of the bleeding points)and Pachanam (measures to relieve the obstruction of channels).
- Foods dominant in Tikta and Kashaya rasa and Tarpanam for Oordhwaga Raktapita
- Madhura rasa and Peya in Adhoga Raktapitta
- Jangala mamasa rasam(the essence of Jangala animal meat)
- Water with Usheeram(vetiver) can be used for external use and drinking
- Ardha chakraasanam
Vasa swarasam/Vaasa kashayam
- Spicy food
- Extremely hot food
- Consumption of alcohol
- Charaka Samhitha – Chikitsitha sthanam
- Sushrutha Samhitha – Uthara Thantram
- Ashtanga Hridayam – Chikitsitham
What is Galnzmann’s disease or Glanzmann Thrombasthenia