An Ayurvedic approach to hemophagocytic lymphohistiocytosis (HLH)

What is hemophagocytic lymphohistiocytosis?

As per Ayurveda Aspect:

  • This disease is caused due to the vitiation of vata, pitta and Kapha dosha. All these three doshas are inter-liked with each other. As the kapha increases, it accumulates in the tissues and also increases pitta dosha. Due to these doshas, the path of vata dosha gets hampered which causes pain in the body tissues and this accumulated kapha dosha causes inflammation in the different organs of the body.

As per modern aspect:

  • Hemophagocytic lymphohistiocytosis (HLH) is a rare condition in which there is an uncontrolled immune activation (production of too many activated immune cells (macrophages and lymphocytes)) which is characterized by clinical evidence of extreme inflammation. It can either be primary or secondary. 
  • It is primary when the condition is inherited that typically has its onset during the infancy period or the early childhood period. In this condition, the T and NK cells of the immune system do not work properly. Over-activation of these cells causes too much inflammation and the immune system begins to damage the body’s own healthy cells and organs which include the liver, brain and bone marrow when the blood is formed.
  • In the secondary condition, it may develop as a result of strong immunological activation of the body’s immune system which may be caused due to severe infections. It may also develop during malignancies or during the treatment of a known malignancy.
causes of hemophagocytic lymphohistiocytosis

What are the causes of hemophagocytic lymphohistiocytosis?

The causes of hemophagocytic lymphohistiocytosis may be inherited or non-inherited.

There are 5 subtypes of inherited hemophagocytic lymphohistiocytosis. Each subtype is caused due to mutation (changes) in different genes that are helpful to regulate the immune system.

  • Type 1: Type 1 is caused by a gene defect on chromosome 9.
  • Type 2: This is caused due to a mutation in the PRF1 gene.
  • Type 3: It is caused by a mutation (change) in the UNC13D gene.
  • Type 4: It is caused by mutations in the STX11 gene.
  • Type 5: Type 5 is caused by mutations in the STXBP2 gene.

All these genes in the normal state provide signals to proteins to destroy activated immune cells. But in an abnormal state, these genes may lead to an increase in the production of immune cells which results in an excessive immune response.

What are the Symptoms of hemophagocytic lymphohistiocytosis?

  • Hemophagocytic lymphohistiocytosis (HLH) is not easy to diagnose and can be a challenge because the initial symptoms may imitate other problems such as common infections.

Symptoms of HLH may include

  1. Persistent fevers
  2. Rash
  3. Enlarged liver
  4. Enlarged spleen
  5. Enlarged lymph nodes
  6. Anemia
  7. Eeizures
  8. Liver failure
  9. Low platelets
  10. Low white blood cells
  11. Jaundice
  12. Hepatitis
  13. Respiratory issues (coughing, respiratory distress)
  14. Altered mental functions

What are the risk factors for hemophagocytic lymphohistiocytosis?

  • Risk factors that increase the susceptibility to hemophagocytic lymphohistiocytosis include:

Serious infections:

  1. Infection caused by a virus that causes mononucleosis: that causes the common cold
  2. Overuse of certain medications
  3. Reaction to immunizations (rare cases)
  4. Cancer: which is associated with T cells lymphomas
  5. Other diseases such as rheumatoid arthritis or lupus
  6. Family history

All these above-mentioned risk factors do not mean that one will get the condition. These factors increase the chances of getting a condition to anyone without any other risk factors.

Planet Ayurveda offers Hemophagocytic Lymphohistiocytosis Care Pack for the treatment.

Kanchnaar guggul:

  • Kaishore guggul contains effective natural blood purifier herbs such as, haritaki, bibhitaki, and Amalaki. These herbs remove toxins from the blood and used as herbal remedy for all metabolic disorders. This formulation balances the hormone production from pituitary and thyroid glands. It contains herbs like kachnar, black pepper, ginger, varuna, guggul, etc. All these herbs has been found useful in blockages in the lymphatic channels and inflammation of lymph nodes.

Dosage: 2 tablets two times a day with plain water after meals.

Punarnava Mandoor:

  • Punarnava mandoor is a classical herbal formulation which is very useful in various health conditions such as anemia, gout, swelling, low platelet, and RBCs count. These capsules are diuretic in nature, therefore helpful in ascites, liver failure, renal failure, puffy eyes, glaucoma, anemia and other problems related to the fluid accumulation in the tissues of the body.
  • This formulation contains several herbs such as punarnava, pippali, shunthi, Haridra, vidanga, chavya, maricha, mandoor bhasma, etc. In kidney failure, it is used along with the combination of mutrakrichantak churna for better results.

Dosage: 2 tablets two times a day with water after the intake of food.

Navkarshik Churna:

  • Navkarshik churna is a classical Ayurvedic formulation that is useful in pacifying Vata dosha and removes all the toxins from the body. It contains giloy which is a natural immune modulator that improves the immunity and daruhaldi  acts as an anti-inflammatory herb. This formulation is made up of herbs like haritaki, bibhitaki, Amalaki, giloy, neem, Vacha, daruhaldi, manjistha, etc and these herbs act together in a synergistic manner and very effective in painful conditions.

Dosage: 1 teaspoon twice a day with water after meals.

Boswellia curcumin:

  • Boswellia curcumin is the most effective formulation offered by Planet Ayurveda. This formulation contains a combination of anti-inflammatory herbs and these herbs not only relieve pain but also reduce the swelling. These capsules exert neuroprotective activity and maintain the health of the cardiovascular system. This powerful combination of Boswellia and curcumin promotes overall health and provides comprehensive immune support.

Dosage: 2 capsules two times daily after meals.

Ashwagandha Capsules:

  • Ashwagandha is a rejuvenating herb that helps in maintaining the proper nourishment of tissues especially of the bones and muscles and supports the proper functioning of adrenals and reproductive system. These capsules are formulated with the pure extract of Ashwagandha and are useful in stress, fatigue, anxiety, weight loss, nervous breakdown, and aging. Regular use of these capsules provides strength to the back muscles and calms down the system.

Dosage: 2 capsules two times a day with water.

Brahmi Capsules:

  • Brahmi capsules are used as a brain tonic because in Ayurveda it is believed that Brahmi has several health benefits on the brain cells. These capsules are prepared from the pure and natural extract of Brahmi. It improves concentration power and also helpful in conditions such as diabetes, cough, jaundice, swelling, and fever. It has a calming effect on the body and mind.

In this shloka actions and therapeutic uses of brahmi are explained.

  • Brahmi is cold and bitter in taste. It cures edema, fever, anemia, pruritus’ and spleen diseases. It pacifies vata  and kapha dosha and also acts as an appetizer. It also helpful in digestion, itching and skin diseases.

(Reference: Dhanvantri nighantu: Page no-148 and shloka-10)

Dosage: 2 capsules two times a day after intake of food.