Polycythemia Ayurveda Understanding

By Dr Raghuram Y.S. MD (Ay) & Dr Manasa, B.A.M.S
Polycythemia means increase in the number of red blood cells in the body. Here the hemoglobin concentration is elevated in the peripheral blood.

Various perspectives of understanding Polycythemia through Ayurveda

A. Ayurveda understanding by definition of polycythemia

Rakta Vriddhi – Polycythemia can be translated to ‘rakta vriddhi – increase in blood quantity’ as explained in Ayurveda. Abnormal red blood cell count can be taken into consideration.
Chirakalina Rakta Vriddhi – The variant of polycythemia i.e. polycythemia vera is a chronic condition and is very difficult to cure. This condition can be accounted to as chronic form of rakta vriddhi which has not been treated comprehensively.
Read – Understanding Blood Functions, Vitiation And Treatment As Per Ayurveda

B. Ayurveda Understanding of Primary and Secondary Polycythemia

Primary Polycythemia – Since primary polycythemia is caused due to inherent problems in the process of production of RBC the below mentioned conditions can be considered from Ayurveda perspective –

  1. Dhatvagni Vaishamya – increase in blood (cells) caused due to error in formation of blood tissue due to mildness / sluggishness of the rasa and rakta dhatvagni i.e. tissue fire of rasa and blood tissue

According to Ayurveda, the tissues are formed in chronological order. The rakta i.e. blood (cells) tissue is formed from rasa (plasma, fluid compartment of the blood) tissue. Each tissue will have its own tissue fire, the balance of which helps in the formation of good quality and quantity of the same tissue and also a portion of that tissue which would nourish the next tissue in the sequence. The sub-tissue and tissue wastes are formed as end products.
Now if the tissue fire is decreased in its functional capacity it leads to the destruction of the tissue and also the next tissue in the sequence. On the contrary if the tissue fire is increased in its functional capacity it leads to increase of that particular tissue and also the next tissue in the sequence.
Taking the case of diminished fire of rasa tissue, there will be increase in quantity of immature rasa tissue. This leads to increased formation of rakta i.e. red blood cells which are immature and not healthy. If the tissue fire of rakta is diminished, there will be increase in quantity of blood (cells) tissue.
Read – Role Of Doshas In Blood Circulation, Nutrition And Aeration Of Body

Both these can lead to rakta vriddhi, a polycythemia like condition. Since it is caused due to interior causes, this kind of rakta vriddhi shall be considered as primary polycythemia.

  • Rakta Vriddhi – There may be tendency to have increased blood tissue and blood cells right from birth. This may predispose one to wide array of health problems and diseases in future. They may develop one or more symptoms / diseases which have been enlisted in ‘rakta vriddhi’.
  • Vata vrddhi / prakopa – Abnormal increase of vata will cause abnormal multiplication and increase of cells, blood cells in case of polycythemia.
    Read – Rakta Pradoshaja Rogas – When Blood Is Vitiated By Doshas

Secondary polycythemia – This occurs as a response to other factors and underlying conditions that promote RBC production.

This, according to Ayurveda, may be caused due to consumption of or exposure to

  • the etiological factors leading increase in blood
  • the etiological factors leading to pathological increase in doshas which in turn might lead to pathological increase in blood (cells)

Below mentioned conditions can be considered as causal for secondary polycythemia or forms of secondary polycythemia.

a. Raktapitta – It is a condition which is caused due to aggravation of pitta and rakta and mutual contamination of morbid blood and pitta. This is caused due to combined etiological factors of blood and pitta aggravation. Since this condition is caused due to exogenous causes, it can be considered as secondary polycythemia.
b. Plihodara – Splenomegaly is often seen in PV. Pliha / Plihodara i.e. enlargement of spleen has also been mentioned amongst the symptoms of increase in rakta. Imbalances of erythropoietin levels can be considered to the imbalances of ranjaka pitta in liver and spleen. Ranjaka Pitta is that subtype of pitta which imparts red color to the rasa tissue and converts it into rakta.
c. Hrdroga / Shwasa – Heart or lung disease can lead to low oxygen levels in the body. Hrdaya i.e. heart is a common root for both pranavaha (oxygen carrying – in this context) and raktavaha (blood transporting) channels of the body. These conditions may lead to hypoxia which further leads to increased erythropoietin which results in formation of increased red blood cells. These events eventually lead to secondary polycythemia.
Read – Hrid Roga: Causes, Types, Symptoms of Heart Disease As Per Ayurveda

Physiological polycythemia – can be considered to be ‘rakta vriddhi’ and a more severe form of this condition i.e. ‘rakta prakopa’ shall be considered as polycythemia (primary or secondary as the case may be).

C. Ayurveda understanding from the perspective of symptoms, signs and complications of Polycythemia and PCV –

1. Spleen and liver enlargement are mentioned amongst the symptoms of rakta vriddhi.
2. Headaches and Vertigo are mentioned amongst the complications of raktapitta.
3. High blood pressure may be caused when there is aggravation of blood i.e. rakta vriddhi / prakopa in the channels carrying blood.
4. Blood clots may be formed when the vitiated blood and pitta in raktapitta are excessively congested by the association of aggravated kapha.

Among the general signs and symptoms of polycythemia – itching and bruising can be considered in kushta, pain in joints in vatarakta, abdominal pain in gulma, dizziness in sammoha explained amongst the symptoms of rakta vriddhi.
Erythromelalgia and joint pains mentioned amongst the symptoms of PCV can be included under Vatarakta. Vatarakta is a symptom of rakta vriddhi.

Also amongst the symptoms of PCV the below mentioned conditions mentioned under the signs and symptoms of rakta vriddhi shall be considered –

Sl NoSymptoms of PCVSymptoms mentioned in rakta vriddhi (pathological increase in blood tissue)
1Itching / reddish (purplish) discoloration of skinkushta, vatarakta  
2Bleeding / bruising / frequent nosebleeds / bleeding from gumsraktapitta  
3Numbness / tingling sensation in body parts, burning sensation in the feet, reddish discolorationvatarakta  
4Fullness / distension of abdomengulma
5Bleeding from gumsupakusha
6Dizziness / vertigo / blurring vision / tiredness / fatiguesammoha  
7Reddish discoloration of skinrakta tvak

The complications of polycythemia can be included into –

Ayurveda treatment in polycythemia and polycythemia vera

Rakta Vriddhi Chikitsa – line of treatment of pathological increase of blood tissue
Raktapitta Chikitsa – treatment of bleeding disorders (blood contaminated with pitta and associated with other vitiated doshas)
Grathita Rakta Chikitsa – A condition wherein the increased blood cells would thicken the blood which will increase the risk of clot formation in polycythemia. Grathita Rakta has been explained in the raktapitta along with its treatment. Granthi in the blood i.e. matting of blood cells leading to the formation of clot may be caused due to increase of pruthvi i.e. earth element of the blood leading to its viscocity. It may also occur due to increase of ama and or kapha in the blood.
Vatarakta Chikitsa – Vatarakta presents with many symptoms of polycythemia and hence the treatment of this condition can be considered. Also in the treatment of vatarakta, bloodletting is the main treatment, and so is phlebotomy an important intervention in the treatment of polycythemia.
Kapha vriddhi chikitsa – If increased kapha is found to afflict the blood in polycythemia, and if symptoms of kapha increase are found, kapha mitigating treatments, medicines and diet shall be included.
Ama chikitsa – Increase of ama in the body and also in circulation would also influence the blood. In this condition ama relieving measures including ama pachana – treatments and medicines / diet to digest and relieve ama shall be included. The status of agni also needs to be looked into and addressed the imbalances therein.
Raktavruta Vata Chikitsa – This is a condition wherein the vitiated blood envelopes and blocks the vata thus hampering the normal functions of vata. This will also cause problems in free flow of blood. The combined aggravation of both vata and blood will lead to wide array of disorders / diseases or symptoms which mimic vatarakta in picture and will also resemble the clinical picture of polycythemia. Avarana treatment works here. The blood needs to be taken into control and later the vata should be addressed.
Vata vrddhi Chikitsa – To reduce hyperactivity in the body, the imbalances of vata should be dealt with properly, if vata aggravation is found in polycythemia.
Read – Vata Dosha – Introduction, 40 Things To Know

Panchakarma in Polycythemia

Raktamokshana – Bloodletting is the best treatment for disorders caused due to contamination of blood and pathological increase of blood tissue. When increased blood is removed through various procedures of raktamokshana (administered considering the dosha association with blood) the pressure over the roots and channels of blood formation and transportation will be released. This may consequentially cure the disorders caused by the same. Phlebotomy has been advised in the cure of polycythemia also. Phlebotomy is said to be a specific treatment for PCV or other primary polycythemia syndromes. Raktamokshana or bloodletting is an ideal treatment for vatarakta, which is a symptom of raktavriddhi.
Virechana – Therapeutic purgation is also an ideal treatment for polycythemia. Purgation is the best treatment for both morbid pitta and rakta. Pitta and blood have resident and residence relationship.
Vamana – Therapeutic emesis can be considered if there is pathological increase in kapha in patients of polycythemia and if plenty of symptoms of kapha increase are found, which also cause clotting of blood in the blood vessels.
Read – Benefits of Vamana and Virechana Treatment – Charaka Sutrasthana 16

Treatment of Secondary Polycythemia

Symptoms of secondary polycythemia shall be treated on the lines of –

  • Kasa / svasa chikitsa – when cough / shortness of breath / hypoxia are present
  • Anidra chikitsa – when sleep disturbance / sleep apnea are present
  • Bhrama / Murccha chikitsa – when dizziness is present
  • Pandu / Dourbalya chikitsa – when fatigue is present
  • Shrama svasa / kshudra svasa / vata prakopa chikitsa – when poor exercise tolerance is present

Formulations

Modern view

Polycythemia is a state in which hematocrit and / or hemoglobin concentration is elevated in peripheral blood. Hematocrit means volume percentage of RBCs in the blood. Due to the presence of these extra cells the blood becomes thicker leading increased risk for many health issues including formation of blood clots. This condition is also known by the term ‘Polyglobulia’.
Polycythemia caused by each cause would have its own treatment options. Aim of treatment is to bring blood cell levels down to normal.
Polycythemia Vera is a chronic condition for which there is no known cure. When treated promptly and comprehensively with effective treatment it can be managed to a considerable extent. (Read more)

Types

1. Primary Polycythemia / Polycythemia vera (PV)
Mainly 2 conditions are included under this –

  • PFCP – Primary Familial and Congenital Polycythemia – This condition is caused by genetic mutations resulting in enhanced responsiveness to normal levels of erythropoietin.
  • PV – Polycythemia Vera – It is a rare condition and typically associated with leucocytosis (elevated WBC count) and thrombocytosis (elevated platelet count).

2. Secondary Polycythemia

Causes of secondary polycythemia –

  • Being placed at a very high altitude
  • Having obstructive sleep apnea
  • Hypoxia – Heart / lung disease which tends to cause low oxygen levels in the body
  • Some kinds of tumor

Classification 2 (simplified from Wikipedia)

Absolute polycythemia – increase in the number of RBCs
Relative polycythemia – decrease in volume of plasma

Other types of Polycythemia
Stress Polycythemia (Gaisbock’s disease, Stress erythrocytosis, Pseudopolycythemia)
Smoker’s Polycythemia
Relative Polycythemia
Polycythemia caused by synthetically made erythropoietin
(Read more here)

Mechanism of Polycythemia (pathogenesis)

Erythropoiesis i.e. red cell production takes place in bone marrow and is regulated by a hormone named erythropoietin. Erythropoietin is largely secreted by kidneys. About 10% of the same hormone is also produced and secreted by the liver. Low oxygen levels in the blood regulate secretion of erythropoietin. The erythropoietin eventually stimulates RBC production in the bone marrow to compensate for the low oxygen levels (hypoxia). This causes more oxygen to be carried to the tissues. In about 1-5% of newborns neonatal polycythemia can be seen. Blood transfusion, transfer of placental blood to the infant after delivery or chronic inadequate oxygenation of fetus due to placental insufficiency are the most common causes related to this condition.

Symptoms

Difficulty in blood flow

In some people there may be no symptoms of polycythemia while they may be minimal in few people. Below mentioned are the general symptoms of polycythemia –

  • Fatigue
  • Weakness
  • Headache
  • Dizziness
  • Bruising
  • Itching
  • Pain in joints
  • Abdominal pain

Risk factors

  • Age – PV is usually seen in people around 60 years of age.
  • Sex – more common in men
  • Family history – PV is not necessarily hereditary. However, there appears to be a connection of PV with a certain genetic mutation.
  • Gene mutations – It is found that almost all people with PV have mutation in Janus kinase 2 gene (JAK2 gene) though its role in the causation of the disease is unclear (Leukemia and Lymphoma Society). TET2 gene has also found to be associated with PV.

Other Risk Factors

  • Smoking – hypoxia caused by chronic lung diseases and smoking (addiction) cause polycythemia, smoking can be a significant risk factor
  • Chronic CO (Carbon Monoxide) exposure – in people working in undergrounds (tunnels) or parking garages, cab drivers who are driving in cities which are highly polluted and congested and in factory workers wherein there is plenty of exposure to engine exhaust
  • Living at high altitudes (due to low oxygen levels in the environment)
  • Certain hemoglobin abnormalities
    Read – Ayurvedic Smokers Diet – Must Have Herbs In Your Diet

Complications

  • Increased levels of circulating RBCs – This would increase the thickness of the blood. This in turn may get associated with risk for clot formation. These events eventually lead to strokes, pulmonary embolism, heart attacks and possibly death.
  • Leukemia
  • Hemorrhage
  • Clotting problems
  • Kidney dysfunction / kidney stones
  • Gout
  • Myelofibrosis (spent marrow)- results in scar tissue – resulting in anemia from marrow failure
    Read – Chronic Renal Failure Causes, Symptoms, Treatment In Ayurveda

Complications of secondary polycythemia

In secondary polycythemia the complications are related to those of the underlying condition / disease.
Right sided heart failure and pulmonary hypertension are complications of chronic hypoxia caused due to severe lung disease.
Generalized swelling, low BP, kidney dysfunction and poor functional status occur in the form of complications due to CHF.
Several organs are affected due to poor blood flow caused by increased thickness or viscosity, in infants suffering from neonatal polycythemia. Kidney dysfunction, increased blood pressure in lungs, hypoxia and intestinal problems may be caused due to this.

Complications of PV – might occur when PV is not treated properly –

  • Enlargement of spleen
  • Formation of blood clots
  • Peptic ulcers
  • Gout
  • Angina / heart disease
  • Stroke
  • Other blood disorders (myelofibrosis, leukemia)

Diagnosis of polycythemia

In suspected polycythemia the below mentioned tests may be advised –

Blood tests – Complete blood count to find out if there is increase in red cells, platelets and white blood cells in bloodstream is generally done. Other specific tests may be advised as and when needed. A routine or accidental blood check will reveal polycythemia many times. Hemoglobin, hematocrit and RBC concentration are evaluated more than once to confirm the diagnosis.
Clinical examination – A thorough medical history shall be taken by the physician followed by physical examination. Chronic hypoxia may be an important clue, the signs of which include cyanosis, clubbing of fingers or pursed lip breathing. Redness of palms and soles may be yet another sign of polycythemia.
To screen for lung or heart disease – a chest X-ray, EKG and ECG may be performed. Hemoglobin analysis may be necessary especially if conditions with high oxygen affinity or 2, 3-BPG deficiency are suspected. Blood test to rule out CO poisoning may also be advised.
It needs a careful interpretation, but EPO (Erythropoietin) blood levels will be helpful. This is more important if there are signs and evidences of hypoxia. EPO levels are low as a response to increased production of RBCs in Polycythemia Vera. Their levels may be extremely high in case of tumors secreting erythropoietin.
Bone marrow biopsy – may be suggested if needed.
Genetic tests – if needed will be done by analyzing the bone marrow of the afflicted person for genetic mutations which could be linked with polycythemia vera.

Diagnosis of Polycythemia Vera

This condition needs to be diagnosed by following the major and minor criteria of diagnosis included in the most recent guidelines (established by ‘WHO’ in 2008). They are –

Major criteria for PV –

  • Hb level greater than 18.5 gm/dl (men) or 16.5 gm/dl in women (or similar numbers based on hematocrit)
  • Presence of JAK2 mutation

Minor criteria for PV –

  • Bone marrow evidence of increased RBC production
  • Decreased EPO levels

When to consult doctor?

Call or consult a doctor / emergency if you find the below mentioned symptoms –

Prevention of Polycythemia

Congenital and primary polycythemia disorders cannot be prevented. Below mentioned are the preventive measures of preventable polycythemia.

  • Omitting / avoiding smoking
  • Omitting / avoiding exposure to carbon monoxide
  • Controlling high blood pressure and diabetes mellitus (risk factors for heart failure)

Treatment

Polycythemia is a chronic condition. It doesn’t have a known cure.
Aim of treatment –manage the condition by not only reducing the RBC count but also to reduce the risk of complications (like blood clots etc). Treatment of polycythemia depends on the underlying cause. Treating the underlying cause might help in resolving the condition.

Below mentioned are the best treatment options for PV
Phlebotomy – The treatment options are more specific in case of PV or other primary polycythemia syndromes. Phlebotomy (bloodletting) is the most essential part of the treatment. It includes manually removing the blood through one of the veins with an aim to reduce the RBC count. Certain amount of blood may be removed at pre-set intervals until the RBC count gets closer to the normal levels. The goal of phlebotomy is to reach the recommended hematocrit i.e. less than 45 in men and less than 42 in women.
RBC reducing drugs – To suppress the abnormal production of RBCs, several medications have been considered along with phlebotomy. It may be recommended if red blood cell growth cannot be controlled by phlebotomy. These drugs are called as myelo-suppressive drugs. Many of these drugs (chemotherapeutic) are linked with side effects. Their use has been controversial and limited.
JAK2 Inhibitors – may be recommended when a person doesn’t respond well to other medications.

Other medications – like aspirin (which helps to reduce the risk of clotting) and antihistamines (which help to relieve itching) may be recommended to control the symptoms.
For primary polycythemia hydroxyurea is the recommended drug especially to those having higher risk of blood clot formation. Aspirin and other anti-platelet agents may be helpful. The goal should be to treat the underlying condition in case of secondary polycythemia.
Read – How To Improve Blood Circulation? 25 Ayurveda And Yoga Tips

Prognosis

The type of polycythemia should be determined. The outlook in this disease depends mainly on the underlying cause. The cause is the key. Barring those with secondary causes, the overall outlook for polycythemia patients is favorable.
In PV the treatment would focus on managing the condition and also the symptoms. Currently there is no comprehensive cure for this condition. In most cases the quality of the people with PV will be almost near to normal and can be effectively managed with proper and timely interventions. Newer interventions will focus towards helping the progression of the disease to slow down.
Primary polycythemia – In many people it is incurable and runs a chronic course. In many others it is controllable and treatable. Example – untreated PV was thought to have a poor prognosis initially. In current day, the prognosis of the same condition has greatly improved to 10-15 years survival after diagnosis, with treatment by phlebotomy alone (against 1-2 years life expectancy which was thought of earlier). The survival rates and chances may improve even more following the addition of medications such as hydroxyurea or aspirin.

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