HAEMATOLOGICAL DISORDERS by MikeJenny

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									             BLOOD
 Hippocrates mentioned that blood is a
 physical representation of the four
 humours. That is, the red portion
 denotes the sanguinous humour, the
 watery component represents the
 phlegmatic humour, the froth
 represents the bilious humour and the
 residue which sinks to the bottom is the
 melancholic humour.
                 BLOOD
 According to Tibb, humours are produced in
 the liver. Therefore, the efficient functioning
 of the liver is essential for maintaining the
 ideal integrity of blood constituents. Whilst
 the physical production of blood cells may
 be in the bone marrow, blood disorders are
 invariably the result of inadequate humours
 produced in the liver.
              BLOOD
 The concept of haematology from the
 Tibb perspective relates not only to the
 qualities they possess but also within
 the physical parameters that these
 qualities translate into. These include
 the viscosity of the blood, the
 temperature of the blood, the moisture
 content and the purity of the blood.
 THE NATURE AND FUNCTION OF
 BLOOD
 Blood is the red, thick, viscous specialized fluid
  which is pumped by the heart around the body via
  the arteries, capillaries and veins of the
  cardiovascular system.
 The main function is the provision of nutrients to
  the cells as well as the elimination of by-products of
  metabolism.
 Provides the vehicle by which an immense variety of
  different substances are transported between the
  various organs and tissues.
 Blood is fundamentally important in assisting
  Physis.
          Function of blood
 Blood serves the body in three ways:
1. Transportation-of O2 from the lungs, nutrients and
   vitamins absorbed from the digestive system, waste
   products such as urea, uric acid, creatinine and CO2 from
   the tissues and hormones from the endocrine glands.
2. Regulation-blood helps maintain the acid-base balance
   (pH) of body fluids; the amount of fluid in the tissues is
   adjusted by altering osmotic pressure; heat generated in
   the muscles is transmitted to other parts of the body.
3. Protection- a) immune system; b) antibodies; c)
   coagulation
                STATISTICS
 Blood accounts for approximately 8% of body weight
 Total volume of approximately 5L in an adult male
  (rather less in an adult female)
 250millilitres in a new-born baby
 Blood makes a complete circuit of the body in about
  one minute
 Blood is classified as connective tissue because nearly
  half is made up of cells.
 Blood varies in colour from bright scarlet to dark red,
  depending on how much O2 it is carrying.
    COMPOSITION OF BLOOD
 PLASMA- contains proteins such as albumins, globulins and
    fibrinogen, nutrients, hormones, mineral electrolytes and metabolic
    end products.
   Blood cells are mostly in the bone marrow-the soft tissue found in the
    centre of bones and this process is called erythropoiesis. Some blood
    cells are also made in the spleen. In children, blood cells are mainly
    made in the marrow of the long bones. In adults, most production
    takes place in the flatter bones e.g. the pelvis
   RED BLOOD CELLS- make up around 99% of all blood cells, contain
    the O2 carrying protein haemoglobin.
   WHITE BLOOD CELLS- include the granulocytes,neutrophils,
    eosinphils and basophils. These have segmented nuclei. Also included
    are the monocytes and lymphocytes. These have single nuclei. The
    white blood cells are all involved in various aspects of the body’s
    defence against micro-organisms and the immune system.
   PLATELETS- are small blood cells crucially important for blood
    coagulation. They are also formed from the stem cell system.
                   Red blood cell




                   platelets




White blood cell
      FORMATION OF BLOOD
 All blood cells are derived form stem cells present in the bone marrow.
    Both red & white blood cells are produced in the bone marrow, and
    broken down by the spleen. In addition, white blood cells are formed in
    the liver, spleen and lymph nodes.
   Red blood cells enter blood vessels via the nutrient circulation.
   Vitamin B12, vitamin C and folic acid are vitally important for the
    synthesis of red blood cells.
   The elements iron, cobalt and copper are also essential.
   Red blood cell production is controlled by a hormone secreted by the
    kidney-erthyropoietin-which stimulates bone marrow.
   Red blood cells are made at an astonishing rate-literally billions per
    day. The reason is simply the fact that their survival time is short, as
    they are worn out very rapidly.
    THE NATURE OF PLASMA
 This clear fluid occupies around 55 % of blood volume. The
   main components are:
1. 90% water
2. 7% proteins (mainly albumin, globulins, fibrinogen) &
   lipoproteins
3. 3% small molecules (mostly salts, vitamins, hormones,
   urea, amino acids)
4. 0.1% glucose
 The proteins help maintain the osmotic pressure, to hold
   fluid within the circulation and counteract the blood
   pressure that forces it out of the capillaries. The gamma
   globulins defend the body against infection.
Nature of Plasma
the plasma proteins include:
 Albumin-is synthesized by the liver. It maintains
  the osmotic pressure of the blood
 Gamma globulin- this contains the various
  antibodies needed by the immune system
 Blood clotting factors- fibrinogen, plasminogen
  and other proteins
 Complement- a group of enzymes which helps
  antibodies fight against pathogens.
Nutrients found in the plasma
1. Glucose- a simple sugar absorbed by the capillaries
   in the intestine following digestion
2. Amino acids- the products of protein digestion; they
   too are absorbed from the digestive tract through the
   intestinal capillaries.
3. Lipids- used as a source of energy, or stored in fat
   deposits for reserve energy.
Serum is the same as plasma, but without the
   fibrinogen.
RED BLOOD CELLS
• A red blood corpuscle is circular, bi-concave in shape & about 7.5
microns in diameter.
• There are approximately 5 to 6 million per cubic millimetre of blood
normally
• The red blood cells contain the red pigment haemoglobin, an iron-
containing protein which carries O2 throughout the CVS. The red blood
cells have only one function in the body- THE TRANSPORT OF O2
• This is achieved by combining O2 reversibly with haemoglobin.
• Healthy men have about 15g of haemoglobin per 100ml blood; and
females 13g per 100ml
• The red blood cell survives for about 80 to 120 days in the CVS
• Approximately two million die every second
• Old and damaged red blood cells are removed by the reticulo-
endothelial system which is present in the liver and spleen.
WHITE BLOOD CELLS
 There are normally about 8000 of these per cubic millimetre of blood (1% of
  blood volume)
 There are two basic groups:
Granulocytes- also called polymorphonuclear leucocytes. These make up about
   65% of the white blood cell population. They survive for less than a week, they
   destroy invaders (allergens, bacteria, parasites etc.) & remove dead and
   damaged tissue cells. There are three different types of granulocytes:
 Eosinophils- cells are involved in defence against parasites, and in the allergic
   reaction
 Basophils- known to be involved in the allergic reaction as they contain
   histamine. They can also ingest foreign particles. In tissues, they change into
   mast cells.
 Neutrophils- are involved in killing bacteria and provide a defence against
   infection.
Agranulocytes-lymphocytes and monocytes. They are formed in the marrow,
   lymph glands, spleen and thymus. They help to maintain the immune system.
   The monocytes are larger cells, distinct from lymphocytes and function
   mainly by phagocytosis. In tissues, monocytes change into macrophages.
               PLATELETS
 These are found in the bone marrow
 They are cytoplasmic fragments of much larger cells
  called megakaryocytes.
 They do not have cell nuclei or DNA & do not divide.
 They do contain mitochondria and enzymes
 Platelets are extensively involved in the blood clotting
  (coagulation) process.
 They contain two contractile proteins, actin and
  myosin which are brought into play during clot
  retraction and wound healing.
 Platelets survive for 7 to 10 days in the bloodstream.
                 HAEMOSTASIS
 Blood flows freely in intact blood vessels due partly to an
    excess of naturally occurring anti-coagulants.
   However, if the blood vessel wall breaks, a series of
    chemical reactions begin to stop the bleeding
    (haemostasis).
    Without this process, even a simple cut would result in the
    bleeding to death.
   Although we can lose up to 15% of our blood without
    serious ill-effects, a greater loss can be severe, leading to
    shock and even death.
   Loss of blood (haemorrhage) internally or externally can
    lead to anaemia in the long term.
              HAEMOSTASIS
EVENTS IN HAEMOSTASIS INCLUDE:
1. Vasoconstriction- contraction of the smooth muscles in
   the blood vessel wall. In the short term, this markedly
   reduces the flow of blood and loss from the rupture in the
   blood vessel wall.
2. Platelet plug- any damage to the blood vessel activates
   platelets, which become sticky and adhere to each other
   and to the damaged area, forming a temporary plug.
3. Blood clotting- the platelet plug is reinforced with a
   fibrin meshwork across the wound. Red & white blood
   cells are trapped within this mesh, forming the familiar
   blood clot. This process is called blood coagulation.
            BLOOD COAGULATION
 The formation of a blood clot is a very complicated process involving over 30 chemicals.
  Some of these, called coagulation factors, enhance clot formation.
 13 coagulation factors are involved in clotting which occurs in injured tissue.
 The end product of this sequence is thromboplastin
 This is an enzyme which catalyses the conversion of a plasma protein, prothrombin, into
  a smaller protein called thrombin. Calcium and other factors are involved.
 Thrombin, in its turn, catalyses the joining of fibrinogen molecules present in the plasma
  to produce the typical fibrin mesh.
 It is this mesh that traps blood cells (red and white blood cells, platelets) in the blood
  vessel wall.
 Once the clot solidifies, the top layer of cells dies, forming the scab.
 The large number of chemical steps involved in the clotting process means that
  coagulation must be tightly controlled. This is extremely important, because unnecessary
  clotting can be very dangerous, especially if it blocks a blood vessel (thrombosis), in a
  major organ.
 When blood is removed from the body, it will automatically clot on contact with a surface
  than the blood vessel lining. The fluid remaining after clotting is the serum.
 Serum contains all the components of blood plasma except the clotting factors.
             FIBRINOLYSIS
 Once the tissue has healed, after 2 to 3 days, the fibrin
  mesh which holds the clot together is dissolved. This
  process , called Fibrinolysis, is catalysed by the enzyme
  plasmin, which is produced from the plasma protein
  plasminogen.
 Plasminogen molecules are incorporated into the blood
  clot during its formation. Here they lie dormant until
  activated by the healing process.
 Most of the body’s plasmin is restricted to the blood clot
 Normally, a fine balance between blood coagulation and
  Fibrinolysis is maintained by the body.
              STEM CELLS
 These are the ancestors of all blood cells.
 All types of blood cells are derived from the stem cells,
  which may go on to form red blood cells, white blood cells
  and platelets.
 There are two basic properties of stem cells:
1. They are potentially immortal, able to divide indefinitely
2. They can develop into adult types of cells.

Poor nutrition or a poorly balanced diet will obviously
result in an imbalance in humours. In addition, poor
elimination would result in accumulation of toxins, low
O2 levels, either in the blood or at the vascular level.
Predisposing factors
 People who have a dominant Phlegmatic temperament will be
    inclined to the haematological disorders.
   This is an outcome which could be predicted from a lack of heat
    and/or lack of dryness
   Most of the conditions associated with blood disorders will
    invariably proceed along pathway 2.
   However, there are exceptions-if the clinical condition is genetic, or
    if there is a sudden and excessive loss of blood due to trauma,
    surgical procedures or menorrhagia.
   The approach to treating disorders of the blood generally revolve
    around restoring the efficiency of the liver, by means of proper
    nutrition. Any deficiencies in the food consumed, either in mineral
    or other component, can be rectified by advising the sufferer to
    adhere to a food regimen that includes a balance of nutrients &
    minerals required for the proper synthesis of haemoglobin, red
    blood cells, white blood cells and platelets.
                 ANAEMIA
 C+M frame, excess phlegmatic humour, pathway 2
 Anaemia is a reduction in the blood’s ability to carry
  O2, due to a decrease in either a) the number of red
  blood cells or b) the amount of haemoglobin they
  contain.
 According to Tibb, anaemia can be the result of a C+M
  imbalance.
 Iron-deficiency anaemia is the most common form
 All age groups and both sexes are affected but anaemia
  is more common in younger women.
Clinical features
 The initial signs of developing anaemia may
  be:
 Loss of appetite
 Constipation
 Amenorrhoea
 Irritability
 Difficulty in concentrating
 Coldness of the hands and feet
Signs and symptoms of anaemia
Symptoms
 Tiredness & lethargy
 Dyspnoea
 Dizziness
 Heart palpitations
Signs
 Pale inner eyelids
 Sore tongue (glossitis)
 Cracking of the side of the mouth (angular stomatitis)
 Brittle hair and pallid skin
 Brittle nails (koilonychia)
 Older patients-chest pain & swollen ankles
Causes and risk factors
 Around 50% of cases of anaemia in the developing world occur in people
     with hookworm infestation.
    Anaemia arises from a number of reasons, depending on the type. The
     major ones include:
1.     Chronic inflammation in the body-this often appears with chronic
       diseases, such as infections, carcinomas, connective tissue disorders and
       kidney failure.
2.     Dietary factors-e.g. Low iron content of the food regularly consumed
       and a dietary deficiency in folic acid. Also, a lack of intrinsic factor
       (substance that gastric glands produce to promote absorption of vitamin
       B12) absorption resulting from auto-immune damage to the stomach
       lining.
3.     Excessive loss of blood from gastrointestinal disorders (e.g. Peptic ulcers,
       alcoholism and piles)
4.     Gynaecological disorders-repeated childbirth and heavy menstrual
       bleeding
5.     Disease-liver damage, thyroid disorders, bone marrow disorders,
       haemophilia, sickle cell disease, thalassaemia, kidney failure
Discuss treatment plan as well as medication for this condition with regard to
Tibb philosophy.
Angular stomatitis & angular cheilitis
There are several types and classifications of anaemia. This is a condition in which the
body lacks the amount of red blood cells to keep up with the body's demand for oxygen.
Understanding the different classifications can help to recognise the symptoms and also
to avoid anaemia in the first place...
Iron deficiency anaemia

Iron deficiency anaemia is a condition in which the body has too little iron in the bloodstream.
This form of anaemia is more common in adolescents and in women before menopause. Blood
loss from heavy periods, internal bleeding from the gastrointestinal tract, or donating too
much blood can all contribute to this disease. Other causes can be from poor dietary habits or
from chronic intestinal diseases.

The signs and symptoms of this disease are:
1. Paleness
2. Headache
3. Irritability

Symptoms of more severe iron deficiency anaemia include:
1. Dyspnoea
2. Rapid heartbeat
3. Brittle hair and nails

Treatment usually takes the form of oral iron supplements and dietary modifications.
Folic Acid Deficiency Anaemia

This form of anaemia is characterised by a lack of folic acid, one of the B group of
vitamins, in the bloodstream. This is usually caused by an inadequate intake of
folic acid, usually found in vegetables or by the overcooking of the vegetables.
Alcoholism can also be a contributing factor in this form of anaemia. During
pregnancy when the folic acid is used more or in infancy, this disease can also
manifest itself. It can also be caused as a side effect of other blood disorders.

Symptoms of the disorder include:
1. Weakness
2. Fatigue
3. Memory lapses
4. Irritability

This condition can be avoided by including foods with folic acid in the diet. Such
foods include beef liver, asparagus, and red beans.
Pernicious anaemia

Pernicious anaemia usually affects people between the
ages of 50 and 60 and is a result of a lack of vitamin B12.
The disease can be hereditary but some forms of the
condition can be autoimmune diseases. People who have
any autoimmune diseases are more likely to contract
pernicious anaemia.

Symptoms of this form of anaemia may include:
1. Fatigue
2. Dyspnoea
3. Heart palpitations
4. Numbness or tingling in extremities
Aplastic anaemia

Aplastic anaemia is caused by an absence or reduction of red blood cells.
This can happen through injury where the blood forming tissue in the
bone marrow is destroyed. Because of this, the sufferer is unable to fight
infection and is likely to be a heavy bleeder.

Symptoms include:
1. Lethargy
2. Paleness
3. Purpura
4. Bleeding
5. Rapid heartbeat
6. Infections
7. Congestive heart failure

There is no definite known cause for aplastic anaemia but it is thought to
be caused by exposure to certain toxins and also to the hepatitis virus.
Polycythemia Vera

This disease is more common in middle aged men and is
characterised by an increase in red blood cells, leucocytes, and
thrombocytes. There is a very fast and intense reproduction of
cells and the bone marrow cells mature more rapidly than usual.
The cause of this condition is unknown.

Symptoms are:
1. Purplish coloured skin
2. Bloodshot eyes
3. Headache
4. Dizziness
5. Enlarged spleen

Although the signs and symptoms of anaemia may seem
overwhelming, the good news is that most forms can be
effectively controlled with medication and dietary changes.
Sickle Cell Anaemia

This form of anaemia is of a hereditary nature and is a result
of an abnormal type of red blood cells. Sickle cell anaemia is a
life threatening disease and there is no prevention.

Symptoms of this condition include:
1. Painful attacks in arms, legs and stomach
2. Jaundice in whites of the eyes
3. Fever
4. Chronic fatigue
5. Rapid heartbeat
6. Paleness

Complications include leg ulcers, shock, cerebral
haemorrhage, and orthopaedic disorders.
What is fibrinogen?
Fibrinogen, also called Factor I, is a blood plasma protein produced by the
liver that plays an important role in blood coagulation. Blood coagulation is a
process in which several components of the blood form a clot. When blood
escapes from a rupture in a blood vessel, coagulation is triggered. Several
proteins, called coagulation factors, go into action to produce thrombin. The
thrombin then converts fibrinogen to fibrin. Fibrin produced from fibrinogen
is the main protein in a blood clot. It surrounds the cells in the blood and
plasma and helps form the clot. The resulting clot, which is stabilized by
Factor XIII, remains intact from 10 to 14 days, the time required for healing to
take place.

When there is a problem with fibrinogen, i.e., either it is missing or it does
not function properly, the clot has difficulty forming. This can result in
haemorrhaging or thrombosis.

The normal volume of fibrinogen in the blood is from 2 to 4 g/l (grams/litre).
The amount of fibrinogen in blood can be measured from a blood sample.
Types of Fibrinogen Deficiency
There are three types of deficiency:
Afibrinogenaemia: (absence of fibrinogen)
In this type of factor I deficiency, there is a complete absence of fibrinogen. The
fibrinogen level is <0.2 g/l of plasma. about 5 people out of 10 million are affected
by it. of the three types, this one causes the most serious bleeding.

Hypofibrogenaemia: (lower than normal level)
In this anomaly, fibrinogen is present, but at a lower level than normal, between
0.2 g/l and 0.8 g/l. This anomaly is less frequent than Afibrinogenaemia. Bleeding
problems may be mild, moderate or severe.

Dysfibrinogenaemia: (malfunctioning)
In Dysfibrinogenaemia, the fibrinogen level is normal, which means between 2
and 4 g/l, but the fibrinogen does not function properly. About 1 person in 1
million is affected by this condition. More than 100 different types of
Dysfibrinogenaemia have been reported. Those affected rarely suffer from
haemorrhaging problems. They may even present the opposite condition:
thrombosis (blood coagulates in the blood stream).
Transmission of Fibrinogen Deficiency
Fibrinogen deficiency is a very rare inherited bleeding disorder. It is transmitted from parent
to child at conception. The disorder is caused by an abnormal gene. It affects both men and
women, as well as people of all races and ethnic origins.
Every cell of the body contains chromosomes. A chromosome is a long chain of a substance
called DNA. DNA is organized in 30,000 units: these are called genes. The genes determine
physical characteristics, such as eye colour. In the case of fibrinogen deficiency, one of the
genes involved is defective.
The defective gene in fibrinogen deficiency is located on a chromosome that is not
responsible for the child’s sex (autosomal). As a result, both girls and boys can be affected
equally.

Afibrinogenaemia (absence of fibrinogen)
This is a recessive disorder, which means that both parents must be carriers. In order for a
person to inherit fibrinogen deficiency, he must receive two defective genes, one from the
mother and the other from the father. A carrier is a person who has only one of the two
defective genes, but is not affected by the disorder: the second gene enables just enough
fibrinogen to be made for good coagulation. The fibrinogen level will be lower than normal,
but there will be no symptoms of the disorder.

Hypofibrinogenemia and Dysfibrinogenaemia
These are inherited disorders that can be either dominant or recessive.
Dominant means that a single parent can transmit the disorder if he or she is a carrier.
Recessive means that both parents must be carriers of the disorder in order to transmit it.
Symptoms
Afibrinogenaemia (absence of fibrinogen)
In congenital Afibrinogenaemia (fibrinogen level <0.2 g/l), bleeding can vary, from slight to severe. many patients
have very long intervals between bleeding episodes. a diagnosis of Afibrinogenaemia is generally made postnatally,
usually because of bleeding from the umbilical cord and/or a haemorrhage following circumcision.
Other types of bleeding have been described:
• bruises
• bleeding from the gums
• epistaxis (nosebleeds)
• gastrointestinal haemorrhage
• genito-urinary haemorrhage
• intra-cranial haemorrhage
• rupture of the spleen and haemorrhage in the spleen
About 20% of those suffering from Afibrinogenaemia present hemarthroses (bleeding in the joints). Because of this
particular feature, the disorder may be confused with haemophilia A or B.

Hypofibrinogenemia (lower than normal level)
Bleeding in Hypofibrinogenemia is much like what is seen in Afibrinogenaemia. It can be more or less serious,
depending on fibrinogen levels, which can vary from 0.2 to 0.8 g/l of plasma.
The higher the fibrinogen level, the less bleeding.
The lower the fibrinogen level, the more bleeding.

Dysfibrinogenaemia (improper functioning)
In Dysfibrinogenaemia, the quantity of fibrinogen is normal, which means between 2 and 4 g/l. Bleeding can vary
depending on how the fibrinogen is functioning. Bleeding may:
be absent (no symptoms);
show a tendency toward haemorrhage (as described in Afibrinogenaemia);
show a tendency toward thrombosis.
 FIBRINOGEN DEFICIENCY
Also called Afibrinogenaemia
C+M frame due to excess
 phlegmatic humour, Pathway2
Fibrinogen is an important factor
 in the coagulation of blood. This
 kind of deficiency can lead towards
 disorder of low coagulation.
Treatments
Many people who have Hypofibrinogenemia or a Dysfibrinogenaemia do not need treatment.
To control or prevent bleeding, all that’s required is to increase the fibrinogen level in the blood with
blood products or substitutes. This kind of treatment is called factor replacement treatment. The aim of
the treatment is to increase the fibrinogen level to 1 g/L when there is minor bleeding, and 2 g/L for
serious bleeding or for surgery.
Fibrinogen concentrate can be administered by drip:
• at the time of surgery;
• to the mother during childbirth or after delivery;
• after a trauma;
• before dental surgery;
• as prophylaxis (prevention) for subjects with Afibrinogenaemia to prevent bleeding.
At the present time, the most frequently used treatment in Canada is fibrinogen concentrate. The
concentrate is obtained from human plasma and contains fibrinogen only. The concentrate undergoes a
viral inactivation process, which eliminates viruses such as HIV and hepatitis A, B and C.
It is impossible to completely eliminate the risk of transmitting infections that are currently unknown.
There are other options that can be considered for treatment:
• plasma
• cryoprecipitate (a precipitate produced by freezing and thawing under controlled conditions. An
example of a cryoprecipitate is the residue obtained from fresh frozen blood plasma that has been
thawed at 4 degrees Celsius . This residue is extremely rich in clotting factor.
However, their use is not recommended much nowadays for various reasons, such as:
slight risk of viral transmission;
possible serious allergic reactions because of the large number of different substances contained in
these products in addition to fibrinogen.
Anticoagulants are sometimes used to reduce the risk of thrombosis among patients with
Dysfibrinogenaemia.
        THROMBOCYTOPENIA
 C+M frame due to excess Phlegmatic humour, Pathway2
 This condition refers to a severe reduction in the quantity
    of platelets (thrombocytes) in the bloodstream.
   It arises either from a) an abnormally low production rate
    or b) an excessive destruction of thrombocytes.
   As a result there is a greater tendency for unexplained
    bruising and bleeding to occur especially in the skin and
    mucous membranes, as the blood is less able to clot.
   Anaemia and an enlarged spleen may develop
   Causes include cirrhosis of the liver, an enlarged spleen,
    alcoholism and a number of allopathic drugs.

								
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