Blood Disorders
There are various disorders of the blood:
Anemia / Qillat al-Dam
A condition in which the hemoglobin level in the blood falls below the prescribed amount is called anemia. At birth, a child's blood contains 20 grams of hemoglobin per liter, but this gradually decreases with age. In adulthood, it remains at 14 grams per liter, while in women it is 12 grams per liter. When hemoglobin falls below this level, anemia develops and the following symptoms appear:
Weakness, fatigue, pale skin color, restlessness and lack of interest in work, weak eyesight, insomnia, headache, shortness of breath, heaviness in the ears and hearing sounds, swelling of the legs, soreness of the mouth, redness and swelling of the tongue, weak and brittle nails, difficulty in swallowing food. Patients, especially children, may eagerly eat मिट्टी (soil) and similar substances. Due to a deficiency of red blood cells, the blood becomes thin. The walls of the arteries become delicate and may rupture, causing bleeding from any body opening. In laboratory tests, H.B falls below 14 and ferritin falls below 30 micrograms. The M.C.V of the red blood cells decreases.
Causes: Toxins, attack of harmful organisms, liver diseases.
Leukemia (Lymphatic White Blood Disease)
The glands of the neck, armpits, and groin become swollen and feel like closed boils or lumps. The two glands beneath the jaw enlarge. Pain is felt beneath the ribs on the left side. Due to enlargement of the spleen, the number of white blood cells increases. Excessive saliva and drooling occur. The entire body feels exhausted and itchy. Albumin appears in the urine test. Appetite is lost, nausea and vomiting occur. The teeth become loose and chewing food becomes difficult. A white coating appears on the patient's tongue. The spleen and liver enlarge. Cough and diarrhea occur, and the fever gradually rises to 104 degrees.
There are four types of leukemia:
Chronic Myelogenous Leukemia
Chronic Lymphocytic Leukemia
Acute Leukemia
Hairy Cell Leukemia
1- Chronic Myelogenous Leukemia:
This occurs due to excessive production of myeloid cells in the bone marrow, resulting in a very high number of white blood cells in the blood and an increase in myeloblast cells.
A major cause of this disease is an abnormal chromosome known as the Philadelphia chromosome.
Symptoms:
It mostly occurs after the age of forty.
Fatigue, weakness, fever, night sweats, enlargement of the spleen, pain in the left ribs and abdomen, difficulty breathing, and darkness before the eyes.
Diagnosis:
In blood tests, the white blood cell count rises from one and a half lakh to five lakh. Blast cells are also present in the blood. Platelets may be normal or greatly increased. In advanced disease, platelet counts decrease. In bone marrow tests, myeloblast cells are less than 7%, and alkaline phosphatase levels in white cells are low. Philadelphia chromosomes are present. If proper treatment is not provided for 4 to 5 years, the patient enters a blast crisis stage, after which survival may only be a few months.
2- Chronic Lymphocytic Leukemia:
This disease generally occurs after the age of fifty. In this condition, the bone marrow does not function properly, resulting in impaired immunity. Large numbers of lymphocytes enter various organs of the body and directly cause damage.
Symptoms:
Fatigue, weakness, pain due to enlargement of the spleen, enlargement of glands in different parts of the body, anemia.
Diagnosis:
In blood tests, lymphocytes become smaller in size but increase from the normal 40% to 75–97%. Gamma globulin levels in the blood decrease.
Biopsy of the lymph nodes confirms the diagnosis. If the lymph nodes become excessively enlarged, platelet counts become very low. Without proper treatment, patients may survive for 6 to 10 years.
3- Hairy Cell Leukemia:
This disease usually occurs in people over fifty years of age. Since hairy cells are visible on the blood slide in this type of leukemia, it is called Hairy Cell Leukemia.
In this type of leukemia, platelet counts and white blood cells decrease, resulting in loss of immunity and repeated infections.
Symptoms:
Fatigue, weakness, pain due to enlargement of the spleen, enlargement of glands in various parts of the body, anemia.
Diagnosis:
Blood tests show reduced platelets and white blood cells. Monocyte counts are lower than normal. Hairy cells are visible on the blood slide. Bone marrow aspiration becomes dry.
4- Acute Leukemia:
The largest number of people are affected by this type, and it can occur at any age. It has two forms:
i- Acute Lymphoblastic
This mostly occurs in children between 3 and 7 years of age, although it may sometimes be found in adults as well.
ii- Acute Myelogenous
This usually occurs in individuals up to fifty years of age and occasionally in children.
Symptoms:
Extreme weakness and debility occur.
Various types of infections develop in the body.
Blue marks begin to appear under the skin. This is subcutaneous bleeding.
Mouth ulcers develop. The liver, spleen, and other lymph nodes often enlarge. Bone pain develops. The patient's complexion becomes yellow.
Diagnosis:
In blood tests, W.B.C, R.B.C, and platelet counts decrease. Blast cells increase in number. More than 30% blast cells are found in the bone marrow. Uric acid levels in the blood increase.
Causes: Excessive use of phlegmatic foods, warm-moist or cold-moist climates, indigestion, liver weakness.
Thalassemia
While in the mother's womb, a child receives all necessities from the mother's blood. During this period of life, the blood contains a unique type of hemoglobin called H.B.F. When the child is born and begins breathing in open air, H.B.F gradually starts to disappear. Another substance called H.B.A takes its place until by the age of two years H.B.F remains only two percent and the rest is H.B.A. This process is called Switching. It has three forms:
i- Complete Switching:
In this condition, H.B.F almost completely disappears and is replaced by H.B.A. This occurs in healthy individuals.
ii- Incomplete Switching:
In this condition, H.B.F remains present in the blood and H.B.A is not produced. These people also live normal lives.
iii- Excessive Switching:
In this condition, H.B.F stops being produced and H.B.A is not formed. This is Thalassemia. If these children do not receive blood for four to six weeks, the resulting deficiency may become fatal. In this disease, the liver and spleen enlarge and the bones become weak.
Nature of the Disease:
Thalassaemia is a Greek word. Thalassa means "sea" and Aemia means "blood." Since this disease was mainly widespread in countries around the Mediterranean Sea, it was named Thalassaemia.
It has two types:
Minor
Major
1- Minor:
The Minor form occurs in children whose parents, or one of the parents, carry thalassemia genes. Daily life is generally not greatly affected in Minor patients, but some patients, especially women, may develop anemia during pregnancy. In some such cases, iron is completely prohibited. Only one tablet of Folic Acid daily may be given.
2- Major:
This is the most severe form of the disease. It usually occurs in children whose mother and father both carry thalassemia genes. The lives of these patients depend upon donated blood. During life, the following symptoms appear:
1- Breathlessness
2- Pallor
3- Fatigue
4- There may also be jaundice
5- Leg ulcer
6- Enlargement of the liver and spleen
7- Gallstones
8- Growth retardation, anorexia, peevishness
9- Dehydration due to loose motions
10- Anaemic state
11- Thickness of skull bones
12- Osteoporosis, fracture of long weight-bearing bones easily
13- A brown pigmentation is seen around the eyes, at the root of hairs, on the back of hands and in other places
14- The venous network on the skin of the head is very pronounced
15- Dyspnoea from slight muscular exertion
16- The life span of erythrocytes in circulating blood is very short
17- Increased bilirubin level in blood
18- Urobilin in urine
19- Due to iron load, damage of the heart muscles
20- Heart failure
Children suffering from this disease should not be allowed to have intestinal worms; otherwise, anemia becomes severe.
Special attention is given to the H.B level of patients with Thalassemia Major. Their H.B should not fall below 9 g/dl. As soon as it drops below this level, a blood transfusion should be arranged immediately. In this way, the growth and development of children continue and the chances of spleen enlargement are minimized.
If the H.B level falls to 6–7 g/dl, it is extremely harmful to health. Efforts should be made to ensure that it never falls below 10 grams. Otherwise, along with anemia, the child's complexion becomes pale and the spleen enlarges.
A patient with Thalassemia Major requires 250 cc of packed red cells. Keeping this requirement in view, 250 cc of packed red cells are needed to raise the H.B level to 8–13 g/dl. When children are small, blood is given at the rate of 20 ml of blood per kg of body weight. Generally, a child weighing 10 to 12 kilograms is given a full 250 ml blood bag.
When children with thalassemia grow older, they require more blood to raise their H.B level. By the age of 10 to 15 years, larger children may receive two blood bags twice a month. Thus, according to the literal meaning of thalassemia, a sea of blood is required for the survival of these patients.
If blood is not provided on time, enlargement of the spleen and liver occurs. Iron load develops.
Patients with thalassemia receive one bottle of blood every month, which contains approximately 200 milligrams of iron. With every blood transfusion, this iron accumulates in the body. This excess iron, called Ferritin, cannot be excreted by the body of thalassemia patients as efficiently as in healthy individuals. This condition is commonly called Iron Load and medically termed Haemochromatosis.
This excess iron begins to accumulate in the body, especially in blood-reservoir organs such as the liver, spleen, kidneys, and voluntary red muscles. Due to Iron Load, these important visceral organs are unable to perform their normal functions. The most dangerous effects of Iron Load occur in the heart. First, due to anemia, dystrophy begins to develop in the middle muscular layer of the heart. Secondly, because of Haemochromatosis, the heart muscles become severely damaged, and death may occur due to cardiac failure.
Iron Load affects the growth and development of the child. Furthermore, if the beta cells of the pancreas become affected in Haemochromatosis, atrophy of the insulin-producing beta cells may occur, resulting in Diabetes Mellitus.
In patients with Thalassemia Major, iron storage begins even before blood transfusions due to the breakdown of red blood cells. From the first month after birth until the age of two years, a considerable amount of excess iron becomes stored in the body.
A specific Ferritin Test is performed to determine the quantity of this excess iron. To prevent Iron Load, this test is carried out every six months. The normal ferritin level in serum is less than 250 mg/ml, but in these patients it continues to increase. When the level exceeds 1000 mg/ml, Desferal injections should be used. If the ferritin level reaches 1000 to 2000, Desferal is administered daily at a dose of 25 mg per kilogram of body weight. If it reaches 3000, the daily dose should be 35 mg per kilogram of body weight. If it exceeds 3000, Desferal should be given daily at 45 mg per kilogram of body weight.
Test for the Diagnosis of Thalassemia:
Haemoglobin Electrophoresis
Causes: Corruption of black bile turning into poison.
Hemophilia
Hemo means "blood" and philia means "love" (i.e., love of blood).
This is a serious disease in which the blood's ability to clot becomes extremely reduced.
Blood Coagulation (Coagulation):
Several substances are required for blood coagulation, including:
1- Prothrombin
2- Thrombin
3- Thromboplastin
4- Fibrinogen
5- Calcium in Ionic Form
6- Vitamin-K
In the presence of calcium ions, thromboplastin converts prothrombin into thrombin. Thrombin then acts on soluble fibrinogen in the plasma, resulting in the formation of insoluble fibrin.
Fibrin forms a special type of mesh in which blood cells become trapped and entangled, thereby forming a blood clot. As fibrin contracts, fibrinogen-free plasma is released, forming blood serum. When blood escapes from any part of the body, thromboplastin is released from the injured tissue and damaged platelets, initiating the process of coagulation.
Blood coagulation can also be understood through the following principle, known as the Cascade Hypothesis:
Prothrombin + Thromboplastin + Calcium Ions — Thrombin
Thrombin + Fibrinogen — Fibrin
Cold temperatures slow down blood coagulation, while warm conditions accelerate it. Smooth body surfaces and substances that bind with inorganic salts such as magnesium, sodium sulfate, and calcium, for example Ethylenediamine Tetra Acetic Acid (E.D.T.A), slow the coagulation process. Snake venom, heparin, and hirudin also slow blood coagulation.
Coagulation Time:
The average normal coagulation time ranges from 6 to 17 minutes.
Coagulation Factors:
The substances technically required for blood coagulation are called factors. These factors are designated by Roman numerals, although each has its own name and function:
i- Fibrinogen Factor
ii- Prothrombin Factor
iii- Tissue Factor
iv- Calcium Ions
v- An unstable protein substance with a molecular weight of 290000
vi- Accelerin (now obsolete)
vii- Proconvertin or Serum Prothrombin Conversion Accelerator
viii- Anti-Hemophilic Factor
ix- Plasma Thromboplastin Component or Christmas Factor
x- Stuart Factor or Power Factor
xi- Plasma Thromboplastin Antecedent
xii- Hageman or Glass Factor
xiii- Fibrin Stabilizing Factor
Types of Hemophilia:
Basically, it has two major types:
Hemophilia A
Hemophilia B
1- Hemophilia A:
Hemophilia A is also called Classic Hemophilia and Factor VIII Deficiency Hemophilia. It is an inherited disease in which bleeding occurs due to deficiency of clotting Factor VIII. In affected patients, the quantity of clotting protein VIII is reduced, although in some patients the quantity may not be reduced but the factor itself is defective.
Hemophilia is a chromosome-linked disease that principally affects males. In rare cases, it may also occur in females.
Classification
1- Mild:
When the amount of Factor VIII-C involved in coagulation is 5%, it is considered mild hemophilia.
2- Moderate:
When the amount of Factor VIII-C involved in coagulation is up to 1.5%, it is considered moderate hemophilia.
3- Severe:
When the amount of Factor VIII-C involved in coagulation is less than 1%, it is considered severe hemophilia.
Symptoms of Hemophilia:
Hemophilia A is a severe inherited bleeding disorder. Worldwide, approximately one person out of every ten thousand is affected by this disease. The tendency to bleed is related to a coagulation factor known as Factor VIII-C or Anti-Hemophilic Factor.
A hemophilia patient may experience bleeding from any opening or location in the body. Common sites of bleeding include the elbows, knees, muscles, and anal region.
Note: Bleeding inside joints, muscles, knees, ankles, and elbows is called Hemarthrosis, while bleeding from body openings is called Hemorrhage.
Patients with mild hemophilia usually experience bleeding after injury or surgery, whereas severe hemophilia may cause bleeding at any time.
Tests for Diagnosis:
1- Activated Partial Thromboplastin Time (A.P.T.T)
2- Factor VIII
3- Factor IX
4- Prothrombin Time
5- Coagulation Factor Assay
6- Von Willebrand Factor Antigen
7- Factor IX Complex
8- Von Willebrand Factor Antigen
9- Platelets Aggregation
10- Fibrinogen
11- Aspirin Tolerance Test
12- C.B.C
13- Platelets Count
Interpretation of Tests:
1- Activated Partial Thromboplastin Time (A.P.T.T / P.T.T)
Normal range: 25 to 39 seconds
It is increased in both Hemophilia A and B.
2- Factor VIII
Synonyms: VIII-C:Ag (FVIII), A.H.F
Normal range: 50% to 150%
Plasma Concentration: 100 ug/L
Result: Reduced in Hemophilia A.
3- Factor IX
Synonyms: Hemophilia B, Christmas Disease Factor, Plasma Thromboplastin Component
Normal range: 50% to 150%
Severe degree: Less than 1%
Moderate degree: 1% to 10%
Mild degree: 11% to 49%
4- Prothrombin Time (P.T)
Normal range: 10 to 13 seconds
Result: Increased in hemophilia.
Note: In hemophilia, P.T.T is prolonged, whereas Prothrombin Time, Bleeding Time, and Fibrinogen levels remain normal.
The amount of VIII-C decreases while the Von Willebrand Factor remains normal.
If plasma from a healthy person is added to the plasma of a hemophilia patient, the P.T.T becomes normal. If the P.T.T does not become normal, it is considered a reliable indication of the presence of a Factor VIII inhibitor. If the platelet count decreases, it indicates HIV-associated immune thrombocytopenia.
Causes: Excessive use of cold-moist foods, toxins.
AIDS
AIDS means Acquired Immuno Deficiency Syndrome (Symptoms of Deficiency of Immunity).
It is a viral infection spread by the HIV virus. After entering the body, the virus may not produce symptoms for many years, but once symptoms appear, the patient remains in a constant struggle between life and death. The lymph glands become swollen but remain painless and may stay enlarged for many months. Influenza occurs, the throat becomes swollen, dysentery develops, eruptions appear on the body, fever develops, and weight begins to decrease. Severe weakness occurs. On C.B.C testing, platelet counts are found to be low and W.B.C counts are also reduced, with a persistent complaint of anemia. Due to muscle wasting, the patient lacks the strength to perform any work. Profuse sweating occurs. Severe itching develops on the skin that does not improve quickly with ordinary medicines, and tumor-like growths form in clusters in the food pipe. Severe cough resembling pneumonia and asthma develops.
Diagnosis:
If "HIV by Screening Test" is positive, then the patient is HIV positive. Further details may be obtained through the Elisa Method.
The HIV virus does not survive on non-living objects; therefore, it is not spread through food, clothing, or other such items.
Eosinophilia
W.B.Cs are an important part of the body's defense system. They fight living and non-living microscopic agents that enter the body and cause disease, protecting the body from their harmful effects.
Eosinophil cells belong to the group of Polymorphonuclear Cells among the white blood cells (Leukocytes). Due to the granules present in the cytoplasm of these cells, they appear granular in structure. Therefore, Polymorphonuclear Cells are also called Granular Leukocytes.
An Eosinophil cell measures 8 microns. Its nucleus consists of two lobes connected by a chromatin thread. Red granules are present in its cytoplasm. Like other leukocytes, they are also an important part of the body's defense system.
Eosinophils constitute about 2–3% of leukocytes, but in certain diseases, especially allergic disorders and parasitic infections, their number increases excessively. An increase above the normal level is called Eosinophilia.
Role of Eosinophils:
Like Phagocytes (whose function is to capture and digest disease-producing microorganisms), eosinophils possess the ability to leave the bloodstream and enter affected tissues where they destroy parasites. Some parasites are so large that eosinophils cannot engulf them. In such cases, eosinophils attach themselves to the parasites or their larvae and release substances that kill them.
In allergic reactions, mast cells and basophils play an important role. These cells release several chemical substances, including heparin and histamine, which are responsible for allergic reactions. These chemicals also stimulate eosinophils to leave the bloodstream and enter tissues where allergic reactions occur. Eosinophils neutralize the excess heparin and histamine that accumulate at the affected site and are released from basophils, mast cells, and damaged tissues during allergic reactions. In this way, inflammation and swelling remain confined to the affected area and do not spread to other organs.
Eosinophils increase in the following diseases:
Intestinal worms, allergy, sneezing, itching, skin ulcers, dermatitis.
Diagnosis:
1- Presence of worms and eggs in stool.
2- Presence of eggs and larvae in sputum examination.
3- Increased levels of IgG and IgE in blood serum.
4- Increased eosinophil count.
Causes: Excessive use of phlegm-producing foods, intestinal worms, and allergic conditions.