Diseases of the Hematological System

Hematological system consists of the blood and blood vessels (a tubular structure carrying blood
through the tissues and organs), bone marrow (a soft fatty substance in the cavities of bones, in which
blood cells are produced), spleen (an abdominal organ involved in the production and removal of blood
cells and forms part of the immune system) and other tissues which are intertwined with all other major
organ systems in the body

Blood – makes up 7% of the body’s total weight. The average adult has about five liters of blood
pumping around their circulatory system. Men have slightly more blood than women because women
have slightly more adipose or fatty tissues, which need very little blood supply.

Composition of blood
1. Transferrin – the protein responsible for the transport of iron
2. Plasma – the liquid portion of the blood where its constituents are dissolved or suspended. It
contains water, proteins (albumin, globulins, fibrinogen and prothrombin), electrolytes, gases,
non-proteinaceous compounds, wastes and nutrients.
3. Leukocytes – are white blood cells that arise from the same parent cells in the bone marrow.
These are the body’s defense against disease.
4. Hematocrit – packed red blood cell volume expressed as a percentage of whole blood upon
centrifugation.
5. Hemoglobin - the four pyrrole ring compound in red blood cells that contains iron centers and is
responsible for the transport of oxygen.

Homeostatic Control of the Hematological System

Homeostatic involves the regulation of bleeding, clotting and blood flow. Dietary factors play a central
role in the regulation of the process.

Nutrients that play an important role in normal clotting, bleeding and blood flow
1. Vitamin K
2. Dietary fats
3. Antioxidants like Vitamin C, Vitamin E and selenium
4. Pro-oxidants like iron and copper

Factors affecting homeostasis

1. Trauma (result of an overwhelming amount of stress that exceeds one’s ability to cope)
2. Autoimmune diseases like systemic lupus, thyroiditis
3. Malignancy (presence of cancer)
4. Inborn errors of metabolism (rare genetic disorder in which the body cannot properly turn food
into energy)
5. Medications
a. Penicillin and streptomycin can cause thrombosis
b. Aspirin, NSAID and certain antibiotics can increase bleeding tendencies
c. Cancer chemotherapy that targets bone marrow and spleen tissue
d. Anti-retroviral for HIV and AIDS causes abnormal clotting by decreasing the red bone
marrow’s ability to produce platelets
e. Statins, antidepressants, antihistamine, anesthetics, calcium channel blocker can increase
risk for bruising, bleeding and hemostatic deregulation in susceptible patients.
Nutritional Anemias
Categories:
1. Macrocytic (large cell size) It results from decreased ability to synthesize new cells and DNA due
to deficiencies in cyanocobalamin, folate, thiamin or pyridoxine.
a. Iron-deficiency anemia is the most common nutritional anemia and affects many different
groups. The most vulnerable groups are children under the age of two, menstruating
females, pregnant women and frail elderly persons. It is a condition where there is a
decrease in the number of normal circulating RBC per cubic millimeter of blood, decreased
levels of hemoglobin or decreased volume of packed RBC per dL of blood as a result of
greater demand on stored iron that can be supplied.
Etiology: blood loss in gastric ulceration or dysmenorrhea (abnormal menses)
Nutrition Therapy: continued iron-dense with Vitamin C rich food, nutrition-dense dietary
intake, supplementation to correct underlying conditions that causes deficits.
b. Functional Anemia is characterized by the presence of adequate iron stores but with
insufficient iron mobilization to adequately support.

2. Microcytic (small cell size) is caused by impaired heme synthesis, as a result of inability to
absorb, transport, store or utilize iron, or impaired synthetic abilities from deficiencies of
protein, iron, ascorbate, vitamin A, pyridoxine, copper or manganese. It may also be from
toxicities of copper, zinc, lead, cadmium or other heavy metals.
a. Pernicious anemia – is associated with Vitamin B12 deficiency that is slow, aggressive and
potentially life threatening. It is specific to gastrointestinal dysfunction like gastric
enterocystic atrophy, with diminished availability of intrinsic factor, HCL and enzymes;
neuropathy results from prolonged deficiency.

3. Hemolytic anemia may be due to deficiencies or excesses of Vitamin E

Clinical signs and Symptoms of Anemia

1. Fatigue
2. Lethargy – the lack of energy and enthusiasm
3. Cheilosis, glossitis
4. Pallor
5. Pale sclera
6. Spoon-shaped fingernails
7. Clubbing of joints in the digits
8. Cold extremities
9. Muscle aches
10. Difficulty concentrating
11. Sleepiness
12. Irritability
13. General malaise
14. Gastrointestinal distress like nausea, vomiting, diarrhea and cramping
15. Reproductive dysfunction like amenorrhea and loss of libido
16. Cardiovascular sequelae like palpitation, tachycardia, dyspnea, angina
17. Paresthesias (tingling, numbness of extremeties) often times seen in pernicious anemia
Megaloblastic Anemias – the RBC has decreased capacity for oxygen transfer and is large, irregular and
immature. It is often observed with deficiencies of folate and cyanocobalamin’.
a. Pernicious Anemia -is specific to the gastrointestinal disorders that results from stomach lining
atrophy and inflammation.
Etiology: seen among persons over 65 as a consequence of aging, secondary to a disease state and
sometimes duet to poor intake of vitamin B12.

Hemochromatosis (iron overload) is a condition in which a number of regulatory mechanisms for iron
are inoperative. Th e primary result is the buildup of iron and pro-oxidant iron damage of cells. Primary
forms of hemochromatosis are hereditary, while secondary hemochromatosis is due to an alternate
condition such as liver disease.
Etiology:
- Increased iron absorption from the gut of heme or nonheme iron irrespective of intake. •
- Decreased removal of iron from circulation.
- Decreased ability to mobilize iron from the liver, so that excessive amounts build up and
cause hepatocytic damage.
- Decreased ability to synthesize hemosiderin, ferritin, transferrin, and iron-containing
cytochromes, the specifi c proteins for iron transport, sensing, storage, and utilization.
- Homozygous and heterozygous mutations of HFE-b2 microglobulin, a protein associated with
the affinity for transferrin receptors in the membrane of the enterocytes lining the
gastrointestinal tract.

Pathophysiology
Chronic toxicity from iron overload leads to functional diabetes, hepatomegaly (enlarged liver),
cicatrix formation, and eventux ally cirrhosis, through oxidative damage to cellular membranes in
nearby locations. Hepatolenticular carcinoma, a cancer of a specifi c set of liver cells, develops in
30% of cirrhotic patients, due to pro-oxidant changes in the nucleic acids of cells. Cardiomyopathy
develops and leads to heart failure in approximately 50% of patients, through a variety of
mechanisms.

Clinical Manifestations
The patient may present with non-specific symptoms. Feeling cold, tired, or irritable or being unable
to eat are common complaints. Slate grey skin is characteristic, along with abnormal blood glucose
levels.

Hemoglobinopathies: Non-Nutritional Anemias

a. Sickle Cell Anemia – found in areas where malaria is endemic. It is an inherited form of anemia, a
condition in which there are not enough healthy red blood cells to carry enough oxygen throughout
your body. There is no cure but treatments can relieve pain and help prevent problems associated with
the disease.
Clinical Manifestation:
a. anemia due to decrease amount of red blood cells that is why the body does not get oxygen it
needs to feel energized, causing fatigue.
b. episodes of pain
c. painful swelling of hands and feet
d. abdominal swelling
 e. fever
f. pail skin or nail beds
g. signs of stroke like one-sided paralysis or weakness in face, arm or legs, confusion, trouble
walking or talking, sudden vision problems, unexplained numbness or a headache.
Treatment:
Medical: blood transfusion, bone marrow transplant or stem cell transplant, chronic anti-
biotic/pain medications
Nutrition:
a. Increase macronutrients
b. Optimize mineral intake
c. improve antioxidant status
d. ensure fluid adequacy and nutrient density

b. Thalassemia – is an inherited blood disorder in which the body makes an abnormal form of
hemoglobin, the protein molecule in red blood cells that carries oxygen.

Clinical Manifestation: stunting, birth defects, organ damage and severe hypoxia. Other signs
are fussiness, paleness, frequent infections, poor appetite, failure to thrive, jaundice and enlarged
organs.

Types:
a. thalassemia intermedia- is the less severe form. It develops because of alterations in
both beta globin genes. Blood transfusions are not needed. We need to have four genes, two from
each parent.
b. Alpha thalassemia – occurs when the body can’t make alpha globin.

Treatment:
Medical: transfusion, bone marrow transplant
Nutrition: ascorbate administration, antioxidant administration and ensure nutritional
adequacy

c. Polycythemia - is a slow-growing blood cancer in which your bone marrow makes too
many red blood cells. These excess thickens the blood, slowing its flow. They also cause complications
like blood clots, which can lead to a heart attack or stroke.

Clinical Manifestation: itchiness especially following a warm bath or shower, headache,

dizziness, bleeding or bruising, weakness, fatigue, blurred vision, excessive sweating, painful swelling of
one joint – often the big toe, shortness of breath, numbness, tingling or weakness in your hands, feet,
arms or legs, bloating in left upper abdomen due to enlarged spleen, fevers and unexplained weight loss

complications:
a. blood clots
b. splenomegaly – enlarged spleen
c. problems due to high levels of red blood cells
d. other blood disorders

Nutrition Therapy:
 1. increase dietary iron
2. ensure nutrient density
3. small frequent feedings
4. increase fluid intake

d. Aplastic anemia – is a rare and serious condition, a condition that occurs when your body stops
producing enough new blood cells. It leaves a patient feeling fatigued and with a higher risk of
infected and uncontrolled bleeding. It can develop at any age.

Etiology:
a. Radiation and chemotherapy treatments
b. Exposure to toxic chemicals
c. use of certain drugs
d. Autoimmune disorders
e. Viral infections
f. Pregnancy
g. unknown factors which doctors call idiopathic anemia

Treatment:
Medical: transfusion, bone marrow transplant, corticosteroid, immunosuppresants
Nutrition: Ensure nutrient density
maintenance of normal fluids
maintenance of sodium
maintenance of adequate macro and micronutrient status
maintenance of calcium and Vitamin D

Hemophilia – is the hereditary inability to make needed clotting factors

Clinical Manifestation: bleeding, joint and soft tissue damage, pain, loss of function
treatment:
Medical: synthetic clotting factor administration
Nutrition: base on TCR to maintain nutritional status
ensure antioxidant adequacy

Hemorrhagic disease of newborn – is a Vitamin K deficiency which is seen in infants with a

sterile GI tract
Clinical Manifestation: bleeding from umbilicus, skin or orifices post delivery
Treatment:
medical: 0.5-1.0 mgs phyloquinone given by IM
Nutrition: optimal feeding practices for young infants

Thrombosis – is the formation of blood clots known as thrombus

Etiology:
1. Atherosclerosis
2. chronic inflammation
3. increased blood viscosity
4. increased platelet activity
 Risk factors:
1. high fat diet 6. hypertension
2. high cholesterol diet 7. oxidative stress
3. smoking 8. hyperhomocysternemia
4. sedentary lifestyle 9. increased coagulation
5. obesity factors
Treatment:
Medical: anticoagulants
Nutrition: vitamin K-rich foods

CONCLUSION:
Many types of blood disorders affect bone marrow and red or white cell types, including nutritional
deficiency anemia, inherited disorders of RBC synthesis, clotting disorders and cancers.
Nutritional interventions differ and will depend on the etiology of the disease. Nutrition therapy should
include adequacy of micronutrient intake with special attention paid to iron,
cyanocobalamin and folate.