Haematological Diseases-1

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HAEMATOLOGICAL DISEASES

 HAEMATOLOGICAL diseases are disorders which primarily affect the blood and blood
forming organs.
IRON DEFICIENCY ANEMIA

A condition of too little iron in the body

CAUSES OF IRON DEFICIENCY ANEMIA


• Blood loss
• An inability to absorb iron
• Pregnancy

RISK FACTORS
• Infants and children
• Vegetarians
• Frequent blood donors
PATHOGENESIS OF IRON DEFICIENCY
ANEMIA
 There are three pathogenic factors
1. Impaired Hb synthesis due to reduced ion supply
2. Generalized defect in cellular proliferation
3. Survival of erythroid precursor and erythrocytes is reduced
STAGES IN THE DEVELOPMENT IF IRON
DEFICIENCY
Stage 1 (prelatent) Stage 2 Stage 3
(latent) (anemia)
Bone marrow iron Reduced Absent Absent
Serum ferritin Reduced <12 µg/L <12µg/L
Transferrin saturation Normal <16% <16%
Free erythrocyte protoporphyrin, zinc Normal Increased Increased
protoporphyrin
Serum transferrin receptor Normal Increased Increased
Reticulocyte hemoglobin content Normal Reduced Reduced
Hemoglobin Normal Normal Reduced
Mean corpuscular volume Normal Normal Reduced
Symptoms Fatigue, Pallor, pica
malaise
STMPTOMS OF IRON DEFICIENCY ANEMIA

• Whole body-Dizziness , fatigue or light headedness

• Heart –Fast heart rate or palpitations

• Also common –Brittle nails , shortness of breath


TREATMENT FOR IRON DEFICIENCY ANEMIA

• Dietary supplement

• Vitamins helps promote normal body function growth and


development

• Consult a doctor for medical advice


MEGALOBLASTIC ANEMIA

MEGALOBLASTIC anemia is a condition in which


the bone marrow produces unusually large ,
structurally abnormal , immature blood cells.

CAUSES OF MEGALOBLASTIC ANEMIA

• VitB12 deficiency

• Folate deficiency
PATHOGENESIS OF MEGALOBLASTIC
ANEMIA
Lack of B12 allows folic acid to be trapped as non functional methyl
tetrahydrofolate (folate trap)

So deficiency of functional FH4 causes impairment of formation of deoxy


thymidine monophosphate which is needed for DNA synthesis

As a result large proerythroblast fails to divided rapidly to make mature RBC


rather immature precursors of erythrocyte appear to cause
megaloblastic anemia
SYMPTOMS OF MEGALOBLASTIC ANEMIA
Aches and Pain

Muscle weakness

Gastrointestinal Abnormalities

Difficulty in breathing
TREATMENT FOR MEGALOBLASTIC
ANEMIA
• VitB12 deficiency , you may need monthly injections of VitB12

• Add more food with VitB12

• Consult a doctor for medical advice


SICKLE CELL ANEMIA

A group of disorders that causes red blood cells to become


misshapen and break down.

CAUSES OF SICKLE CELL ANEMIA

• Caused by defect in a gene


A person will be born with sickle cell disease only if two genes
are inherited one from the mother and one from the father.
A person who inherits just one gene is healthy and said to be a
carrier of the disease.
PATHOGENESIS OF SICKLE CELL
ANEMIA
 Sickle cell anemia cause by a point mutation at the sixth position of the –
globin chain

 This leads to the substitution of Valine residue for a glutamic acid residue

 Tis abnormal physiochemical properties of the resulting sickle cell


hemoglobin (HbS) are responsible for the sickle cell anemia
SYMPTOMS OF SICKLE CELL ANEMIA

 Pain areas – In joints

 Pain types – Can be sudden in chest

 Whole body – Dizziness , fatigue , low oxygen in body

 Urinary – Blood in urine

 Also common – Abnormal break down of RBCs , inflamed fingers or


toes , pallor yellow skin and eyes
TREATMENT FOR SICKLE CELL
ANEMIA
 Allowing patients to get pain medication when they need it by pressing a button of
machine . Medication is the deliver through an IV

 Vitamin

 Blood transfusion

 Consult a doctor for medical advice


THALASSEMIA

A blood disorder involving lower


than normal amounts of an oxygen
carrying protein & fewer red blood
cells in the body than normal.
PATHOGENESIS OF THALASSEMIA
 Thalassemia occurs when there is a quantitative reduction of
globin chains that are usually structurally normal

 They are caused by mutations that nearly all affect the globin
locus and are extremely heterogeneous
SYMPTOMS OF THALASSEMIA

 Whole body- weakness

 Also common –pallor , shortness of breath , yellow skin and eyes ,


iron overload
TREATMENT FOR THALASSEMIA
 Vitamin – helps promote normal body function

 Iron reducer – removes excess iron from the body

 Consult a doctor for medical advice


HEREDITARY ACQUIRED ANEMIA

Hemolytic anemia can be


acquired :
Acquired hemolytic anemia happens when
parents pass the gene for the condition on to
their children
Acquired hemolytic anemia is not something
you are born with. You develop the conditions
later
PATHOGENESIS OF HEMOLYTIC
ANEMIA
A form of anemia due to abnormal breakdown of red blood cells ,
either in the blood vessels (intravascular) or
else where in the body (extravascular)

Increased red cells destruction (and increased erythropoiesis)

This can develop into anemia if:


Erythrocyte destruction accelerates beyond the compensatory capacity of the
marrow
CAUSES OF ACQUIRED HEMOLYTIC ANEMIA

Acquired hemolytic anemia is caused by factors outside the red blood cells,
such as

• Antibodies from an autoimmune disorder

• Certain infections, which may be viral or bacterial

• Medicines, such as penicillin, antimalarial medicines

• Mechanical heart valves that may damage red blood cells as they leave
the heart
SYMPTOMS OF ACQUIRED HEMOLYTIC
ANEMIA
• Yellowish skin, eyes and mouth(jaundice)

• Dark-colored urine

• Fever

• Weakness

• Dizziness

• Can’t handle physical activity

• Abnormal heart rate


Treatment for acquired hemolytic anemia

• Treatment to strengthen your immune system (using intravenous immune globin)

• Rituximab

In more sever cases, the following treatments may be needed:

• Surgery to remove spleen

• Medicine to reduce the strength of your immune system (immunosuppressive


therapy)
HAEMOPHILIA

A disorder in which blood


can’t clot properly , excessive
bleeding (external and
internal) occurs after any
injury or damage
PATHOGENESIS OF HEMOPHILIA
SYMPTOMS OF HAEMOPHILIA

 Pain areas – In the joints

 Also common – bleeding , blood in urine , internal bleeding ,


nosebleed , swollen joint
TREATMENTS FOR HAEMOPHILIA
 Clotting promoter – helps form blood clots to reduce risk of excess bleeding

 Consult a doctor for medical advice

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