3. HEMATOLOGICAL DISORDERS
DEFINITION:
• “Hematological disorders can be defined as
the diseases of the blood and blood-forming
tissues.”
• “A disorder marked by aberrations in structure
or function of the blood cells or the blood-
clotting mechanism.”
4. • “Blood Disorders also called as
Hematologic diseases are disorders
affect one or more parts of the blood
and prevent your blood from doing
its job.”
13. SICKLE CELL ANEMIA
• DEFINITION:
• It is a severe hemolytic anemia.
–“A severe hereditary form of anemia in
which a mutated form of haemoglobin
distorts the red blood cells into a crescent
shape at low oxygen levels.”
15. CLINICAL MANIFESTATIONS:
• Weakness,
• Pain in the large joints of the extremities in
the back and in the abdomen,
• Anorexia and vomiting and
• Fever.
• Growth in height and weight may be retarted
and sexual maturation may be delayed in
older children.
19. THALASSEMIA
DEFINITION:
• “Any of a group of inherited disorders of
hemoglobin synthesis affecting the globin
chain that are characterized usually by mild
to severe hemolytic anemia, are caused by a
series of allelic genes, and tend to occur
especially in individuals of Mediterranean,
black, or southeast Asian ancestry;
especially: Cooley’s anemia or
Mediterranean anemia”
27. HEMOPHILIA
DEFINITION:
• “It is a bleeding disorder characterized by
deficiency of factor VIII, IX and XI.”
• Hemophilia A: caused by a missing or
reduced amount of the blood clotting
protein Factor VIII
• Hemophilia B: caused by a missing or
reduced amount of the blood clotting
protein Factor IX
29. CLINICAL MANIFESTATIONS:
Severe Hemophilia
• factor VIII or factor IX clotting factor activity is less
than 1%.
• Spontaneous bleeding can occur unrelated to injury
and is most dangerous when it occurs in the head
• Joint bleeding is common
Moderate Hemophilia
• factor VIII or IX clotting factor activity is 1–5%.
• Bleeding episodes are usually related to injury
• Joint bleeding can occur
30. • Mild Hemophilia
factor VIII or IX clotting factor activity is 6–40%.
• Episodes of bleeding are usually related to surgery
or dental extractions
• Joint bleeding is uncommon
Other symptoms of hemophilia include:
• Bruising
• Soft tissue bleeding
• Bleeding from the nose, gums or mouth
• Pain, swelling and limited mobility in the joints
and muscles
• Blood found in the urine or stool, or vomiting
blood
• Excessive bleeding following circumcision
33. PURPURA
DEFINITION:
• “It is a disease of the skin and other
organs that most commonly affects
children. In the skin, the disease causes
palpable purpura (small hemorrhages);
often with joint and abdominal pain.”
34. CLINICAL MANIFESTATIONS:
• purpura, the raised, reddish purple rash
• joint pain and swelling
• stomach pain
• blood in the urine or other kidney problems
• fever
• headache
