HEMATOLOGICAL

DISORDERS
BY DR. SAFWAT HASSAN
 RED BLOOD CELL
Size : 7.5 micro m
Life span :120 days
Function : carry o2 around the body
NEUTROPHILS
Size : 2*RBC
Life span : days
Function : inflammation
 EOSINOPHILS
Size : 2*RBC
Lifespan :
Function : parasites
 BASOPHILS
Size : 2* RBC
Lifespan : years +
Function : allergy
 MONOCYTES
Size : largest 3*RBC
Lifespan :
Function :macrophages
 LYMPHOCYTES
Size :almost same size as RBC with large
neucls
Life span: month to years
Function : B and T cells / immunity
 PLATELETS
Size : the smallest
Life span : days
Function : heamostasis
What is Anemia
Pathophysiology of Anemia
Hemoglobinopathies

HbA
HbA

HbA2

HbF
Alfa chorm. 16
Beta chorm. 11
Gamma
Delta
 Hbopathies
Quantitative or Qualitative
Qualitative
Sickle cell anemia
Defective beta subunit.

hydroxyuria
increase HbF
 Quantitative
Alpha and Beta Thalassemia
Decrease alpha and beta production
alpha encoded by 2 genes
have 4 alleles to determine
the severity of the disease
Beta encoded by one gene
have only 2 alleles
the body produce instead delta
so a lot of HbA2 is suggestive of
Beta Thalassemia
 Why we get Anemia

P PC R

Normal P R 120 days

Abnormal P R 10-20 days

RBC damage Hemolysis

H.A
 Hemolytic Anemia
intrinsic and extrinsic causes
Intrinsic
1) Hb-opathies

sickle cell/Thalassemia
2) Membranopathies
Hereditary spherocytosis /Eleptocytosis

3) Enzymopathies: G6PD
 Extrinsic causes
1) IMHA

2) Microangiopathic HA (MAHA)

a) Thrombotic Thrombocytopenic purpura schistocytes

b) Disseminated intravascular coagulation
3) Malaria
4)Transfusion
5)Fetal RH incompatibility
 How hemolytic anemia will
present
Increase bilirubin
Splenomegaly
Reticulocytes
Positive coombs test
Spherocytosis/ eliptocytes/ schistocytes
 Different types of Anemia
Microcytic Anemia
Macrocytic Anemia

How you differentiate

MCV MCH
MIA <80 <26
Normo 80-99 fento/l 26-32
picogram/cell
MaA >100
 Microcytic Anemia
Bad Hb
Causes :
1)Iron deficiency Anemia

How we treat :
Iron
2)Anemia of chronic disease (AOCD)
Functional iron deficiency

Treat the infection

3)Thalassemia
Alfa and Beta
4)Lead poisoning
5)Myelodysplastic syndrome(sidroplastic
anemia)
How to differentiate
 Macrocytic Anemia
Bad DNA
Pathology

If not copied correctly it will keep growing and

then it will break down causes intermagalary
hemolysis so body will compensate by
erythroid hyperplasia in the bone marrow but
still same can not pass from G2 to M normally.
Megaloblastic Anemia (big immature cells)
 Causes of macrocytic
anemia
Folate deficiency (THF) tetrahydrofolate
B12 deficiency

Both are essential for DNA production (purines)

Homocystine B12 & THF methionine
THF purines (A/G)
Folate malnutrition
Treatment : oral folic acid
B12 malabsorption as in resection,
Tapeworm
intrinsic factor: pernicious Anemia
Treatment : B12 parenteral
Folic acid dhfr THF (methotrexate)
Approach to a child with Anemia
THANK YOU