Approach to anemia and hemophilia. In this presentation you will be able to learn the clinical approach to anemia and what is hemophilia , its types and management accordingly

1. APPROACH TO ANEMIA
DR ASAD ABBASI

2. Anemia is defined as reduction in the blood
hemoglobin concentration 2 standard
deviations below the mean of age and sex
OR
Anemia is defined as a reduction of the
hemoglobin concentration or red blood cell
volume below the range of values occurring in
healthy person

3. APPROACH TO ANEMIA
3basicquestion?
Isthechildisanemic?
Howseveretheanemiais?
Whatiscauseandtypeofanemia?

4. CLINICAL GRADING OF ANEMIA
Pallor restricted to conjunctiva and mucous membrane is -
mild anemia
Pale skin- moderate anemia
Pallor of palms, skin and mucus membrane-severe anemia

5. Questions to ask in history
History of blood loss from gums ,mucosa or any skin bleed.
History of blood in vomiting (hematemesis)
History of black of black tarry stool
History of per rectum bleed
History of bleed in urine
History of low grade fever with chills ,rash or night sweat
History of worms and pica
History of abdomen distension & pain

6. History of weight loss & bone pain.
History of constipation ,weight gain & cold intolerance.
History of chronic exposure of radiation.
History of drug use that causing the bone marrow
suppression.
History of yellowish discoloration of skin and eyes.
History of oliguria, anuria or polyuria
History of recurrent and episodes of pallorness
History of blood transfusion

7. NUTRITION HX :
 At which age weaning is started
 Vegetarian or non vegetarian
History of TOP feed

8. BIRTH HISTORY :
Term /preterm-------low birth weight
History of jaundice ,phototherapy ,exchange transfusion
History of exclusively breast feeding

9.  DEVELOPMENTAL HX :DELAY IN
 Fanconi anemia
 lead poising
 Gaucher disease
 Hypothyroidism
 FAMILY HISTORY :
 History of cousin marriage
 History of anemia
 History of jaundice
 History blood transfusion
 History of gall stone
 History of surgery

14. MORPHOLOGICAL CLASSIFICATION

15. LABORATORY APPROACH TO ANEMIA
Screening test :
CBC
HEMATOCRIT
RETIC COUNT
PERIPHERAL SMEAR
BLOOD INDICES (MCV, MCH,MCHC)
RDW

21. HEMOPHILIA
Most common and serious congenital coagulation factor
deficiencies.
X-linked so mostly present in males
But it can effect females too via lyonization.
Types
Hemophilia-A (factor VIII deficiency)
Hemophilia-B (factor IX deficiency)
Hemophilia-C (factor XI deficiency)

23. CLINICAL MANIFESTATIONS
Neither factor VIII nor IX cross the placenta so, bleeding may be present from birth or
may occur in fetus.
Only 2% neonate with hemophilia = ICH
30% male infants with hemophilia bleed with circumcision.
30% of cases of hemophilia have no family history.
Obvious symptoms easy bruises, IM hematomas and hemarthrosis begins when child
starts to cruise.
Bleeding from minor laceration in mouth which persist for hours to days.
The hallmark of hemophilic bleeding is hemarthrosis.
Earliest joint hemorrhages appears in most oftenly in ankle. While in older children
knee and elbows are involved mostly.
Target joint?

24. LABORATORY FINDINGS AND
DIAGNOSIS
Platelet count, bleeding time, PT and TT are normal.
APTT is prolonged, in severe hemophilia PTT is 2-3 times of upper
limits.
Specific assay for factors VIII and IX will confirm the diagnosis.
25-35% of pts receiving infusion of factors develops specific
antibodies directed against the active clotting site are termed as
inhibitors.
BETHESD assay performed to measure inhibitors antibodies titer.

25. GENITICS AND CLASSIFICATION
Hemophlia occurs in approximately 1: 5000 males, with
85% having factor VIII defiency and 10-15% having factor
IX defiency
Severity depends upon the baseline level of factors VIII
and IX.
Severe hemophilia <1% activity
Moderate hemophilia 1-5% activity
Mild hemophilia >5% activity

28. Thank you