Dr. Sakeena NourEldine

Consultant in Paediatrics

Definition:

- Areduction of the red blood cell or hemoglobin

concentration below

the range of values occuring

The Anaemias

.The leuel of Hb differ according to

* Age

* Race . black have levels about o.s gm lower than white

and Aslan

* Ethnic group.

Physiological adjustment to anemia includes:

• Increased cardiac output

• Increased A.V oxygen differences

• shunt of blood flow toward vital organ and

tissues

• Increase conc. Of 2,3 –DPG within the RBCs• Increase conc. Of 2,3 –DPG within the RBCs

• shift to the right of the oxygen dissociation curve

- in moderate anemia few symptoms and findings

may be evident

- in severe anemia tachypnoea tachycardia and

congestive heart failure occur

COMMON CAUSES OF ANEMIACOMMON CAUSES OF ANEMIA

Hypochromic microcytic anemia :

Cause

1. iron deficiency anemia

2. Thalassemia

3. chronic inflammatory disease 3. chronic inflammatory disease

4. Copper defficiency

5. Sideroblastic anemia

6. Hereditary

.NORMOCHROMIC NORMOCYTIC

1.Chronic inflammatory disease.

2.Recent blood loss

3.Malignancy and marrow infiltration

4.Marrow aplasia or hypoplasia

5.Chronic renal failure5.Chronic renal failure

6.H I V

7.Hemophagocytic syndrome

Macrocytic Anemia:

Cause:

1. Vitamin B12 deficiency

2. Folate deficiency

3. Hypothyroidism

4. Marrow failure 4. Marrow failure

Hemolytic Anemia:

Cause:

1. Hemoglobinopathy eg(Hbs)

2. Enzymatic deficiency eg(G6PD deficiency)

3. Cell membrane defect eg(heriditary

spherocytosis)

4. Extrinsic factor eg(DIC,HUS)4. Extrinsic factor eg(DIC,HUS)

5. Immune hemolytic anemia eg(Autoimmune

deficiency,Drug induced )

IRON DIFICIENCY

ANEMIA

-It is the common hematologic diseae -It is the common hematologic diseae

of infancy and childhood

-30 percent of the globalpopulation

suffers from it mostof them in the

developing countries

Etiology

• inadequate dietary intake

• .blood loss

• Occlut bleeding in different sites

• -peptic ulcer • -peptic ulcer • -mechel diverticulum

• -polyps

• haemangioma

Pulmonary haemosiderosis

• Hook worm infestation

Clinical manifestations:

• Pallo,r irritability.

• If hemoglobin falls below 5gm

tachycardia and cardiac dilatation

occur as well as systolic murmur. occur as well as systolic murmur.

• Iron deficiency have effects on

neurologic and intellectual function. It

affects attention, alertness and

learning.

Laboratories findings:

• Serum ferritin and serum iron.

• Iron stores, bone marrow hemosiderin

• Iron binding capacity "transferrin" .

• Later the RBCs become smaller and the • Later the RBCs become smaller and the hemoglobin content deceases-- Microcytic, hypochromic.

• MCV and MC H decreases.

• Thrombocytosis.

• Hyper cellular marrow with Erythroid hyperplasia.

.

TREATMENT:

• Iron salts supplementation in ferrous forms ,eg

• fumarate sulphate or gluconate.

• Dose : 4-6 m g / kg / day

• Parenteral iron rarely used, eg in malabsorption

• Iron rich food

• Parents education

• Iron medication should be continued for 8 wks after

• blood values returned to normal.

• Blood transfusion is rarely indicated.

HEMOLYTIC ANEMIAS

• Definition:

• Hemolysis is the premature destruction of

red blood cells.

• Anemia results when the rate of • Anemia results when the rate of

destruction exceeds the capacity of the

marrow to produce RBCs

• The normal RBC survival time is 110-120

days

classification of hemolytic anemia

1) cellular :

resulting from intrinsic

abnormalities of the membrane ,

Enzymes or hemoglobin Enzymes or hemoglobin

2) Extracellular :

resulting from antibodies ,

mechanical factors or plasma factors .

.

SICKLE CELL ANEMIA• Hemoglobin S results from a change which

encodes valine instead of glutamine in the 6+h

position in the beta globin molecule

• Sickle cell anemia occurs when both beta globins

genes have the sickle cell mutation.

• Sickle cell disease accrues when one betaglobin • Sickle cell disease accrues when one betaglobin

gene have the sickle cell mutation and the other

carry gene mutation other than sickle cell

mutations.

• In sickle cell anemia Hbs is commonly as high as

90% of the total hemoglobin where as in sickle cell

disease Hbs is < 50%

• Inheritance is Autosomal recessive

Clinical Manifestations and treatment

• children with sickle cell anemia have abnormal immune function due to functional asplenia or autosplenectomy

• Bacterial sepsis is one of the greatest • Bacterial sepsis is one of the greatest causes of morbidity and mortality .

• Children with sickle cell anemia have deficient levels of serum opsonin of the alternate complement pathway against pneumococci. (encapculated)

• Children with sickle cell anemia should receive prophylactic oral penicillin V.

• Acute infection with parvovirus B19 is usually associated with red cell is usually associated with red cell oplasia – aplastic crises – fever, .pain , splenic sequestration and ACS

Dactylitis

• often refered to as hand – foot

syndrome

• It is the first manifestation of pain • It is the first manifestation of pain

in children with sickle cell anemia

• Occur in 50% of children by 2

years of age and is often

symmetrical

Acute splenic sequestration

• It is a life threatening complication .

• Approximately in 30 % of pts .

• The etiology is unknown

• Clinically it is associated with an engorged,

enlarged spleen , evidence of hyopvolemia

and reduction of hemoglobin

• Could be associated with URTI or viral

infection

• Treatment require early intervention and

maintenance of homodynamic stability by

either isotonic fluid or blood transfusion .

• Repeated episodes of splenic • Repeated episodes of splenic

sequestration are comon .

• Prophylactic splenectomy is the only

elective strategy for prevention of future

life threatening episodes

Vaso – occlusive episode

the pain is characterized as unremitting

discomfort that may occur any where but

most often in the chest , Abdomen and

extremities

The pathogenesis of pain /disruption of The pathogenesis of pain /disruption of

blood flow in the micro vessels by sickle

cells Resulting in tissue ischemia .

Precipitating factors include physical

stress , infection , dehydration , hypoxia ,

acidosis and exposure to cold.

Treatment or painful episodes requires

1) education of the parents and the patient about

symptoms and management strategy .

2) acetaminophen or anon steroidal agent early in the

course of pain .

3)hospital admission with iv administration of morphine

or morphine derivatives

4) Iv hydration is appropriate for children with

dehydration or is unable to drink as a \result of pain

5) Hydroxyurea a myelosuppresive agent which

reduces the frequency of painful episodes It acts

by raising the level or Hb F and the hemoglobin level

Priapism

• It is an involuntary penile erection lasting longer than 30 min and it is painful .

• It may persist for hours

• Treatment : • Treatment :

1. Acute treatment – supportive initially but if it persist for 4 hours urology consultation should be done for further treatment.

2. Preventive therapy-hydroxyurea has promising results .

Neurologic

• Presentation of stroke may occur as young as 1

year of age .

• stroke may run in particular families .

• other neurologic complications include

headaches, seizures , cerebral venous

thrombosis .thrombosis .

• Treatment of stroke includes :

• oxygen administration to maintain O2 sat more

than 96% .

• Blood transfusion as quickly as possible

exchange transfusion to reduce Hbs to < 50%

prevention of stroke by;

• Regular blood transfusion after the

stroke to decrease Hbs level .

• primary prevention can he • primary prevention can he

accomplished by transcranial

Doppler assessment or the blood

velocity

Acute chest syndrome

• characterized by fever, respiratory distress , chest pain and a new radio density on chest radiograph.

• Early detection of ACS will alter the • Early detection of ACS will alter the clinical management.

• Other pulmonary complications include pneumonia and bronchiolitis

Treatment of acute chest synome includes:

–Oxygen administration.

–Simple or exchange transfusion.

–Admission to the ICU.

–Antibiotics – Macrolide and a third

generation cephalosporine.generation cephalosporine.

Sickle cell nephropathy include gross

• hematuria.

• Papillary necrosis.

• Nephrotic syndrome.

• Pyelonephritis.

Other complications

• Sickle cell retinopathy.

• Delayed onset of puberly. • Delayed onset of puberly.

• Avascular necrosis of the

femoral head, and humerus.

• Leg ulcer.

Diagnosis of sickle cell disease

• Complete blood count.

• Hemoglobin analysis –

Electrophoresis.

• Newborn screening programs. • Newborn screening programs.

• Other sickle cell syndrome:

• Hb Sc

• Hbs beta thalassemia.

• Hb So.

Sickle cell trait

• The amount of Hbs in individuals

with sickle cell trait is less than

50% and Hb A is more than 50%.

• Life span is normal and serious • Life span is normal and serious

complications are very rare.

• Complications include splenic

infarcts at high alttitude .

• Hematuria, hyposthenuria

Thalassemia syndromes

• These are genetic disorders in globins chain

production.

• Beta thalassemia is either:

– beta0 thanlsemia due to complete absence of beta globins

gene production.

– beta+ thalassemia due to partial reduction. – beta+ thalassemia due to partial reduction.

• Alpha thalassemia is also due to cither absent or

partially reduced alpha – glbobin gene production.

• In thalassaeima the primary pathology is the quantity

of globins gene production where as in sickle cell

disease the pathology is the quality of globins

production.

Homozygous beta-thalassemia:

• Called also thalasssemia major or colley

anemia.

• Usually become symptomatic in the 2nd

half of the first year.

• The classic findings includes maxillary • The classic findings includes maxillary

hyperplasia, flat hasal bridge and frontal

bossing – thalassemic facies .

• Pathologic bone fiactuces.

• Marked hpatosplenomegaly and cachexia.

• Secondary hypersplenism.

• Features of ineffective erythropoiesis. Eg

expanded medullary spaces.

• Pallor, hemosiderosis – jaundice.

• Symptoms due to excessive iron stores: • Symptoms due to excessive iron stores:

– Endocroine dysfunction includes hypothyroidism,

gonadal failure, hypoparathyroidism and DM.

– Cardiac dysfunction include congestive heart failure

– cardiac arrhythmia.

Laboratory findings

• CBC showed severe anemia Hb 5gm

reticulocyte count 8%.

• Chematy: showed increased

unconjugated bilirubin unconjugated bilirubin

• Hemoglobin electropholesis.

• Treatment:

• Confirm the diagnosis.

• councelling.

• Regular blood treastnsion morthly to

keep the hemoglobin > 9.5 < 10.5

• Iron chelating agent to avoid

transfusional hemosiderosis. E.g

deferoxamine subcutaneous infusion.

• Bone marrow thransplantation can • Bone marrow thransplantation can

give cure if an HLA matched sibling is

available.

Alpha Thalassemia• Infants are identified in the newborn period by the

increased production of barts hemoglobin (gama

4) during fetal life and its presence at birth.

• Occur most frequently in southeast Asia.

• The hemoglobin analysis is normal except during

the newborn period when Hb bart's is found < 8% the newborn period when Hb bart's is found < 8%

• The deletion of 1 alpha globin gene is not

identifiable hematological.

• The deletion of 2 globin genes lesnlts in alpha

thalassemia trait with considered as having iron

diffiency anemia – 10 w Mcv and Mc

• The deletion of 3 alpha globin genes give adiagnosis of Hb H disease

• The deletion of all alpha globins genes causes profound anemia during fetal life resulting in hydrops fetalis

• Children with alpha th major are transfusion dependant and bone marrow transfusion dependant and bone marrow transplant is the only cure.

• Treatment includes.

–Folic acid supplementation

–Possible splenectomy

–Chromic transfusion therapy

–Bone marrow transplantation

•THANK YOU

•AND HAVE ANICE

•DAY