Anemia

Question 1

Physiologic Anemia

Answer 1

• The most common cause of anemia in
young infants. • Between six to nine weeks of age. • It is due to decreased erythropoiesis
(Due to improved tissues oxygenation
in comparison with fetal life) and
shorter RBCs life span.

Question 2

Risk Factors

Of IDA

Answer 2

• Exclusively breast-fed infants who
continue on exclusive breast feeding
after 6 months of life. • Prematurity. • Infants fed cow’s milk when younger
than 1 year of age. • Toddlers fed large volumes of cow’s
milk. • Menstruating adolescent females who
are not receiving supplemental iron.

Question 3

Cl inical Associations of IDA

Answer 3

In addition to anemia symptoms:
• Poor muscle strength, gastrointestinal
dysfunction, and impaired WBC and T-
cell function
• It is associated with later cognitive
deficits and poor school performance.

Question 4

Diagnosis of IDA

Answer 4

• Microcytic, hypochromic anemia (low
MCV and MCH). • High RDW. • Low serum ferritin. • If the history is consistent with IDA,
CBC usually sufficient without the need
for the iron profile.

Question 5

Management of IDA

Answer 5

Dietary advice (Decrease cow’s milk
intake, increase intake of iron-
containing items) • Elemental iron for at least 3 months
with follow up blood test. • The usual therapeutic dose of
elemental iron induces an increase in
hemoglobin of 0.25-0.4 g/dL per day
• If the Hemoglobin failed to increase,
careful re-evaluation for ongoing blood
loss, development of infection, poor
compliance, malabsorption, or other
causes of microcytic anemia is
required.

Question 6

Causes of Red Cell Aplasia

Answer 6

• Congenital red cell aplasia (Diamond–
Blackfan anemia). • Transient erythroblastopenia of
childhood. • Parvovirus B19 infection (in children
with inherited hemolytic anemias).

Question 7

Clues for Red Cells Aplasia

Answer 7

• Low reticulocyte count despite low Hb • Normal bilirubin • Negative direct antiglobulin test
(Coombs test) • Absent red cell precursors on bone
marrow examination.

Question 8

Diamond– Blackfan anemia

Answer 8

• Genetic disease (Autosomal
Dominant).
• 80% sporadic.
• Presents with anemia between 2-3
months of age.
• Associated with short stature,
abnormal thumbs.
• Treated with steroid but if does not
respond may require regular Blood
transfusions.
• BMT can cause cure.

Question 9

Fanconi Anemia

Answer 9

This is the most common inherited
form of aplastic anemia (Bone Marrow
Failure).
• Autosomal Recessive.
• Associated with congenital
abnormalities: short stature, abnormal
radii and thumbs, renal malformations,
microphthalmia, and pigmented skin
lesions.
• Hematological manifestations appear
after 5 years of age. (pancytopenia) • Increased risk of malignancies due to
DNA repair issues.

Question 10

Autoimmune hemolytic anemia

Answer 10

• Acute, self limiting condition.
• Caused by production of Antibodies that
cause RBCs production.
• Can follow certain infections (EBV,
Mycoplasm, URTI) or associated with other
autoimmune diseases or drugs (penicillin). • Presents with symptoms of anemia.
• Investigations: Coombs positive, incresed
reticulocyte count and indirect biliriubin. • 80% resolve spontaneously.
• Treatment: Transfusion not always
effective (Why?), steroid in severe cases
only.

Question 11

SCD

Answer 11

ماعندهم بيتا فماعندهم HB A

Question 12

Sickle Cell Anemia

Answer 12

Dactylitis can be the presenting sign in

infants.

Question 13

Sickle Cell Anemia CP

Answer 13

Si ckle Cell Anemia
Dactylitis can be the presenting sign in
infants.
Main clinical features are: - Anemia, - Infection, - Painful vaso-occlusive crises, - Sequestration crises, - Splenomegaly in some young children, - Growth failure, - Behavior and learning problems.

Question 14

Types of crises: SCA

Answer 14

1- Vaso-occlusive crisis: - Intermittent episodes of acute pain. 2- Splenic Sequestration crisis: - Sudden splenomegaly and pallor, BP and Hb drop. 3- Hyperhemolytic crisis:
- Sudden increase in hemolysis with worsening anemia. 4- Aplastic crisis. (Caused by Parvovirus B19).

Question 15

The most serious clinical complications SCA

Answer 15

The most serious clinical complications
are bacterial infection, acute chest
syndrome, strokes and priapism
(persistent, usually painful erection).

Question 16

Management of SCA

Answer 16

• Prophylactic penicillin. • Immunization against encapsulated
bacteria. • Folic acid (Why?). • Maintain good hydration. • Treat crises. • Long-term: hydroxyurea or occasionally
bone marrow transplant

Question 17

B -Thalassaemia major

Answer 17

Splenomegaly and hepatomegaly

Question 18

Hemostasis

Answer 18

• Prothrombin time (PT) – measures the
activity of factors 2, 5, 7 and 10. • Activated partial thromboplastin time
(APTT) – measures the activity of
factors 2, 5, 8, 9, 10, 11 and 12.

Question 19

History of bleeding

Answer 19

Age of onset • Site of bleeding: skin (petechiae,
bruising) mucus membrane (epistaxis,
GIT), deep tissue (joints, muscles) • Always check for other bleeding site • Skin and mucus membrane bleeding
may suggest platelets disorders or in
their interaction with blood vessels. • Soft tissue, muscle, and joints bleeding
suggest coagulation factors’ disorders.

‏• Did the symptoms correlate with the
‏degree of injury or trauma? 
• In Acute cases: - History of recent URTI - History of constitutional symptoms (fever, weight loss, decrease appetite)
• Child abuse should be considered in
unexplained recurrent bleeding.

‏Family history: Bleeding history only in
‏male siblings and maternal uncles is
‏suggestive of X-linked recessive
‏diseases, such as hemophilia A or B. 
Drug history (herbal medicine, warfarin
poisoning, NSAIDs).

Question 20

Diagnostic Testing in bleeding

Answer 20

Diagnostic Testing
• CBC including platelet count. • Peripheral Blood smear. • Prothrombin time (PT). • Activated partial thromboplastin time
(APTT). • Other lab workups depend on the case
history.

Question 21

‏Hemophilia

Answer 21

• Hemophilia A – Factor 8 deficiency. • Hemophilia B- Factor 9 deficiency. • X-Linked recessive. • Hemophilia A is more common.

Question 22

Bleeding Sites in hemophilia

Answer 22

• Hemarthrosis (ie, hemorrhage into a
joint) is the most common site for
bleeding in ambulatory patients,
representing up to 80 % of
hemorrhages. • Spontaneous Hemoarthrosis is
characteristic of severe disease.

Bleeding into muscles with hematoma
formation is common. • In severe cases may cause
compartment syndrome.

Hematuria is a frequent manifestation
of severe hemophilia.

Question 23

‏ Ma nagement of hemophilia

Answer 23

‏Ma nagement
‏• Replacement therapy for acute
‏bleeding is the hallmark of hemophilia
‏management. • 
• The desired level of factor’s activity
depends on the bleeding severity.

‏In severe hemophilia, prophylactic
‏replacement therapy is given on
‏regular basis to prevent chronic
‏arthropathy.

Inhibitors are IgG against infused
factors ( 8>9). It occurs in up to 15% of
patients with severe factor 8
deficiency.
العلاج / frequent steroid 
Desmopressin (DDAVP) can be used
instead of replacement therapy in mild
to moderate hemophilia A (Not
Hemophilia B). • It is given by infusion and stimulates
endogenous release of FVIII:C and vWF.

Question 24

von Willebrand Disease

Answer 24

von Willebrand Disease
• Usually Autosomal Dominant. • 1% of population • Caused by deficiency in von Willebrand
factor (vWF). • vWF function: ü Facilitates platelet adhesion. üActs as Factor 8 carrier protein. • vWF deficiency can be quantitative or
qualitative.
Ma nagement
• DDAVP Causes secretion of factor 8 and vWF into plasma. • Plasma-Derived Factor 8 in severe
cases. • Avoidance of anticoagulants, NSAIDs
and IM injections.
Hemorrhagic disease of the newborn
Due to vitamin K deficiency which is
essential for coagulation factors
production. • Risk is increased in breast fed infants. • 1-8 weeks of age. • Bleeding can vary between mild to
severe. • All newborns should receive Vitamin K
at birth.

Question 25

Immune Throbocytopenic Purpura (ITP)

Answer 25

Immu ne Throbocytopenic Purpura (ITP)
• The most common cause of
thrombocytopenia in children. • It is an autoimmune disease caused by
autoantibodies against platelets.
• 2-10 years of age. • The bleeding is usually preceded by a
viral infection. • Petechiae, purpura, or superficial
bruising. • Can cause mucus membrane bleeding
such as epistaxis. • Intracranial hemorrhage is uncommon
despite the lower platelets.
ITP is a diagnosis of exclusion. • Any atypical features such as anemia,
leukocytopenia, hepatosplenomegaly
or marked lymphadenopathy should
raise the suspicion for possible
malignant process.
• CBC: Low platelets, normal RBC and
WBC counts. • Bone morrow examination is not
necessary unless there is atypical
history, physical examination or initial
workup. • BM (if done) reveals increased
megakaryocytes and normal erythroid
and myeloid elements.

For children with no active bleeding: - Observation (First line treatment) - Oral steroid - Intravenous Immunoglobulin (IVIG) • For children with moderate bleeding: - IVIG or short course of corticosteroid • For children with life-threatening
bleeding: - Intravenous steroids and IVIG, may consider platelet transfusion

Chronic ITP
ITP that persists for 12 months is
classified as chronic ITP. • Secondary causes of chronic ITP,
especially SLE and HIV infection, should
be ruled out.

Question 26

Wiskott-Aldrich syndrome

Answer 26

Wiskott-Aldrich syndrome
• X-linked recessive. • Triad of immune deficency, eczema,
and thrombocytopenia. • Hematopoietic stem cell
transplantation cures the
immunodeficiency and
thrombocytopenia.