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PAEDIATRICS > HAEMATOLOGY > Flashcards

HAEMATOLOGY Flashcards

1
Q

What are the components of Hb?

A

Fe
Globin chains - Beta, Alpha

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2
Q

Define anemia according to age

A

Neonates - <14g/dL
1 to 12M - <10g/dL
1 to 12yrs - < 11g/dL

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3
Q

Causes of Anemia

A
  1. Reduced RBC production
    BM Failure-Leukemia, Infections
    Malnutrition - Fe def, Folic acid Def, B12 def
  2. Increased destruction
    Enzyme - G6PD Def
    Membrane - Hereditary spherocytosis, Hereditary Elipsocytosis
    Hb - Thallesemia, Sickle Cell Anemia
    Antiboides - Autoimmune, ABO
  3. Blood Loss
    Parasites - Hookworm
    GIT - Meckel Diverticulum
    GUT - Menstrual bleeding
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4
Q

Raw materials of Hb Production

A

Fe
Vit B12
Folic Acid

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5
Q

What is the most common cause of Anemia in SL? Why?

A

Fe Def Anemia

Poverty - Less consumption of Fe containing food
Parasites
Tea Consumption - Tannin inhibits Fe absorption
Poor Knowledge about Fe containing food

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6
Q

Causes of Iron Deficiency Anemia (IDA)

A

Poor Intake
Malabsorption
Blood loss
Hook Worm

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7
Q

C/F of IDA

A

Pallor
Glossitis
Koilonychia - Spoon shaped nails
In Severe anemia - Breathlessness, HF features

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8
Q

Food to avoid in excess in toddlers to prevent IDA

A

Cow’s milk
Tea - Tannin inhibits Fe absorption
High fibre food - Phytates inhibit Fe aborption

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9
Q

Ix for IDA

A

FBC - Low Hb
Blood Picture - Microcytic, hypochromic (MCV, MCH) cells. Aniscocytosis (Different shapes), Poikilocytosis (Different Sizes), Red cell Distribution Width (RDW) is high
S. Feritin is low

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10
Q

Causes of microcytic anemia

A

IDA
Thalassemia
Anemia of Chronic Disease
Sideroblastic anemia

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11
Q

What vitamin increases Fe absorption?

A

Vit C

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12
Q

What are the differences between IDA and Thalassemia Minor?

A

IDA - RDW is high, S. Feritin is low
Thalassemia Minor - RDW normal, S. Ferritin is high

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13
Q

IDA Mx

A

Mx depends on the severity and the cause
If anemia is severe, with breathlessness and HF features - Blood transfusion
Otherwise blood isn’t required
If underlying cause is malnutrition - Dietary advice: Eat fish, meat, green leafy vegetables
Rx worm infections
All IDA patients must be supplemented with Fe - Ferrous sulphate - 6mg/kg/day.
Should continued until after 3 months Hb returns to normal levels.
IV Fe if patient has malabsorption

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14
Q

Efficacy of Oral and IV Fe is the same. T/F?

A

T

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15
Q

What is Thalasemia?

A

A genetic disorder where abnormal alpha and beta globin chain production occurs.

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16
Q

Percentages of children of parents with thalassemia genes

A

25% - Healthy
50% - Carriers
25% - Diseased: Thalassemia Major

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17
Q

Pathophysiology of Beta Thalassemia

A

Autosomal recessive. If both parents have the gene, the child will inherit.

Beta globulin chain production is abnormal/less.
Alpha chains will bind with Gamma to form HbF.
Alpha chains will bind with Delta to form HbA2.

Red blood cells containing abnormal Hb will be destroyed in the spleen causing anemia.

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18
Q

What is done to identify the different types of Hb?

A

Hb electrophoresis
High performance liquid chromatography (HPLC)

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19
Q

C/F of a child with Thalassemia

A

Pale
Jaundice - Due to increased bilirubin (hemolysis)
Thalassemic facies - Frontal bossing, prominent maxilla, protruding teeth.
Hepatosplenomegaly
Short stature
Poor academic performance

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20
Q

What is the reason for Thalassemic facies and hepatosplenomegaly?

A

Its due to the increased production of Hb to compensate anemia

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21
Q

Rx of Thalassemia

A

Blood Transfusion

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22
Q

Side effect of the Rx of thalassemia

A

Fe overload

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23
Q

Complications of Fe overload in Thalassemia in Rx

A

Affects various organs - Fe induced organ damage
Brain - Pitutary: Decreased GH, FSH, LH
Pancreas - Diabetes
Thyroid - Hypothyroidism
Parathyroid - Decreased Ca, tingling sensation
Skin - Pigmentation
Liver - Hepatomegaly, Cirrhosis
Heart - Cardiomyopathy
Antibodies
Infections (Not common anymore) - Hepatitis, HIV, Malaria

24
Q

Fe overload Mx

A

Fe chelation
Desferioxamine - Subcutaneous: atleast 8hrs/day, 6days/week
Deferisirox tablet - Dissolved in water

25
Q

Cure for Thalassemia

A

Bone Marrow
Preferably from siblings

26
Q

Upon inspection of a Thalassemic child, what is important to notice?

A

Insulin injection sites
Skin color in comparison to parents
Tingling sensation due to low Ca

27
Q

Ix for Thalassemia

A

FBC - low Hb
Blood picture - Microcytic, hypochromic cells. Anisocytosis, Poikilocytosis
HbLC - HbA decreased, HbF Increased, HbA2 increased
S. Ferritin increased
Sugar levels
Ca Levels
Thyroxine levels
Abdominal scans - Hepatosplenomegaly
2D Echo

28
Q

How do you calculate the amount of blood to be transfused in a Thalassemic patient?

A

(Expected Hb - Current Hb) * 4 * Body weight.

Amount of blood transfused per day - 10ml/kg

29
Q

Construct a Mx plan for a Thalassemic Patient

A

Blood transfusion
Fe chelation to prevent Fe overload
Screening of Siblings using HPLC
Advice - Dietary advice, Explanation of diseases, Clinical visits
Cure - Bone marrow (usually sibling, Available in SL, 4-5mil cost)
Social support
Economical support - 500/month from SL Government

30
Q

BM failure aka?

A

Aplastic anemia

31
Q

Causes of aplastic anemia

A

Inherited
Acquired - Viral infections (Hep, Parvovirus) Drugs (sulphanomide, chemotherapy) Toxins (Benzene)
Idiopathic

32
Q

C/F of aplastic anemia

A

Decreased rbc leading to anemia
Decreased WBC leading to infections
Decreased Platelets leading to bleeding and bruising

33
Q

Rx of Aplastic anemia

A

Depends on the cause,
Blood transfusion if child is with sever anemia.
WBC transfusion if WBC low (Buffy coat of blood)
Platelet transfusion if low
If underlying cause can be identified, treat
If due to drugs, stop drugs

34
Q

Main causes of Bleeding disorders

A
  1. Depleted clotting factors - Hemophilia.
    Joint and muscle bleeding, Increased clotting time.
  2. Platelet deficiency - Immune thrombocytic purpura.
    Mucosal bleeding - gums, nose
    Increased bleeding time
35
Q

What are the commonest severe inherited coagulation disorders?

A

Hemophilia A and B

36
Q

Inheritance patterns of Hemophilia

A

X linked recessive - Hemophilia A and B
1/3rd sporadic - Parents normal. Due to new mutations

37
Q

C/F of Hemophilia

A

Bleeding into joints and muscles, may lead to arthiritis if not Rx.
40% in neonates, particularly with intracranial hemorrhage, post-circumscion, from heel stick and venopuncture sites.

38
Q

Describe different levels of severity of Hemophilia A

A

Severe - <1% - Spontaneous Joint and muscle bleeding
Moderate - 1 - 5% - Bleeding upon mild trauma
Mild - >5 - 40% - Bleeding after surgery

39
Q

Mx of Hemophilia

A

Recombinant factor 8 for Hemophilia A
Recombinant factor 9 for Hemophilia B
Major surgery and life threatening bleeding requires levels to be raised to 100%
Intramuscular injections, Aspirin, Ibuprofen, NSAIDS must be avoided.
DDAVP/ Desmopressin may help mild Hemophilia A without blood products.
Desmopressin is ineffective in Hemophilia B.

40
Q

Hemophilia Ix

A

Clotting time increased
Bleeding time normal
aPTT increased
Factor 8 decreased - Hemophilia A
Factor 9 decreased - Hemophilia B
PT/INR Normal

41
Q

Diseases in which PT/INR increases

A

Liver failure
Vit K def
DIC

42
Q

Problem of vWD

A

Combination of platelet and clotting problems
vWF facilitates platelet adhesion - Deficiency leads to defective platelet plug formation
Its the carrier protein of factor 8

43
Q

Cause of vWD

A

Quantitative or qualitative deficiency of factor 8

44
Q

C/F of vWD

A

Bleeding from mucosa - gum, epistaxis
Bleeding from joints and muscles

45
Q

Ix of vWD

A

Bleeding time, clotting time and aPTT increased

46
Q

Mx of vWD

A

Desmopressin - Mild
Factor 8 Concentrate - Severe disease

47
Q

Inheritance pattern of vWD

A

AD

48
Q

Commonest subtype of vWD

A

Type 1
Fairly mild
Not diagnosed until puberty

49
Q

What is the commonest cause of thrombocytopenia in children?

A

Immune thrombocytopenia

50
Q

Cause of ITP

A

Immune mediated destruction of platelets. Megakaryocytes in BM
Onset after 1 to 2 weeks of a viral infection

51
Q

C/F of ITP

A

Petechiae and purpura (Bleeding under the skin)
Superficial bleeding
Epistaxis, mucosal bleeding
Platelet counts falls to <10 * 10^9/L

52
Q

D/d for thrombocytopenia

A

Dengi
ITP
Autoimmune
Malignancies
BM Failure

53
Q

Complication of ITP

A

Intracranial hemorrhage

54
Q

ITP Dx

A

Dx of exclusion
If hepatosplenomegaly, marked lymphadenopathy present, BM biopsy to exclude acute leukemia or aplastic anemia.
A bone marrow examination must be done is the child is treated with steroids
SLE must be excluded

55
Q

ITP Mx

A

Supportive/symptomatic Mx
Steroids
Immunoglobulin
Splenectomy

If platelet count, >20,000 - Symptomatic Mx, advice

<20,000 + no bleeding - Prednisone
<20,000 + high risk of bleeding ( past hx of bleeding) or child doesn’t heed advice - IV Ig

56
Q

Is there a place for platelet transfusion in ITP? Why?

A

No.
How much platelets given, it will be destroyed by antibodies.

PAEDIATRICS (15 decks)

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