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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
Cure for Thalassemia
Bone Marrow Preferably from siblings
26
Upon inspection of a Thalassemic child, what is important to notice?
Insulin injection sites Skin color in comparison to parents Tingling sensation due to low Ca
27
Ix for Thalassemia
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
How do you calculate the amount of blood to be transfused in a Thalassemic patient?
(Expected Hb - Current Hb) * 4 * Body weight. Amount of blood transfused per day - 10ml/kg
29
Construct a Mx plan for a Thalassemic Patient
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
BM failure aka?
Aplastic anemia
31
Causes of aplastic anemia
Inherited Acquired - Viral infections (Hep, Parvovirus) Drugs (sulphanomide, chemotherapy) Toxins (Benzene) Idiopathic
32
C/F of aplastic anemia
Decreased rbc leading to anemia Decreased WBC leading to infections Decreased Platelets leading to bleeding and bruising
33
Rx of Aplastic anemia
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
Main causes of Bleeding disorders
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
What are the commonest severe inherited coagulation disorders?
Hemophilia A and B
36
Inheritance patterns of Hemophilia
X linked recessive - Hemophilia A and B 1/3rd sporadic - Parents normal. Due to new mutations
37
C/F of Hemophilia
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
Describe different levels of severity of Hemophilia A
Severe - <1% - Spontaneous Joint and muscle bleeding Moderate - 1 - 5% - Bleeding upon mild trauma Mild - >5 - 40% - Bleeding after surgery
39
Mx of Hemophilia
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
Hemophilia Ix
Clotting time increased Bleeding time normal aPTT increased Factor 8 decreased - Hemophilia A Factor 9 decreased - Hemophilia B PT/INR Normal
41
Diseases in which PT/INR increases
Liver failure Vit K def DIC
42
Problem of vWD
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
Cause of vWD
Quantitative or qualitative deficiency of factor 8
44
C/F of vWD
Bleeding from mucosa - gum, epistaxis Bleeding from joints and muscles
45
Ix of vWD
Bleeding time, clotting time and aPTT increased
46
Mx of vWD
Desmopressin - Mild Factor 8 Concentrate - Severe disease
47
Inheritance pattern of vWD
AD
48
Commonest subtype of vWD
Type 1 Fairly mild Not diagnosed until puberty
49
What is the commonest cause of thrombocytopenia in children?
Immune thrombocytopenia
50
Cause of ITP
Immune mediated destruction of platelets. Megakaryocytes in BM Onset after 1 to 2 weeks of a viral infection
51
C/F of ITP
Petechiae and purpura (Bleeding under the skin) Superficial bleeding Epistaxis, mucosal bleeding Platelet counts falls to <10 * 10^9/L
52
D/d for thrombocytopenia
Dengi ITP Autoimmune Malignancies BM Failure
53
Complication of ITP
Intracranial hemorrhage
54
ITP Dx
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
ITP Mx
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
Is there a place for platelet transfusion in ITP? Why?
No. How much platelets given, it will be destroyed by antibodies.

PAEDIATRICS (15 decks)

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