Symptoms and treatment

Question 1

1/3 makes have jaundice

Majority are asymptomatic

Neonatal hyperbolirubinea

Acute/chronic HA

Answer 1

G6PD

Question 2

Remove triggers to remove HA by decreasing ROS Chance

Remove fava beans

Remove medication (dapsone)

Answer 2

G6PD

Question 3

What drug is increases ROS Chance

Answer 3

Dapsone

Question 4

Jaundice

Splenegamoly

Extramedary hematopoiesis

Hepatosplengamoly

Answer 4

Autoimmune HA

Question 5

Prednisone

Corticosteroids

Cytotoxic immunosuppresive drugs

Cyclosporine

Splenectomy if corticosteroids dont work 3months

Monoclonal AB

Answer 5

Autoimmune HA

Question 6

Monoclonal AB treatment for aHA

Answer 6

Campath 1H

Question 7

Why does harmolysis occur in RBC in HbH disease

Answer 7

Excess of B globin /homotetramer (HbH)

Recognised by spleen and destroyed

Extravascular

Question 8

Hepatosplenomegaly

Ineffective haemopoirsis

Answer 8

HbH

Question 9

Treatment generally not required

Antibiotics

Regular folic acid if preganant

Splenectomy

BT

Answer 9

HbH

Question 10

Vado-occlusive

Severe bone/joint pain

Acute chest syndrome

Dactylitis (children)

Jaundice

Gall stones

Splenegomaly

Answer 10

Sickle cell

Question 11

Life long prophylactic penicillin with folate replacement

Avoid triggers if trait (altitude.stress)

Blood transfusions +iron chelation

HSCT

Hydroxurea

Mumurs / hamaturia/renal tubular defects /strokes

Answer 11

Sickle cell

Question 12

What does hydroxurea do

What’s it used in

Answer 12

Increase HbF

Sickle cell

Question 13

What bacterial infection is common in sickle cell patients

Answer 13

Staphylococcus aureus

Question 14

Avascular Necrosis if head of femur / growth retardation

Answer 14

Sickle cell

Question 15

Parlour of mucuos membrane

Conjunctival mucosa

Brittle nails

Ulceration around mouth

Glottitis

Jaundice

Answer 15

IDA

Question 16

Ferrous sulphate

Original cause dealt with

Answer 16

IDA

Question 17

How much ferrous sulphate given to IDA patient

Answer 17

100-200mg daily

Rise of 2g/dl for 3wks

Question 18

Sclera of rye

Glossitis

Jaundice

Answer 18

Megaloblastic anaemia

Question 19

6x1000ug 2/3wks

1000ug/3months maintenance dose

Answer 19

B12

Question 20

How long does it take to reach normal haemoglobin levels with treatment of b12

Answer 20

60 days

Question 21

5mg daily 4months of b12

Answer 21

Folate deficiency

Question 22

Pallor /jaundice /splenomegaly /family history

Answer 22

Herdiatory spherocytosis

Question 23

Splenectomy

Partial cholecystectomy for fall stones

Phototherapy for neonatal jaundice

Answer 23

Herdatory spherocytosis

Question 24

Erythroid hyperplasia anaemia

Cholelithiasis (gall stones)

Iron overload

Bacterial infection

Helatosplenomegaly

Recurrent leg ulcers

Answer 24

B-thala major

Question 25

Why does iron overload exist for b thala

Answer 25

Increased absorption Multiple blood transfusions

Question 26

Blood transfusions Infections dealt with(ab) Splenectomy HSCT Long term folic Acid + gene therapy

Answer 26

B major thala

Question 27

What infections are b thala prone to

Answer 27

Klebsiella

Question 28

Why does increase bone marrow occur in b thala

Answer 28

Increased EPO release