Hematology

Question 1

Features of ALL

Answer 1

Pallor, lethargy, bruising, Petechiae, nose bleed, infection, bone pain, malaise, anorexia, hepatosplenomegaly, lymphadenopathy, fever.

Relapse: CNS like headache, vomiting, nerve palsies, testicular enlargement, skin nodules

Question 2

Tests for ALL

Answer 2

FBC, PBF (blast cells), BM exam for aspirate, cytogenetics, molecular studies, LP for blast cells, CXR for mediastinal masses

Question 3

Management of ALL

Answer 3

Correct anemia, transfuse platelets and treat any infection
Hydrate + allopurinol to protect renal function against rapid cell lysis
2-3 months of combination chemo

Question 4

How to manage HSP

Answer 4

Admit, monitor vital signs and input/output
Monitor renal function
Analgesia of joint pain
Oral pred for acute abd pain
Upon discharge, set follow up for urine MC&S, BP, renal function

Question 5

Features of HSP

Answer 5

Rash - urticarial then maculopapular, purpuric, takes weeks to recover
joint pain, joint swelling
colicky abdominal pain, hematemesis, melena
Hematuria, mild proteinuria
Diffuse alveolar hemorrhage
Change in mental status
Focal neuro signs

Question 6

Tests for thalassemia

Answer 6

FBC, PBF, reticulocytes, iron studies, electrophoresis, X-Ray

Question 7

Managing beta thalassemia major

Answer 7

Regular blood transfusions 3-4 weekly
Desferrioxamine over 10 hours nightly
Splenectomy
Cure: stem cell transplant from BM, cord blood, peripheries

Question 8

How to manage HbH and beta thalassemia minor

Answer 8

Give folic acid supplement

Question 9

Tests and management for Hodgkin Lymphoma’s

Answer 9

LN biopsy, radiological assessment of nodal sites and bone marrow biopsy

Combination chemo with or without radio, PET scan to monitor treatment response, 80% can be cured

Question 10

Tests and management for non-Hodgkin’s Lymphoma

Answer 10

Biopsy, CT/MRI of all nodal sites, BM exam and CSF

Multi-agent chemo, survival rate > 80%

Question 11

IDA management

Answer 11

FBC, ferritin, PBF
Dietary advice:
- limit cow’s milk, avoid tea, avoid high-fiber food
- eat red meat, liver, kidney, oily fish
- eat food containing vit c
Oral iron ferrous sulphate
- 3-6 mg/kg/d
- continue for 3 months after Hb return to normal
- can cause black stool and constipation
Blood transfusion
- only if severe anemia and urgent surgery/HF
- done slowly, keep hb at 6-8 g/dk
follow-up
- reticulocyte response and ferritin at 4 weeks

Question 12

ITP investigations

Answer 12

FBC, PBF, normal PT/FTT

Bone marrow exam if treating with steroids cause can mask ALL

Question 13

Management of ITP

Answer 13

normally benign and self-limiting within 6-8 weeks
Low risk (no active bleed, painless oral petechiae)
- no mx
- repeat FBC and review in a week
- educate family
If diagnosis uncertain/PBF not ready/poor access to hospital/worsening, admit
- if active bleed, oral pred 2mg/kg/d for 2 weeks
- normal human IvIg
- platelet transfusion if life-threatening hemorrhage

Question 14

Family education for ITP

Answer 14

Avoid contact sports/any activities with risk of trauma
Avoid anti-platelet, anti-coag, NSAIDS, IM injection
Monitor for bleeding
Monitor for signs of ICH and go immediately to ED if head injury/severe headache

Question 15

Hemophilia tests

Answer 15

Prolonged APTT, normal PT.
Reduced factor VIII/IX
Normal platelet count and bleeding time

Question 16

Management of hemophilia

Answer 16

Whenever bleed, give Recombinant factor concentrate by prompt IV infusion
- raise level to 30% of normal for minor bleed and simple joint bleed
Major surgery
- raise to 100%, then maintain at 30-50% up to 2 weeks to prevent secondary hemorrhage
- continuous infusion
- or 8-12 hourly for 8, 12-24 hourly for 9
Avoid IM, aspirin, NSAIDS
Home management encouraged to avoid delay in treatment
Severe patients:
- prophylactic factor starting age 2-3
Desmopressin for mild hemophilia A
- for minor surgery and extraction