haematology interactive cases

Question 1

describe this blood film

Answer 1

Anaemic – space between the blood cells
No plts
Abnormal cells – blast cells

Question 2

Answer 2

Acute lymphoblastic leukaemia
v high WCC in child - highly likely it is leukaemia
low Hb dn plts - suggest bone marrow failure

mediastinal mass is thymus - infiltrated by T lymphocytes

Question 3

best technique to dx acute lymphoblastic leukaemia

Answer 3

immunophenotyping

Question 4

treatment for ALL

Answer 4

need combination chemo and CNS directed therapy

ALL Can cross the BBB
Many drugs don’t cross BBB – so either have to choose drugs that can – or inject into the brain

a high WCC lessens chance of cure

Question 5

48-year-old male – railway engineer
2-week history bleeding gums
Attended dentist - severe bleeding
1 episode of haematuria
Minor bruising
Attended Accident and Emergency department
Left subconjunctival haemorrhage
Small bruises over abdomen
No enlarged lymph nodes
No hepatosplenomegaly

What test is most likely to reveal the cause of the problem?
* Liver function tests
* Creatinine
* Coagulation screen
* Blood count, film and coagulation screen

Answer 5

Blood count, film and coagulation screen

Want to know if thrombocytopenic
Need film to make sure not pseudothrombocytopenia/false counting on blood count

Question 6

How could you explain a SHORT APTT and a low fibrinogen?

Answer 6

DIC

consumption of fibrinogen
Short APTT is affecting activation of coagulation

Question 7

test to help dx of DIC

Answer 7

D-dimer

Question 8

Do you think these are myeloid cells or lymphoid?
How would you prove it?

Answer 8

Big compared to red cells
Have Auer rods – so are myeloid cells

Cytochemistry
Immunophenotyping
But note that neither test is actually necessary because can see auer rods

Question 9

describe this bone marrow

Answer 9

Brightly stained granules – uniformal population – obliterated the normal marrow cells/.

Question 10

difference between CML and acute promyelocytic leukaemia

Answer 10

bopth have myeloid hyperplasia
but CML would have more differentiated cells

Question 11

test to confirm acute promyelocytic leukaemia

Answer 11

Cytogenetic analysis/FISH/molecular genetic analysis

Want to be sure have the fusion gene

Immunophenytyping can be done quickly – but still not definitive

FISH is fast while waiting for cyto and molecular genetics - PML-RARA fusion

Question 12

treatment for acute promyelocytic leukaemia

Answer 12

with platelets, chemotherapy and all-trans-retinoic acid (ATRA)

good px

Question 13

A 68-year-old retired secretary
Gradual onset of fatigue, lethargy and exertional dyspnoea
Non-smoker, not much alcohol, good diet
On examination
Pallor (conjunctival and nail bed)
Mild ankle oedema
What one test would you do next?

Answer 13

fbc

Question 14

FBC
WBC 4.7 × 109/l (3.7–9.5)
Hb 76 g/l (115–150)
MCV 110 fl 82–98 fl
Neutrophil count 1.4 × 109/l (NR 1.7–6.1)
Platelet count 182 × 109/l (NR 145–350)
Which test should be done next?

Answer 14

blood film

Question 15

describe this blood film

Answer 15

macrocytes but no oval macrocytes or hypersegmentation of neutrophils
Aisocytosis – more variation in size of cell
Some hypochromic cells

Question 16

describe this marrow and the dx

Answer 16

12% blast cells (normal < 5%)
45% of erythroblasts were ring sideroblasts

acquired sideroblastic anaemia

DIAGNOSIS: myelodysplastic syndrome (MDS) (MDS with excess of blasts)

Question 17

if only going to live a year - does ferritin of 875 μg/l (NR 20–200) matter

Answer 17

Probably not because we think only going to last a yr

Not likely to get haemosiderin deposition in tissues until ferritin was in the 2000s – so just need to monitor

Question 18

describe this blood film

Answer 18

Oval macrocytes
Tear drop
6 lobes of neutrophil – hypersegmentatuion

Question 19

A 72-year-old Indian woman
Vegetarian, teetotal, non-smoker
Shortness of breath on exertion
Fatigue
Painful gums and tongue
Unable to eat spicy food
On examination: pallor only

what is the most important test

Answer 19

Vitamin B12 and folate assays

Don’t need marrow – can take dx from film

Question 20

Vitamin B12 180 ng/l (NR 125‒600)
Red cell folate 227 pg/l (NR 215‒650)
Thyroid function normal
Liver function tests mildly impaired (bilirubin 20 μm/l, AST 110 iu/l (NR 40‒135)
Lactate dehydrogenase (LDH) 3870 iu/l (NR 50‒450)
What would you do next?

Answer 20

bone marrow aspirate

note: B12 can be normal ie false normal, when have intrinsic factor ab

Question 21

Blood gases were normal
Blood count was not
WBC 18.6 × 109/l
Hb 180 g/l
Platelet count 1648 × 109/l
Ultrasound examination of the abdomen showed normal kidneys and increased splenic size
gangrenous toes
reduced distal pulses
breathless on exertiion and morning cough
smoker

dx?

Answer 21

polycythaemia vera

Question 22

test to confirm polycythaemia vera

Answer 22

Molecular analysis for JAK2 mutation
Most people with polycythaemia vera have mutation in JAK2

Question 23

mx for polycythaemia vera

Answer 23

Venesection plus hydroxycarbamide

Venesection – get iron down
Hydroxycarbamide to reduce plts