Myeloid disease and leukaemia

Question 1

Briefly describe the pathophysiology of leukaemia

Answer 1

genetic mutation in one of the precursor cells in the bone marrow which leads to excessive production of a single type of abnormal white cell. Excessive production can lead to suppression of the other cell lines, causing underproduction- pancytopenia

Question 2

Define pancytopenia

Answer 2

anaemia + leukopenia + thrombocytopenia

Question 3

Three features of leukaemia presentation?

Answer 3

non-specific

• fatigue
• fever
• pallor
• petechiae, abnormal bruising
• abnormal bleeding
• lymphadenopathy
• hepatosplenomegaly

Question 4

Two differentials for petechiae

Answer 4

leukaemia
meningococcal septicaemia
vasculitis
HSP
ITP
non-accidental injury

Question 5

Three investigations for leukaemia

Answer 5

FBC
Blood film
Bone marrow biopsy
CXR- infection, mediastinal lymphadenopathy
Lymph node biopsy
CT/PET/MRI for stagin

Question 6

Name two types of bone marrow biopsy

Answer 6

Aspiration- liquid

Trephine- solid core (better sample but takes longer to analyse than aspirate)

Question 7

Site of bone marrow biopsy?

Answer 7

iliac crest

Question 8

Are leukemias myeloid or lymphoid in origin?

Answer 8

can be both!!

Question 9

Which leukeaemia is associated with Down’s syndrome?

Answer 9

ALL

Question 10

Which is the childhood leukaemia?

Answer 10

ALL

Question 11

Name two genetic associations of acute lymphoblastic leukaemia

Answer 11

t(15:17) translocation 30%

t(9:22) philadelphia chromosome 30%

Question 12

In which leukaemia is the philadelphia chromosome most strongly associated with?

Answer 12

CML

Question 13

Which leukaemia causes warm autoimmune haemolytic anaemia?

Answer 13

CLL

Question 14

In which leukaemia does Richter’s transformation occur in?

Answer 14

CLL, wherein it can transform to high-grade lymphoma

Question 15

Which haematological condition shows smudge or smear cells?

Answer 15

CLL

Question 16

In which condition does the philadelphia chromosome exist?

Answer 16

CML

Question 17

Which is the most common acute adult leukaemia?

Answer 17

AML

Question 18

Which leukaemia is associated with auer rods?

Answer 18

AML

Question 19

Which leukaemia results from a myeloproliferaivte disorder e.g. polycythemia vera?

Answer 19

AML

Question 20

Two complications of chemotherapy?

Answer 20

tumour lysis syndrome
infections
cardiotoxicity
secondary malignancy 
stunted growth and development in kids 
failure

Question 21

Two general management options for leukaemia?

Answer 21

Steroids
Chemo
Radiotherapy
Bone marrow transplant
Surgery

Question 22

Which chemo drug is used in CML?

Answer 22

imatinib

Question 23

In which leukaemia is there a chronic, accelerated, and blast stage?

Answer 23

CML

Question 24

Name three myeloproliferative disorders

Answer 24

Primary myelofibrosis
Polycythaemia vera
Essential thrombocythaemia

Question 25

Name the abnormal proliferating cell in the following myeloproliferative disorders: primary myelofibrosis polycythaemia vera essential thrombocythaemia

Answer 25

Primary myelofibrosis= haematopoeitic stem cell Polycythaemia vera= erythroid cells Essential thrombocythaemia= megakaryocyte

Question 26

Which cancer can myeloproliferative disorders transform into?

Answer 26

AML

Question 27

Mutations in which gene are associated with myeloproliferative disorders?

Answer 27

JAK2 mutation

Question 28

What is a complication of all myeloproliferative disorders?

Answer 28

myelofibrosis= bone marrow fibrosis, leading to anaemia and leukopenia

Question 29

Name three signs/symptoms of myeloproliferative disorders

Answer 29

systemic symptoms: fatigue, night sweats, fever, weight loss Symptoms from complications: anaemia, splenomegaly, thrombosis, infections

Question 30

List three complications of myeloproliferative disorders

Answer 30

``` anaemia splenomegaly thrombocytopenia thrombosis luekopenia ```

Question 31

Name one feature on blood film of myelofibrosis

Answer 31

tear-drop RBCs and blasts

Question 32

Briefly describe the pathophysiology of myelodysplatic syndrome?

Answer 32

myeloid bone marrow cells don't mature properly and therefore do not produce healthy resulting cells

Question 33

Name three complications of myelodysplastic syndrome relating to poor cell differentiation

Answer 33

anaemia thrombocytopenia neutropenia

Question 34

How to diagnose AML?

Answer 34

>20% cells in BM are blast cells