CML and CLL

Question 1

CML is

Answer 1

chronic clonal proliferation of myeloid cells (basophils, neutrophils, neutrophils) = myeloproliferative disorder
Philadelphia chromosome in 80% cases

Question 2

Presentation of CML and progression

Answer 2

Chronic and insidious 
Systemic symptoms = weight loss, tiredeness, fevers, sweats
Gout features due to purine breakdown 
abdominal discomfort and fullness 
infection 
thrombotic episodes 

Progression:
fever and infection
bone and joint pain
haemorrhage and thrombosis

Question 3

examination for CML

Answer 3

Splenomegaly
Hepatomegaly
Anaemia
Bruising

Question 4

Ix for CML

Answer 4

FBC = WBC high, normochromic anaemia, low neut alk phos (NAP)
Bone Marrow Aspirate = hypercellular
Cytogenetic analysis = Philadelphia chromosome

Question 5

Management of CML

Answer 5

Chemotherapy : imatinib

Stem cell transplant: allogenic bone marrow is curitive and used in young patients or those intolerant to imatinib

Question 6

CLL is?

Answer 6

Accumulation of mature B cells that have escaped apoptosis and undergone cell-cycle arrest. Commonest Leukaemia

Question 7

Staging for CLL

Answer 7

Rai Staging
0 = lymphocytosis alone
I = lymphocytosis + lymphadenopathy
II = lymphocytosis + splen or hepatomegaly
III = lymphocytosis + anaemia (Hb < 110)
IV = lymphocytosis + platelets < 100

Question 8

History of CLL

Answer 8

Often nothing - incidental findings on blood 
= recurrent infections 
= anaemia 
= painless lymphadenopathy
= LUQ discomfort splenomegaly

Question 9

Examination of CLL

Answer 9

fever
anaemia
enlarged, tender, rubber nodes
splenomegaly + hepatomegaly

Question 10

Ix

Answer 10

FBC 
- High lymphocytes > 5 
- later, decreased Hb, neutrophils and platelets secondary to bone marrow infiltration 
Blood Film 
 - Normal appearing lymphocytes 
 - no immature blast cells 
Bone marrow 
 - heavily infiltrated with lymphocytes 
Direct Coomb's test 
 - may be +ve if haemolytic anaemia

Question 11

when to and what to manage in CLL

Answer 11

0 = watch and wait
I - II = treat if progression
III - IV = treatment indicated

Absolute indication:

• marrow failure
• recurrent infection
• systemic symptoms
• massive splenomegaly or lymphadenopathy
• haemolysis

Supportive Tx
- Steroids, transfusion, IvIg, Prophylactic Abx, allopurinol for hyperuricaemia

Specific Tx

• FCR (chemo)
• Allogenic stem cell transplant

Question 12

Complications of CLL

Answer 12

AI haemolysis
Infections due to low IgG
Marrow Failure