Leukaemia

Question 1

what are the 4 broad groups of leukaemia?

Answer 1

Acute lymphoblatic leukaemia ( ALL)

Acute myeloid leukaemia

Chronic lymphocytic leukaemia

Chronic Myeloid leukaemia

Question 2

summarise leukaemia?

Answer 2

bone marrow disease-> Series of mutations in a single lymphoid or myeloid stem cell

mutations lead the progeny of that cell-> show abnormalities-> lead to steady expansion of leukaemic clone

Question 3

How is leukaemia different from other cancers?

Answer 3

does not exist as a solid tumour but rather as leukaemic cells replacing normal bone marrow cells in circulation

-> but rather as leukaemic cells replacing normal bone marrow cells in circulation-> this is why it can disseminate quickly

Question 4

what is acute leukaemia?

Answer 4

rapid increase in immature blood cells which crowd out the bone marrow

abnormal differential+ excessive proliferation

Acute leukaemia= severe leukaemia

Question 5

what is chronic leukaemia?

Answer 5

excessive build up of abnormal but relatively mature white blood cells

normalish differentiation and excessive proliferation

mainly in older people

Question 6

summarise the epidemiology of acute lymphoblastic leukaemia?

Answer 6

most common malignancy of acute lymphoblastic leukaemia

peak incidence 2-5 years old

second peak in incidence in the elderly

annual UK incidence: 1/70,000

Question 7

what is AML?

Answer 7

rapid proliferation of myeloblasts

features of neutropenia, anaemia and thrombocytopenia

Question 8

what are the risk factors for AML?

Answer 8

incidence increases with age

Downs syndrome, irradiation, anti-cancer drugs

Question 9

what are the signs and symptoms of AML?

Answer 9

bone marrow failure,: pallor, bleeding, infections

Tissue infiltration: swollen gums, mild splenomegaly

Question 10

what is an agressive subtype of AML?

Answer 10

acute promyelocytic leukaemia

Question 11

what is the cause of acute promyelocytic leukaemia?

Answer 11

genetic translocation t(15,17) fuses the PML gene with RAR-alpha gene

Question 12

what is seen on cytology for acute prolmyelocytic leukaemia?

Answer 12

faggot cells on cytology- lots of auer rods

Question 13

what is ALL?

Answer 13

rapid proliferation of lymphoblasts

features of neutropenia, anaemia and thrombocytopenia

Question 14

what are the risk factors for ALL?

Answer 14

Most common childhood cancer

genetics, radiation, influenza

Question 15

what are the signs and symptoms of ALL?

Answer 15

bone marrow failure: pallor, bleeding, infections

tissue infiltration: lymphadenopathy, hepatosplenomegaly, swollen testes tender bones

Question 16

how is ALL diagnosed?

Answer 16

more than 20% lymphoblasts on bone marrow

Question 17

what is CML?

Answer 17

hyperpoliferation of granulocyte precursors-> features of bone marrow failure , hyper metabolism and hyper viscosity

Question 18

what are the phases of CML?

Answer 18

chronic phase
accelerated phase
blast crisis

Question 19

Summarise the epidemiology of CLL?

Answer 19

• 90% are > 50 yrs
• Commonest leukaemia – incidence 4/100,000/yr
• More common in MALES
• Rare in Asians

Question 20

what are the risk factors for CML?

Answer 20

male to female ratio= 1.4: 1

philadelphia chromosome: t(9:22) BCR-ABL 1 fusion gene

Question 21

what are the signs and symptoms of CML?

Answer 21

up to 50% are asymptomatic

90% have massive splenomegaly

hypermetabolic symptoms: weight loss, malaise, sweating

Bone marrow failure: pallor, bleeding infections

Hyperviscocity symptoms: thrombotic events, headaches

Question 22

what is CLL?

Answer 22

Progressive accumulation of functionally incompetent lymphocytes

occasional symptoms of bone marrow failure, hyper metabolism

caused by a failure of apoptosis

Question 23

what are the risk factors for CLL?

Answer 23

male to female ration= 2:1

genetic risk factors

Question 24

what are the signs and symptoms of CLL?

Answer 24

50% are asymptomatic

occasionally non-tender lymphadenopathy ( small lymphocytic lymphoma)

occasional bone marrow failure symptoms

Question 25

describe the diagnosis of CLL?

Answer 25

usually diagnosed by routine blood test- leukocytosis

smear. smudge cells on blood film

Question 26

what are the investigations for leukaemia?

Answer 26

bloods= FBC, LDH, blood smear

Biopsies= bone marrow aspirate

Other tests= immunophenotyping, CXR

Question 27

what is the typical presentation of CLL?

Answer 27

enlarged rubbery lymph nodes, non tender, also smear cells (Rai and Binet Staging)m increased lymphocyte

Question 28

What is the typical presentation of CML?

Answer 28

Philadelphia chromosome, sweats, more likely splenomegaly, increased WCC (granulocyte at different stages of maturation)

Question 29

what is the typical presentation of ALL?

Answer 29

bruising, pale, usually children btw 2-4 years old, hepatosplenomegaly and swollen testes and mediastinal mass (thymus enlargement if T cell infiltration)

Question 30

what is the typical presentation of AML

Answer 30

Auer rods (sudan black stain - and preferentially stains myeloblasts), SOB, bone pain

Question 31

what is the typical presentation of promyelocytic leukaemia?

Answer 31

subtype of AML, very aggressive, associated with DIC, t(15;17)

Question 32

what are the buzz words for leukaemias?

Answer 32

AML= Auer rods

ALL= children < 6 years

CML= philadelphia chromosome t(9,22), BCR-ABL gene

CLL= smear/smudge cells

Question 33

what is seen on cytology in AML?

Answer 33

auer rods