lukaemia

Question 1

definition of leukaemia

Answer 1

a disorder where there is a defect in the Bone marrow + other blood forming organs causing over proliferation of cells and increased number of leukocytes

most common childhood cancer less than 1% of uk cancer cases. nearly 800 new cases each year.

Question 2

ALL pathophysiology + risk factors

Answer 2

lymphoid cells- affecting T or B cell lineages. B is more common

immature blast cells hyper proliferate, leading to marrow failure and tissue infiltration.

occurs early in the lymphoid lineage as opposed to chronic which occurs later on.

Risk factors: less than 5 years old, family history of ALL, radiation exposure (espesh during pregnancy), male sex,

Question 3

signs and symptoms of ALL + diagnosis of ALL

Answer 3

lymphadenopathy, hepatosplenomegaly, pallor, ecchymoses or petechiae, fever, fatigue/ dizziness/ palpitations/ dyspnoea, bruising, epistaxis, menorrhagia.

diagnosis: presence of > 20% lymphoblasts in bone marrow is diagnostic for ALL.

the presence of leukaemic lymphoblast’s ≥1 x 10⁹/L (≥1000/microlitre) in the peripheral blood is sufficient to defer a bone marrow examination at presentation.

Question 4

investigations for ALL

Answer 4

FBC with differential: normocytic normochromic anaemia with low reticulocyte
leukocytosis (high WCC- worse prognosis)
neutropenia
dec Ca, inc K, + phosphorous
inc uric acid
clotting screen

Blood smear: presence of blasts
LP- look for the presence of WBC in the CSF. more than 5 per microliter is significant.

CXR- to check for mediasteinal involvement

Bone marrow biopsy

Question 5

why is there the need for CNS surveillance and chemotherapy

Answer 5

whilst it is rarely diagnosed all pts receive Methotrexate regardless of findings.

if omitted vast majority relapse in the CNS.

ALL lymphoblasts can survive in the CNS ‘niche’ for long periods of time, and ones that are refractory to therapy my preferentially go to the CSF.

Question 6

what is CD2 and CD10 in ALL

Answer 6

T cells express CD2

B cells express CD10. essentially.

Question 7

prognosis and risk of ALL

Answer 7

Question 8

treatment of ALL

Answer 8

maintenance- 1x montly IV + intrathecal chaemotherapy

Question 9

what is tumor lysis syndrome

Answer 9

due to extra metabolites go into renal failure

give allopurinol (prevents formation of uric acid by inhib xanthine oxidase)

hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia

risk factors
High WCC
mediastinal mass
bulky abdo disease
high LDH and uric acid
reduced urine output and renal function
renal involvement by leukaemia

Question 10

overview of AML

Answer 10

much more rare. more common with age. 1/10 000
worse prognosis rapid death 2/12. 20% 3 yr survival.

more common in trisomy 21

Rx more intense but shorter than ALL.- often with stem cell transplant.

Question 11

classification system for AML

Answer 11

FAB subtype

Name

M0 Undifferentiated acute myeloblastic leukaemia

M1 Acute myeloblastic leukaemia with minimal maturation

M2 Acute myeloblastic leukaemia with maturation

M3 Acute promyelocytic leukaemia (APL)

M4 Acute myelomonocytic leukaemia

M4 eos Acute myelomonocytic leukaemia with eosinophilia

M5 Acute monocytic leukaemia

M6 Acute erythroid leukaemia

M7 Acute megakaryoblastic leukaemia

Question 12

signs and symptoms of AML
diagnosis of AML

Answer 12

anaemia, infection, bleeding.
hepatosplenomegaly, gum hypertrophy, skin involvement.

inc WCC, but can be normal/ low. occasionally small numbers of blast cells in blood –> bone marrow biopsy –> auer rods present (key distinguisher from CML)

Question 13

treatment of AML

Answer 13

V intense chaemo- long periods of marrow suppression. (neutropaenia, dec platelets)

Danuorubicin, cytarabine

stem cell transplant.

lower relapse 60% long term survivors. sig mortality of 10%.

Question 14

overview CML

signs and symptoms of CML

Answer 14

present with large WCC
2-3% of leukaemia in children (15% of all leukaemia’s) . most common in 40-60 years old.

generally ok- then progresses to blast cell crisis
Philadelphia chromosome (translocation between 9-22 linked (both ML)

chronic and insidious- dec weight, tiredness, fever, sweats. features of gout (purine breakdown), bleeding, abdo discomfort(hepatosplenomegaly).
30% are detected by chance

Question 15

investigations +

treatment for CML

Answer 15

Ix: FBC-> significantly increased WCC (more than 100 often) dec hb, inc urate, inc b12, hypercellular bone marrow.

hydroxycarbamide
tyrosine kinase inhibitors
blast crisis- intense chameo. bone marrow transplant

Question 16

stages of CML

Answer 16

chronic phase- few symptoms, lasts years
accelerated phase- inc symptoms, size of organs, difficulty in controlling counts.

blast phase- features of acute leukaemia and death.

Question 17

CLL overview, signs and symptoms

Answer 17

the commonest leukaemia
progressive accumulation of of a malignant clone of functionally incompetent B cells.

often few symptoms- anaemic or infection prone, dec weight, sweats, anorexia

enlarged rubbery non-tender nodes, splenomegaly, hepatomegaly.

Question 18

investigations and treatment for CLL

Answer 18

FBC- inc lymphocites.
later autoimmune haemolysis, marrow infiltration, dec hb, dec neuts, dec plts.

consider drugs if symptomatic- fludarabine- rituximab - cyclophosphamide is 1st line.
supportive care.
stem cell may have a roll in carefully selected pts.

Question 19

complications and stages for CLL

Answer 19

autoimmune haemolysis, infection due to dec igG, marrow faliure.

1/3 never progress, and even re-gress.
1/3 origress slowly
1/3 progress actively.