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Flashcards in Malignant Haematology Deck (38):
1

Causes of haematological malignancies

1. Leukaemias
2. lymphomas
3. Myelodysplasias
4. myeloproliferative disorders

2

Types of leukaemias

Acute lymphoblastic leukaemia (ALL)
Acute myeloid leukaemia (AML)
Chrninic lymphocytic leukaemia (CLL)
Chronic myeloid leukaemia (CML)

3

Acute lymphoblastic leukaemia (ALL) definition

malighnancy of lymphoblast cells (T & B cells)

"arresting maturation and uncontrolled proliferation of immature blast cells"

4

Causes of Acute lymphoblastic leukaemia (ALL)

Genetic (transloactions + Klinefletors)
+
env trigger

5

Presentation of Acute lymphoblastic leukaemia (ALL)

Tired, pale, bruised limping child !!

Anaemia: weakness and lethargy

Haemorrhage: purpura, bruising

Infiltration: lymphadenopathy, splenomegaly

decreased functioning WCC: infection

6

Investigations of Acute lymphoblastic leukaemia (ALL)

FBC
- WCC and pancytopenia

Peripheral blood film
- immature lymphoblasts
-FAB classification

BM biopsy
- Blast infiltrate >20%
-cytogenetics

CT and CXR
-lymphoblastic lymphoma

Lumar puncture
-CNS involvement

7

FAB classification of Acute lymphoblastic leukaemia (ALL)

FAB classification:
L1 – small uniform blast cells with scanty cytoplasm
L2 – large heterogenous blast cells with nucleoli and low nuclear-cytoplasmic ratio
L3 – basophilic vacuolated blast cells

8

Chemo Management of Acute lymphoblastic leukaemia (ALL)

Complex, specialist units

Often in clinical trial

Prednisolone, cyclophosphamide, anthracycline, vincristine, etoposide

1)Remission Induction: achieve complete remission

2)Consolidation Therapy: reduce risk of relapse

3)CNS Prophylaxis: irradiation / intrathecal

4)Maintenance Therapy: 1-2 years (or SCT)

9

Supportive management of Acute lymphoblastic leukaemia (ALL)

Tranfusion – if symptomatic

Fresh frozen plasma – for coagulopathy or DIC

Platelet transfusion – if bleeding/bruising and during fever/sepsis/DIC

Allopurinol and good urine output – to prevent tumour lysis syndrome:
-occurs in leukaemias with high WCC due to tumour necrosis (from treatment) causing renal, cardiac and neurological complications; allopurinol prevent hypercalcaemia and good urine output prevents hyperkalaemia

Antibiotics – prophylaxis against neutropenia; also should have antifungal, antiviral, good mouthcare, avoid certain foods (cream, paté, raw foods)

Growth factors – if neutropenic

Granulocytes – if unresponsive to treatment of infection

10

Chronic lymphocytic leukaemia definition

Clonal expansion of lymphocytes (mainly B cells)

Accumulate in blood, lymph nodes, bone marrow, spleen and liver

11

Chronic lymphocytic leukaemia features

males> females (2;1)
Elderly
mean age is 67

12

Presentation of Chronic lymphocytic leukaemia

50% incidental
- Lymphocytosis on FBC

Symptomatic

Anaemia: lethargy, pallor, dyspnoea
Thrombocytopenia: bruising/bleeding

Lymphadenopathy, splenomegaly, hepatomegaly

Recurrent infections

B symptoms
-Fever
-Night sweats
-Weight loss

13

Investigations of Chronic lymphocytic leukaemia

FBC
-Lymphocytosis (>5x109/L), pancytopenia

Blood film
-Larger normal mature lymphocytes & smear cells

Bone marrow biopsy
-Increased numbers of lymphocytes
-Nodular or diffuse infiltrate

Immunophenotyping
Via flow cytometry: CD5, 19, 20, 23 +ve

14

Staging of chronic lymphocytic leukaemia

Binet staging system (or RAI)

Stage
A- 3 LN involved no anaemia or thrombocytopenia 5 years
C - Anemia or thrombocytopenia 2 years

15

Management of chronic lymphocytic leukaemia

Stage A:
- Observation only

Stage C or symptomatic:
-Fludarabine, Cyclophosphomide, Rituximab
- Allogeneic SCT

Palliative:
-Minimum toxicity chemotherapy
-Radiation to refractory, bulky lymphadenopathy

16

Complications of chronic lymphocytic leukaemia

Immune complications

-Autoimmune haemolytic anaemia
-Autoimmune thrombocytopenia
- Manage with steroids

Infections
-As likely to die of infection as from CLL
-Pulmonary infections common
(Bacterial, viral, pneuomocystis, fungal)

17

Prognosis of chronic lymphocytic leukaemia

Mainly incurable

-5 year survival 18 months-10 years
-Poor prognosis from cytogenetics
-17p and 11q deletions
(Loss of p53)- Chemo can’t work

18

Acute myeloid leukaemia definition

Prolieration of immature NON-lymphoid cells

19

Acute myeloid leukaemia Features

Presentation similar to ALL
Increases incidence with age

20

Risk factors of Acute myeloid leukaemia

Myelodysplasia
Down Syndrome
Prior chemotherapy ± radiotherapy
Benzene exposure

21

Investigations of Acute myeloid leukaemia

FBC
- increased WCC, pancytopenia

Blood smear
-Blasts

Bone marrow
-Blasts, Auer rods
-immunophenotyping & cytogenetics

22

Management of acute myeloid leukaemia

Intensive chemotherapy ± SCT
65, 5 year survival

23

Prognosis of acute myeloid leukaemia

5 year survival
Children: 60-70%
Adults (60): 10-20%

Prognostic factors
t(15:17) = favourable
Normal = intermediate
t(6:9), 2° AML, relapsed AML, elderly = unfavourable

24

Define leukaemia

Malignancy of haematopoietic stem cells resulting in the accumulation of abnormal cells in the bone marrow ± peripheral circulation

25

Chronic myeloid leukaemia definition

uncontrolled proliferation of myeloid precursors, ability to differentiate remains intact

26

Cause of chronic myeloid leukaemia

t (9:22)- reciricol transloaction between long arm of 9 and long arm of 22

forms fusion gene with BCR-ABL

has tyrosine kinase acitivty

KNOWN AS THE PHILIDELPHIA CHROMOSOME

27

Presentaiton of chronic myeloid leukaemia

insidious onset
-weight decrease
-fatigue
-sweats
-fever

abnormal discomfort (splenomegaly )

28

Investigations of chronic myeloid leukaemia

BM
-hypercellular

FBC
-increased WCC (often > 100x 10 9)
-neutrophils, basophils, eosinophills meylocytes
-decreased hb or normal
-Plts variable
-urate and B12

Cytogenetics
-look for philly chromosome

29

Phases of chronic myeloid leukaemia

Chronic
-few symptoms can last years

Accelerated:
-increaseing symptoms and splenic size- symptoms form cellular accumulation

Acute -
blast trasnformations from; symptoms from bone marrow failure

30

Treatment of chronic myeloid leukaemia

Imatinib
Tyrosine kinase inhibitors

31

Prognosis of chronic myeloid

1/3 never rpogress
1.3 progress slowly
1/3 progress rapidly

32

Prognosis of ALL

5 year survival
90% in children
40% in adults

Cytogenetics
t(10:14) = favourable
Normal = intermediate
t(9:22) = adverse

33

Myeloma Definition

haematological malignancy of plasma cells

34

Causes of myeloma

Abnormal prolferation of a single clone of plasma or lymphocytes
Leads to secretion of monoclonal (paraprotein) Ig light chain (igG - most common, IgA second most common)

Accumulates in blood
Arises in BM
Microenvironment produces cytokines - osteoclastogenesis

35

Presentation of myeloma

Majority of new cases have non-specific symptoms
-backache or rib pain (60%)
-fatigue
-symptoms from hyper Ca++ (30%)
-recurrent infections
-renal impairment (25-30%)

CRAB
C- hypercalcamia
R- renal dysfunction
A- anaemia
B- bone pain

36

Triad of myeloma

1. Increased plasma cells in bone marrow
2. Clonal immunoglobulin or paraprotein
3. Lytic bone lesions

37

Investigations of myeloma

Blood tests
-FBC - anaemia or thrombocytopenia
-U and Es - increased Ca2+, increased urea, + creatiie

Urinalysis
-to look for M-protein in urine – “Bence-Jones protein
- Red cell casts

Bone marrow aspirate
Imaging

Serum and urine electrophesis M band

38

Myeloma treatment

Fit for autograft?
Chemotherapy – novel therapies eg thalidomide, bortezomib

Radiotherapy
BMT – autograft if fit; allograft rare

Supportive therapy
-biphosphonates
-blood transfusion/EPO
-surgery
-interventional radiology