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Malignant haematology > Chronic lymphocytic leukaemia > Flashcards

Chronic lymphocytic leukaemia Flashcards

(51 cards)

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1
Q

What is CLL

A

Type of blood cancer occurs due to monoclonal proliferation of B lymphocytes

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2
Q

Who gets CLL

A

Older adults
Exceedingly rare in children
More common in men

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3
Q

Causative factors

A

Cause unknown but more likely if:
Deletion in chromosome 13 can be 11 or 17 too
FH - 6-9x risk if first degree family had
Immunity - HIV/AIDS 3x likely

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4
Q

Pathophysiology of CLL

A

CD5+, CD23+ clonally expand, low surface IgGs
-> accumulation lymphocytes bone marrow -> lymph nodes and other lymphoid tissues

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5
Q

Main mechanisms of CLL

A

Deletion of chromosome
Hypogammaglobinaemia and haematopoieses
Transformation to aggressive forms

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6
Q

Genetic changes in CLL

A

13q chromosome deletion 50^
deletions at 11q, 17p (p53 tumour supressor) chromosomes

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7
Q

What is 17q deletion CLL ass with

A

p53 tumour supressor gene inactivated
Rapid progression, short remission, decreased overall survival

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8
Q

What haem disorders are people with CLL more likely to get

A

AI haemolytic anaemias
AI thrombocytopenia

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9
Q

What can CLL transfomr to

A

B cell pro lymphocytic leukaemia -> non hodgkin lymphoma

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10
Q

What is richters transformation

A

Richters transformetion = CLL -> diffuse large B cell lymphoma

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11
Q

Clinical features CLL

A

Often asymptomatic esp in early stages
Symmetrical enlarged lymph nodes
hepatomegaly/splenomegaly
Anaemia - tired
Recurrent infections as low Igs
Bleed/brusie - TO

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12
Q

Signs that CLL is active

A

B cell symptoms
Frequent sev night sweats
Unezplained weight loss >10% BW in last 6 months
High fever absence infection >38

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13
Q

Investigating CLL what do

A

FBC
Reticulocyte count
Direct antiglobulin test - DAT
immunophenotype
Biochem and serum IgGs
Peripheral blood smear

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14
Q

What do lymphocytes do in CLL

A

> 5000 B lymphcytes/uL - lymphocytosis

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15
Q

What test confirms CLL

A

Immunophenotyping of lymphocytes

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16
Q

How confrim lymphocytosis

A

Peipheral blood smear
Small lymphcytes - scant cytoplasm and nuclei w clumped chromatic
Smudge cells

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17
Q

What are smudge cells

A

Artefacts from damaged lymphocytes in slide prep as so fragile

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18
Q

What cells are suggestive of CLL on smear

A

Smudge cells

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19
Q

What genetic test should be performed on all patients before treatment with CLL

A

Detect TP53 gene
Adverse prognosis if deleted
Determines treatment

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20
Q

Additional investigaitons for CLL

A

Bone marrow aspiration and biopsy - before therapy as baseline
DAT/coombs test - in all anaemic patients and before commencing therapy
Imaging - CT scan chest abdo pelvis - uropathy or AW obstruction from lymph node compression on organ or structures

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21
Q

What do DAT/coombs test for

Study These Flashcards
A

All anaemic patients and before commencing therapy look for AI related haemolytic anaemia

22
Q

Mantle lymphoma vs CLL

Study These Flashcards
A

Mantly lymphoma doesnt express CD23 antigen

23
Q

Marginal zone lymphoma vs CLL

Study These Flashcards
A

similar presentation and immunophenotype as CLL, but typically has a bright surface immunoglobulin and CD20, and bone marrow examination often reveals lymphocytes with notched nuclei.

24
Q

How tell B cell pro-lymphocytic leukaemia from CLL

Study These Flashcards
A

> 55% prolymphocytes CD5 negative circulating cells

25
What staging is used for CLL
Binet staging
26
Stage A of CLL
>10g/dL Hb, platelets at least 100x109/L, < 3 lymph node areas invovled
27
Stage B CLL
Stage A but >3 lymoh node areas involved
28
Stage C CLL
Hb <10g/dL PLatelets <100x109 or both
29
What is one are of lymph nodes
Lymphadenopathy in one area of body irrelevant of if one or both sides
30
Strategy w early CLL
Watch and wait - regularly monitor
31
Indicaitons for chemotherapy in CLL
Evidence progressive marrow failure Massive or progressive or symptomatic splenomegaly or lymphadenopathy Progressive lymphocytosis >50% increase 2 months Lymphocyte doubling time <6 months AI anemia or TP not repsonding to prednisolone ONe or more of CLL related smyptoms
32
CLL related symptoms indicating treatment
Night sweats High fever Extreme fatigue Unintentional weight loss >10%
33
Initial therapy for CLL without Tp53 deletion
Fludarabine Cyclophosphamide Rituximab FCR
34
First line treatment CLL if FCR not appropriate
Bendamistine For Stage B/C
35
3rd option for treatment CLL if dont tolerate other two
Obinutuzumab combo w chlorambucil
36
What use in Tp53 mutations CLL
Ibrutinib Alemtuzumab
37
What treat AI cytopenia with
Corticosteroids
38
Why are steroids sometimes given before chemo
Improve bone marrow function where theres significant bone marrow infiltration
39
Relapsed vs refractory CLL
Relapse - Disease responded to therapy but after 6 > months stopped responding Refractory - doesnt result in remission but more be stable or gets worse within 6 months last treatment
40
Curative treatmetn CLL
HSCT
41
Complications CLL
Infection - defctive complement, t cell dysunction, low neutrophils Anaemia incl AI haemolytic - warm antibody type Transformation to other lymphoma Hyperviscosity syndrome Malignancies - higher risk seconadry
42
Richters sydnrome symtpoms
Sudden and dramatic increase in size of lymph nodes in neck, axilla, abdo (spleen) or groin Drmatic unexplained weight loss gever and night sweats
43
Clinical features hyperviscosity syndorme
Extremely high WCC -> Altered CNS function/resp insufficiency Headahce, dizzy, vertiy=go, hearing loss, visual disturb, nystagmus
44
Most common secondary malginancies risk higher w CLL
Melanoma, soft tissue sarcoma, colorectal cancer, lung cancer, SCC, BCC
45
Risk factors for worse prognosis CLL
Older Men Tp52 and 17p dletion Trisomy 12 Lymphocyte doubling time < 12 months Diagnosis at later stage
46
What features mean survival >10 years
Lymphocytosis only in CLL
47
What intermediate features mena survival of around 7 years
Lymphadenopathy, hepatomegaly or splenomegaly + lymphocytosis
48
What parameters mean survival likely <4 years w CLL
Hb <110 Platelets <100,000/mm3
49
Normal bone marrow what made up of
50% fat 50% cellular acitivty
50
What makes a leukaemia acute vs chronic
Acute - arrested before differentation eg blasts Chronic - mature cell mutation
51

Malignant haematology (16 decks)

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