lymphoproliferative disorders

Question 1

leukaemia

Answer 1

cancer seen in the blood

progressive, malignant disease of blood forming organs
distorted proliferation + development of leukocytes and their precursors in blood and bone marrow

Question 2

lymphoma

Answer 2

cancer of lymphoid origin

can present with: lymphadenopathy, extranodal involement, bone marrow invovlement

Question 3

B symptoms

Answer 3

weight loss
fever
night sweats 
pruritus
fatigue

Question 4

diagnosing leukaemia/lymphoma: biopsy

Answer 4

lymph node, bone marrow

tells us what type it is - makes diagnosis

Question 5

diagnosing leukaemia/lymphoma: clinical exam and imaging

Answer 5

CT
extent and staging of disease
prognosis
can sometimes influence treatment

Question 6

acute lymphoblastic leukaemia originations

Answer 6

lymphoid progenitor cells

bone marrow

Question 7

lymphoma/chronic lymphocytic leukaemia originations

Answer 7

B lymphocytes
T lymphocytes

lymph nodes

Question 8

multiple myeloma originations

Answer 8

plasma cells

bone marrow

Question 9

lymphoma subsets

Answer 9

hodgkin lymphoma

non-hodgkin lymphoma (high or low grade)

Question 10

lymphoproliferative disorders

Answer 10

ALL
CLL
Hodgkin’s lymphoma
non-hodgkin’s lymphoma

Question 11

ALL

Answer 11

cancerous disorder of lymphoid progenitor cells

no differentiation, rapid uncontrolled growth and accumulation

75% cases <6yrs
74-90% B cell lineage

Question 12

ALL risk factors

Answer 12

<5yrs
mid 80s
smoking

Question 13

ALL features

Answer 13

impaired vision
retinal haemorrhages on fundoscopy 
weight loss, fever, fatigue
SOB, dizzy
anaemia, thrombocytopenia, leucocytosis
>20% cells in bone marrow lymphoblasts

Question 14

ALL investigations

Answer 14

FBC, WCC, Hb, platelets

bone marrow aspiration

Question 15

ALL cell characteristics

Answer 15

large cells
express CD19
express CD34, TFT (markers of early, immature cells)

Question 16

ALL standard Rx

Answer 16

• induction chemotherapy to obtain remission (multi-agent)
• consolidation therapy to maintain remission
• CNS directed therapy: through LP, methotrexate
• maintenance Rx 18mo
• STC if high risk recurrence/relapse

Question 17

ALL newer therapies

Answer 17

bi-specific T cell engagers

chimeric antigen receptor T cells

Question 18

ALL: T cell immunotherapies side effects

Answer 18

cytokine release syndrome: fever, hypotension, SOB

neurotoxicity; confusion w normal conscious level, headache, focal neurology, seizures

Question 19

ALL poor prognostic factors

Answer 19

inc age - inc risk relapse
increased WCC
slow/poor response to Rx

Question 20

CLL

Answer 20

indolent lymphoproliferative disorder where monocloncal B cells predominantly found in peripheral blood

lymphocyte count >5

Question 21

CLL cel characteristics

Answer 21

slow growing
mature
low grade condition
carry normal B cell markers

Question 22

CLL risk factors

Answer 22

> 60yrs
male
white
FHx CLL

Question 23

CLL clinical features

Answer 23

asymptomatic; incidental finding FBC
bone marrow failure: thrombocytopenia, anaemia
splenomegaly 
fever, sweats 
lymphadenopathy

Question 24

CLL indications for Rx

Answer 24

progressive bone marrow failure 
lymphocyte doubling time <6mo
progressive splenomegaly 
massive lymphadenopathy 
systemic features
autoimmune cytopenias

Question 25

CLL binet staging A

Answer 25

<3 node ares

Question 26

CLL binet stage B

Answer 26

3+ node ares

Question 27

CLL binet stage C

Answer 27

3+ node areas | thrombocytopenia or anaemia

Question 28

CLL Rx

Answer 28

often nothing, watch + wait cytotoxic chemo: fludarabine, bendamustine monoclonal antibodies: rituximab -novel agents: P13K inhibitor, BCL2 inhibitor

Question 29

CLL poor prognostic markers

Answer 29

``` rapid lymphocyte doubling time advanced disease: Binet B, C atypical lymphocyte morphology loss/mutation P53 CD38+ expression ```

Question 30

lymphoma presentation

Answer 30

``` lymphadenoapthy hepatosplenomegaly extranodal disease B symptoms bone marrow involement ```

Question 31

staging of lymphoma: how it is done

Answer 31

lymph node biopsy CT scan bone marrow aspirate + trephine

Question 32

lymphoma stage 1

Answer 32

one nodal site involvement

Question 33

lymphoma stage 2

Answer 33

2 nodal sites involvement, either above or below diaphragm not both

Question 34

lymphoma stage 3

Answer 34

multiple nodal sites involvement above and below diaphragm

Question 35

lymphoma stage 4

Answer 35

extranodal disease e.g. liver, bone

Question 36

non-hodgkin lymphoma: how can it be classified

Answer 36

lineage: B cell or T cell grade: high or low

Question 37

NH lymphoma low grade

Answer 37

indolent, often asymptomatic | responds to chemo but incurable

Question 38

NH lymphoma high grade

Answer 38

aggressive, fast growing | can be cured (combo chemotherapy)

Question 39

diffuse large B cell lymphoma

Answer 39

commonest subtype | high grade

Question 40

follicular lymphoma

Answer 40

low grade | often watch and wait

Question 41

lymphoma Rx

Answer 41

combination chemotherapy - anti-CD20 monoclonal antibody (rituximab) - chemo

Question 42

hodgkin lymphoma

Answer 42

B cells | characterised by presence hodgkin's cells and reed-stenburg cells

Question 43

hodgkin lymphoma risk factors

Answer 43

EBV glandular fever geographical and familial clustering

Question 44

hodgkin lymphoma Rx

Answer 44

combination chemotherapy +/- radiotherapy monoclonal antibodies (anti-CD30) immunotherapy

Question 45

ALL bone marrow finding

Answer 45

>20% bone marrow is progenitor cells