Haematology Lymphoma

Question 1

Lymphoma types

Answer 1

40

Question 2

3 types of lymphoma

Answer 2

Hodgkins lymphoma (15% of lymphoma)
Non-hodgkins lymphoma:
a. Low grade
b. High grade

Question 3

Low grade examples

Answer 3

Follicular
Lymphoplasmacytoid
Marginal zone

Question 4

High grade examples

Answer 4

Diffuse large cell lymphoma (most common) – 40%
Lymphoblastic lymphoma
Burkitts lymphoma

Question 5

Hodgkins lymphoma: Epi

Answer 5

Male predominance
Incidence 6 per 100000
Peak incidence – young/adults

Question 6

Hodgkins lymphoma:Association with

Answer 6

EBV infection

HIV infection

Question 7

Hodgkins lymphoma: Clinical Features

Answer 7

Painless lymphadenopathy: contiguous spread.
Neck
Mediastinum
Axilla (usually above the neck)

Question 8

Hodgkins lymphoma: B symptoms

Answer 8

Weight loss >10%
Sweats
Fever +33oC
Pruritis

Question 9

Hodgkins lymphoma: ‘Patho-pneumonic’

Answer 9

Drink alcohol and lymph node hurts

Question 10

Hodgkins lymphoma:Radiology can show

Answer 10

Hilar Lymphadenopathy
Differentials:
Sarcoid
Lymphoma

Question 11

Hodgkins lymphoma: CT showing

Answer 11

Retoroperitoneal lymphadenopathy – enlarged lymphnodes in abdomen

Question 12

Hodgkins lymphoma:Pet Scn

Answer 12

Glucose radioactive tracer. Assesses response

Question 13

Hodgkins lymphoma: Histology

Answer 13

Lymphocyte poor
Nodular sclerosing
Mixed cellularity – slightly worse prognosis

Question 14

Hodgkins lymphoma: Pathology characterised by

Answer 14

Presence of Reed-Sternberg cells

Question 15

Hodgkins lymphoma: Investigation

Answer 15

Investigation:
1.	Serum immunoglobulins
2.	Chest Xray
3.	Pet scan
4.	Lymph node biopsy
5.	U and E and LFT and LDH
Staging 
1.	Ct scan of abdomen and thorax
2.	Bone marrow trephine biopsy

Question 16

Hodgkins lymphoma: Clinical Staging

Answer 16

Anarbour classification
1. Disease limited to single region of nodes or one extranodal site (IE)
2. Disease at two sites on some side of diaphragm
3. Disease at several sites on both sides of diaphragm (includes spleen, Waldeyers ring)
4. Spread of disease to extra lymphatic structures e.g. bone marrow, gut, lung, liver
A=no symptoms
B = Wt loss, sweats, fever
(IE) – Clump of nodes in bowel w/ lymph node

Question 17

Hodgkins lymphoma: Treatment/Prognosis

Answer 17

Depends on stage

Question 18

Hodgkins lymphoma: Stage 1A-2A: treatment/prognosis

Answer 18

3-4 cycles of chemotherapy (eg ABVD) followed by radiotherapy to affected nodal areas.
Cure 80-90%

Question 19

Hodgkins lymphoma: Stage 2B-4B: treatment/prognosis

Answer 19

Combination chemotherapy alone (e.g. 6-8 cycles of ABVD)

Cure 60-80% (in young adults – worse inelderly +600

Question 20

Hodgkins lymphoma: ABVD combination and benefit

Answer 20

Adreiamycin
Bleomycin
Vinblastine
Dacarbazine

→ Fertility sparing

Question 21

Hodgkins lymphoma: Late complications of therapy

Answer 21

1. Inferility
2. Radiation pneumonitis and pulmonary fibrosis
3. Secondary cancers including acute myeloblastic leukaemia

Question 22

Non-Hodgkins Lymphoma:Epi

Answer 22

Incidence has doubled over the past two decades
20/100000: increasing exponentially with age.
Rises to 70 cases per 100,000 in over 65s

Question 23

Non-Hodgkins Lymphoma: Description

Answer 23

More commonly disseminated disease at multiple sites and extra – lymphatic infiltration

Question 24

Non-Hodgkins Lymphoma: May be reflected by

Answer 24

Symptomatology e.g. presentation with neurological, gastrointestinal or respiratory symptoms or evidence of bone marrow infiltration (Anaemia) and circulating lymphoid cells in the blood.

Question 25

Non-Hodgkins Lymphoma: Two main Groups

Answer 25

Low grade | High grade

Question 26

Non-Hodgkins Lymphoma: Low grade lymphomas

Answer 26

* Often-good response to chemotherapy but relapsing and remitting course. * Survival 7-10 years but only rarely disease – free long term. * Patients die from resistant disease, infection or transformation of lymphoma to high-grade disease.

Question 27

Non-Hodgkins Lymphoma: Low grade lymphoma Treatment

Answer 27

• 1. Wtch and see • Single agent e.g. chlorambucil • Combination chemotherapy →R-CVP (Rituximab, cyclosphamide, vincristine, prednisolone) → Rituximab = humanised therapeutic monoclonal antibody (anti-CD20)

Question 28

Non-Hodgkins Lymphoma: Rituximab action

Answer 28

CD20 on the surface of B cells lymphocyte (B cells and T cells)

Question 29

Non-Hodgkins Lymphoma: High Grade Lymphomas

Answer 29

Aggressive and rapidly progressive course if untreated. | With combination chemotherapy, however, 65%, longterm survivale/care.

Question 30

Non-Hodgkins Lymphoma: High grade lymphoma treatment

Answer 30

Treat with an attempt to cure Combination chemo: R-CHOP (rituximab, cyclophosphamide, Adriamycin, vincristine and prednisolone): 6-8 cycles If partial remission or relapse, lower remission and cure rates (15%) with 2nd line therapy (platinum – based chem and stem cell autograft)

Question 31

Non-Hodgkins Lymphoma: Autograft

Answer 31

Own stem cells (rescue side effects from chemo vs. allograft (15-20% chance of death)

Question 32

Myelomatosis: Description

Answer 32

Malignant tumour involving proliferation of plasma cells which produce monoclonal immunoglobulin (paraprotein) and/or light chain (Bence Jones proteins)

Question 33

Myelomatosis: Epi

Answer 33

Disease of late middle age and elderly

Question 34

Myelomatosis: Clinical Features

Answer 34

1. Marrow infiltration leading to anaemia 2. Infections due to hypogammaglobuinaemia – redcued of autoantibodies 3. Skeletal disease → Bone Pain → Pathological fracture → Hypercalcaemia 4. Renal Failure 5. Peripheral neuropathy 6. Hyper-viscosity syndrome ``` Calcium Renal failure Anaemia Bone pain → indication for treatment ```

Question 35

Myelomatosis: Hyperviscosity Syndrome

Answer 35

Confusion Chest Pain Vision disturbance

Question 36

Myelomatosis: Investigations

Answer 36

1. Blood count, Very high ESR, plasma viscosity 2. U and E, urea and serum calcium – deposition and damage in kidneys 3. Serum immunoglobulins and urine for BFP (Bens jones) 4. Bone marrow examination (>10% plasma cells) = myeloma definition 5. Skeletal survey (or MRI scan) – osteolytic bone lesions in myeloma

Question 37

Myelomatosis: Blood Films show

Answer 37

Blood film showing rouleaux in myeloma | Stick blood – stacked together like coins: not diagnostic for myeloma

Question 38

Myelomatosis: Identification of monoclonal immunoglobulin

Answer 38

Serum protein electrophoresis

Question 39

Myelomatosis: Treatment general

Answer 39

* Prompt treatment of infections * High oral fluid intake – support kidneys * Alllopurinol – prepare kidneys for onslaught of malignant cell breakdown * Bisphosphonate therapy (e.g. sodium clondrinate) * Plasmapheresis – hypoerviscosity * Management of renal failure and hypercalcaemia in acute situations

Question 40

Myelomatosis: Treatment Specific

Answer 40

* Melphalan/prednisoone/thalidomide for elderly ptients * Cyclosphosphamide/thalidomide/dexamethasone for patients <65 years followed by peripheral blood stem cell autograft * Velcade and Revlimid used following relapse * Local radiotherapy to site of bone pain/fractures

Question 41

Myelomatosis: Prognosis

Answer 41

``` Relapsing Remitting course Eventually dur resistance occurs and long term survival/cure is rare Mean survival: • 3-5 years in older patients • 5-7 years in younger group • (<65 yrs) intensively treated. ``` Low level paraproteins found in 3% of elderly individuals termed. MGUS (monoclonal gammaopathy of undetermined significane). 10% will progress to myeloma over 10 years follow-up.