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Flashcards in Haematology Lymphoma Deck (41):
1

Lymphoma types

40

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3 types of lymphoma

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

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Low grade examples

Follicular
Lymphoplasmacytoid
Marginal zone

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High grade examples

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

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Hodgkins lymphoma: Epi

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

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Hodgkins lymphoma:Association with

EBV infection
HIV infection

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Hodgkins lymphoma: Clinical Features

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

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Hodgkins lymphoma: B symptoms

Weight loss >10%
Sweats
Fever +33oC
Pruritis

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Hodgkins lymphoma: ‘Patho-pneumonic’

Drink alcohol and lymph node hurts

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Hodgkins lymphoma:Radiology can show

Hilar Lymphadenopathy
Differentials:
Sarcoid
Lymphoma

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Hodgkins lymphoma: CT showing

Retoroperitoneal lymphadenopathy – enlarged lymphnodes in abdomen

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Hodgkins lymphoma:Pet Scn

Glucose radioactive tracer. Assesses response

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Hodgkins lymphoma: Histology

Lymphocyte poor
Nodular sclerosing
Mixed cellularity – slightly worse prognosis

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Hodgkins lymphoma: Pathology characterised by

Presence of Reed-Sternberg cells

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Hodgkins lymphoma: Investigation

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

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Hodgkins lymphoma: Clinical Staging



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

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Hodgkins lymphoma: Treatment/Prognosis

Depends on stage

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

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

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

Combination chemotherapy alone (e.g. 6-8 cycles of ABVD)
Cure 60-80% (in young adults – worse inelderly +600

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Hodgkins lymphoma: ABVD combination and benefit

Adreiamycin
Bleomycin
Vinblastine
Dacarbazine

→ Fertility sparing

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Hodgkins lymphoma: Late complications of therapy

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

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Non-Hodgkins Lymphoma:Epi

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

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Non-Hodgkins Lymphoma: Description

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

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Non-Hodgkins Lymphoma: May be reflected by

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

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Non-Hodgkins Lymphoma: Two main Groups

Low grade
High grade

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Non-Hodgkins Lymphoma: Low grade lymphomas

• 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.

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Non-Hodgkins Lymphoma: Low grade lymphoma Treatment

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

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Non-Hodgkins Lymphoma: Rituximab action

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

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Non-Hodgkins Lymphoma: High Grade Lymphomas

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

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Non-Hodgkins Lymphoma: High grade lymphoma treatment

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)

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Non-Hodgkins Lymphoma: Autograft

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

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Myelomatosis: Description

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

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Myelomatosis: Epi

Disease of late middle age and elderly

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Myelomatosis: Clinical Features

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

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Myelomatosis: Hyperviscosity Syndrome

Confusion
Chest Pain
Vision disturbance

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Myelomatosis: Investigations

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

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Myelomatosis: Blood Films show

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

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Myelomatosis: Identification of monoclonal immunoglobulin

Serum protein electrophoresis

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Myelomatosis: Treatment general

• 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

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Myelomatosis: Treatment Specific

• 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

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Myelomatosis: Prognosis

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.

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