Haematology Lymphoma Flashcards
Lymphoma types
40
3 types of lymphoma
Hodgkins lymphoma (15% of lymphoma)
Non-hodgkins lymphoma:
a. Low grade
b. High grade
Low grade examples
Follicular
Lymphoplasmacytoid
Marginal zone
High grade examples
Diffuse large cell lymphoma (most common) – 40%
Lymphoblastic lymphoma
Burkitts lymphoma
Hodgkins lymphoma: Epi
Male predominance
Incidence 6 per 100000
Peak incidence – young/adults
Hodgkins lymphoma:Association with
EBV infection
HIV infection
Hodgkins lymphoma: Clinical Features
Painless lymphadenopathy: contiguous spread.
Neck
Mediastinum
Axilla (usually above the neck)
Hodgkins lymphoma: B symptoms
Weight loss >10%
Sweats
Fever +33oC
Pruritis
Hodgkins lymphoma: ‘Patho-pneumonic’
Drink alcohol and lymph node hurts
Hodgkins lymphoma:Radiology can show
Hilar Lymphadenopathy
Differentials:
Sarcoid
Lymphoma
Hodgkins lymphoma: CT showing
Retoroperitoneal lymphadenopathy – enlarged lymphnodes in abdomen
Hodgkins lymphoma:Pet Scn
Glucose radioactive tracer. Assesses response
Hodgkins lymphoma: Histology
Lymphocyte poor
Nodular sclerosing
Mixed cellularity – slightly worse prognosis
Hodgkins lymphoma: Pathology characterised by
Presence of Reed-Sternberg cells
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
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
Hodgkins lymphoma: Treatment/Prognosis
Depends on stage
Hodgkins lymphoma: Stage 1A-2A: treatment/prognosis
3-4 cycles of chemotherapy (eg ABVD) followed by radiotherapy to affected nodal areas.
Cure 80-90%
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
Hodgkins lymphoma: ABVD combination and benefit
Adreiamycin
Bleomycin
Vinblastine
Dacarbazine
→ Fertility sparing
Hodgkins lymphoma: Late complications of therapy
- Inferility
- Radiation pneumonitis and pulmonary fibrosis
- Secondary cancers including acute myeloblastic leukaemia
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
Non-Hodgkins Lymphoma: Description
More commonly disseminated disease at multiple sites and extra – lymphatic infiltration
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.
Non-Hodgkins Lymphoma: Two main Groups
Low grade
High grade
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.
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)
Non-Hodgkins Lymphoma: Rituximab action
CD20 on the surface of B cells lymphocyte (B cells and T cells)
Non-Hodgkins Lymphoma: High Grade Lymphomas
Aggressive and rapidly progressive course if untreated.
With combination chemotherapy, however, 65%, longterm survivale/care.
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)
Non-Hodgkins Lymphoma: Autograft
Own stem cells (rescue side effects from chemo vs. allograft (15-20% chance of death)
Myelomatosis: Description
Malignant tumour involving proliferation of plasma cells which produce monoclonal immunoglobulin (paraprotein) and/or light chain (Bence Jones proteins)
Myelomatosis: Epi
Disease of late middle age and elderly
Myelomatosis: Clinical Features
- Marrow infiltration leading to anaemia
- Infections due to hypogammaglobuinaemia – redcued of autoantibodies
- Skeletal disease
→ Bone Pain
→ Pathological fracture
→ Hypercalcaemia - Renal Failure
- Peripheral neuropathy
- Hyper-viscosity syndrome
Calcium Renal failure Anaemia Bone pain → indication for treatment
Myelomatosis: Hyperviscosity Syndrome
Confusion
Chest Pain
Vision disturbance
Myelomatosis: Investigations
- Blood count, Very high ESR, plasma viscosity
- U and E, urea and serum calcium – deposition and damage in kidneys
- Serum immunoglobulins and urine for BFP (Bens jones)
- Bone marrow examination (>10% plasma cells) = myeloma definition
- Skeletal survey (or MRI scan) – osteolytic bone lesions in myeloma
Myelomatosis: Blood Films show
Blood film showing rouleaux in myeloma
Stick blood – stacked together like coins: not diagnostic for myeloma
Myelomatosis: Identification of monoclonal immunoglobulin
Serum protein electrophoresis
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
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
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.
