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Week 4 - Haematology > Lymphoma & Myeloma > Flashcards

Lymphoma & Myeloma Flashcards

1
Q

Define lymphoma [1]

A

tumour of the lymphoid tissue involving abnormal proliferation of B or T cells

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2
Q

What are the 2 main types of lymphoma? [2]

A
  1. Non-Hodgkin’s lymphoma
  2. Hodgkin’s lymphoma
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3
Q

How does lymphoma typically present? [9]

A
  1. Lymphadenopathy
    • Painless
    • Rubbery
  2. Splenomegaly
  3. B symptoms (similar to red flags in other conditions)
    • Night sweats
    • Weight loss
    • Unexplained fever
  4. Anaemia
    • Several causes
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4
Q

What investigations should be carried out on a patient with suspected lymphoma? [12]

A
  1. Blood Tests
    • FBC, U&Es, LFTs, Ca
    • Erythrocyte Sedimentation Rate (ESR)
      • Marker of inflammation
      • Used for diagnosis of Hodgkin’s lymphoma
  2. Lactate dehydrogenase (LDH)
    • Involved in tumour metabolism
  3. Imaging Tests
    • CT scan
    • PET scan
  4. Bone marrow biopsy
    • Aspiration biopsy = suck out liquid bone marrow up a syringe
    • Trephine biopsy = removal of 1 or 2cm core of bone marrow in one piece
  5. Additional tests (may be required pre-treatment)
    • Echocardiogram
    • Pulmonary function tests
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5
Q

Describe the Ann-Arbor Classification System for staging lymphoma [5]

A
  • Stage I: single lymph node group
  • Stage II: more than one lymph node group on the same side of the diaphragm
  • Stage III: lymph node groups on both sides of the diaphragm
  • Stage IV: extranodal involvement e.g. liver, bone marrow
  • A or B added after to signify absence or presence of B symptoms
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6
Q

What are the types of Non-Hodgkin’s lymphoma? [4]

A
  1. Follicular B-cell lymphoma (indolent)
  2. Diffuse Large B-cell lymphoma (aggressive)
  3. Burkitt’s lymphoma (aggressive)
  4. T cell lymphoma (rare - indolent or aggressive)
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7
Q

Describe Non-Hodgkin’s follicular lymphoma under the following headings:

  1. definition? [1]
  2. who gets it? [1]
  3. histological features? [2]
  4. genetic basis? [2]
  5. presentation? [2]
  6. prognosis? [2]
  7. treatment? [8]
A
  1. Definition
    • Low grade B cell lymphoma
  2. Who gets it?
    • Median age at diagnosis = 65 years
  3. Histological features
    • Resemblance to normal germinal centres
    • Slow growth but reduced cell death
  4. Genetic basis
    • Characterised by translocations involving BCL2 gene
    • T14;18 translocation
  5. Presentation
    • Often presents with stage 4 disease
    • B symptoms less common
  6. Prognosis
    • Indolent clinical course (which means that it persists/fails to heal)
    • Usually incurable
  7. Treatment
    • Aimed at alleviating symptoms
    • Not all patients require treatment at diagnosis
    • Early stage (1A and some 2A) → localised radiotherapy (possibly curable?)
    • Advanced stage
      • If asymptomatic, no bulk, no end organ compromise → watch and wait
      • If symptomatic and/or organ compromise → treatment with immunochemotherapy
        • Rituximab = anti-CD20 monoclonal antibody
        • Chemotherapy = CVP or CHOP or bendamustine*
        • Followed by maintenance rituximab every 2 months for 2 years
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8
Q

Describe Non-Hodgkin’s diffuse large B-cell lymphoma under the following headings:

  1. definition? [1]
  2. who gets it? [2]
  3. histological features? [3]
  4. genetic basis? [2]
  5. presentation? [10]
  6. prognosis? [2]
  7. treatment? [4]
A
  1. Definition
    • High grade B cell lymphoma
  2. Who gets it?
    • Mostly adults but wide age range and can occur in children
    • Incidence increases with age (>60yrs)
  3. Histological features
    • Resemblance to activated B-cells (immunoblasts, centroblasts)
    • High proliferation fraction
    • Variable rate of cell death
  4. Genetic basis
    • Associated with various translocations and genetic abnormalities
    • Complex karyotype
  5. Presentation
    • Heterogeneous entity = i.e. variable phenotype
    • Lymphadenopathy - usually rapidly enlarging lymph node mass
    • Extra-nodal presentation is common (30-40%)
      • Waldeyer’s ring
      • GI system
      • Skin
      • Bone
      • CNS etc.
    • B-symptoms
      • PUO - pyrexia (fever) of unknown origin
      • Night sweats & weight loss
  6. Prognosis
    • Aggressive but curable in >50%
  7. Treatment
    • Requires aggressive chemotherapy with intention to cure (response is variable)
    • Early stage (1A) = R-CHOP x 3 + radiotherapy
    • All other stages = R-CHOP x6
    • Elderly/unfit patients - need assessment of general frailty and comorbidities
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9
Q

What are the drugs that make up R-CHOP chemotherapy? [5]

A
  1. R = rituximab
  2. C = cyclophosphamide
  3. H = Adriamycin (doxorubicin)
  4. O = vincristine
  5. P = prednisolone
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10
Q

Describe Non-Hodgkin’s Burkitt lymphoma under the following headings:

  1. definition? [1]
  2. histological features? [5]
  3. genetic basis? [1]
  4. presentation? [13]
  5. prognosis? [2]
  6. treatment? [3]
A
  1. Definition
    • High grade B cell lymphoma
  2. Histological features
    • Resemblance to proliferating geminal centre cells
    • Very high proliferation rate (nearly all cells are in the cell cycle)
      • Due to MYC translocation and over-expression
    • High rate of cell death (apoptosis)
      • Can lead to tumour lysis syndrome
  3. Genetic basis
    • Characterised by translocations involving MYC gene (but simple karyotype)
  4. Presentation
    • Usually short history
    • Marked B symptoms
    • Rapidly growing tumours with massive tumour bulk
    • Most cases present with extranodal disease:
      • Jaws and facial bone (endemic BL)
      • Ileocaecal region of GIT - often primary site)
      • Ovaries
      • Kidneys
      • Breast
      • Lymph nodes and bone marrow
        • More frequently affected in immunosuppression-associated BL
      • CNS involvement at presentation or relapse is common
        • Brain, meninges or both
  5. Prognosis
    • Short survival rate unless treated
    • Responds well to chemotherapy
  6. Treatment
    • Requires intensive chemotherapy (CODOX-M/IVAC)
    • Aim of treatment is to cure - 70-90% long term survival rate
    • Elderly tend to have poorer outcomes as they are unable to tolerate intensive therapy
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11
Q

Describe the classic Hodgkin’s lymphoma under the following headings:

  1. definition? [1]
  2. who gets it? [2]
  3. histological features? [2]
  4. associated conditions? [1]
  5. presentation? [8]
  6. spread? [2]
  7. treatment? [4]
A
  1. Definition
    • High grade lymphoma with prominent component of reactive cells
  2. Who gets it?
    • Bimodal age incidence
    • Peak in 20-30’s then later peak (>50 years)
  3. Histological features
    • Neoplastic cell resembles atypical activated B cell as is seen in some viral infections (e.g. EBV)
    • Characterised by strong expression of CD30 and loss of some B-cell antigen
      • CD30 = cell membrane protein of the tumour necrosis factor receptor family and tumour marker
  4. Associated conditions
    • EBV infection (40% of cases)
  5. Presentation
    • Usually presents with painless lymphadenopathy (lymph node enlargement)
      • Neck lump
      • Cough
      • Shortness of breath
    • May have B symptoms
    • May present with chest x-ray mass
    • Itch may precede diagnosis for many months
    • Alcohol related pain - very rare
  6. Spread
    • Spreads from one nodal group to immediately adjacent nodes
    • Later, haematogenous spread to liver, lung and BM
  7. Treatment
    • Very effective with high cure rates
    • Early stage -> usually combined modality Rx, i.e. chemotherapy followed by radiotherapy
    • Advanced stage –> chemotherapy
    • Late effects are important, e.g. malignancy, cardiac, pulmonary, fertility, endocrine
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12
Q

What are the typical symptoms of hypercalcaemia? [5]

A
  1. stones,
  2. bone pain,
  3. depression,
  4. polyuria,
  5. nausea
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13
Q

Describe the classic triad of plasma cell (multiple) myeloma? [3]

A
  1. Increased plasma cells in bone marrow
  2. Clonal immunoglobulin or paraprotein
    • in most myeloma patients, abnormal plasma cells produce an abnormal “monoclonal protein” called a paraprotein or “M protein” of which there are 5 different types (IgG, A, M, D, E)
    • Usually IgG or IgA paraprotein are produced
    • Sometimes only part of the Ig molecule is produced - this is called “light chain myeloma”
    • Rarely, no Ig is produced - this is called “non-secretory myeloma”
  3. Lytic bone lesions
    • Caused by plasma cells producing osteoclast activating factor (OAF) which alters bone turnover resulting in osteolytic lesions, fractures, pain and hypercalcaemia
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14
Q

What are the biomarkers of malignancy in plasma cell myeloma? [3]

A
  • Clonal plasma cell percentage ≥60%
  • Serum free light chain ratio ≥100
  • >1 focal lesion on MRI
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15
Q

The CRAB criteria is used to determine whether treatment is required in multiple myeloma. What is the CRAB criteria? [4]

A
  1. Calcium elevation
  2. Renal dysfunction
  3. Anaemia
    • Hb < 100g/L or 2g < normal
  4. Bone disease
    • Lytic lesions or osteoporosis
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16
Q

What are the treatment options for multiple myeloma? [8]

A
  1. All patients receive chemotherapy usually including a steroid and thalidomide
  2. Radiotherapy — e.g. severe bone pain
  3. Supportive therapy
    • bisphosphonates
    • blood transfusion/EPO
    • surgery
    • interventional radiology
17
Q

What are the advantages of using bisphosphonates in the treatment of multiple myeloma? [4]

A
  1. reduce pain
  2. reduce pathological fractures
  3. reduce hypercalcaemia
  4. reduce need for radiotherapy

Week 4 - Haematology (9 decks)

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