Haematology - Lymphomas and Multiple Myeloma Flashcards
Outline a classification for lymphomas
What are lymphomas? Name an important virus that has been linked to lymphoma
Lymphomas: group of disease caused by malignant lymphocytes that accumulate in LN and cause characteristic clinical features of lymphadenopathy. Can occasional spill into blood (leukemic phase) or infiltrate organs outside lymphoid tissue. Major division is:
- Hodgkin: Reed-Sternberg cells (B lymphoid lineage)
- Non-Hodgkin: everything else
*EBV genome has been detected in over 50% of cases in Hodgkin tissue but role in pathogenesis is unclear
Hodgkin Lymphoma: clinical features
- Peak incidence: young adults (but can present at any age) M(2):F(1) ratio
- Lymphadenopathy: neck, axilla, inguinal region – non-tender and rubbery
- B symptoms: fever (38), weight loss, night sweats
- Other: fatigue, itching, cough, SoB, abdo pain, recurrent infections
Name the types of hodgkin lymphoma
Name some investigations you should perform on someone with suspected Hodgkin lymphoma
- Excisional LB biopsy (do NOT do FNA) – histological classification (immunohistochemistry) and TB testing – will show Reed-Sternberg cells
- Bloods: FBC with differential (normochromic normocytic anaemia), LDH (raised), LFT, U+Es, urate levels, Bone profile, serum Ig, CRP
- HIV/HEP screen
- CT CAP
- CXR
- PET CT: increased FDG uptake
- BM examination (biopsy and trephine)
How do you stage hodgkin lymphoma?
Ann Arbour Classification
What do these images show?
What is the treatment for hodgkin lymphoma?
- Standard treatment: chemotherapy (ABVD) +/- Rt to target bulky disease
- Issues to consider before starting treatment: future fertility, cardiac and kidney function, pulmonary fibrosis (Bleomycin), effect on work/study and financial implications
What is NHL?
- Large group of clonal lymphoid tumours in which lymph nodes, spleen or other solid organs are involved. (Leukaemias tend to be predominantly in BM and circulating tumour cells).
- Characterised by irregular pattern of spread and significant proportion of patients develop extra-nodal disease.
- The lymphomas are classified within a group of mature B cell and T cell neoplasms, which includes some chronic leukaemias and myeloma.
NHL: Clinical features
- Superficial lymphadenopathy: asymmetric, painless enlargement of LNS in one or more peripheral LN regions
- Constitutional sx: fever, night sweats, wt loss (less frequent than Hodgkin and associated with disseminated disease)
- Oropharyngeal involvement
- Symptoms due to anaemia/neutropenia or thrombocytopenia
- Abdominal disease: liver and spleen are often enlarged
NHL: what investigations should you do?
- Excisional LB biopsy (do NOT do FNA) – histological classification (immunohistochemistry) and TB testing – will show Reed-Sternberg cells
- Bloods: FBC with differential (normochromic normocytic anaemia), neutropenia, thrombocytopenia, LDH (raised), LFT, U+Es, urate levels, Bone profile, serum Ig (paraprotein), CRP
- HIV/HEP screen
- CT CAP
- CXR
- PET CT
- BM examination (biopsy and trephine)
NHL: treatment options and how do you monitor response to treatment?
- Low grade: watch and wait (clinical examinations every 3-6/12)
- Triggers for treatment: B symptoms, stage III disease, lymphadenopathy with compression (oedema/SOB) or significant cytopenia
- Higher grade or Stages III-IV: Combined therapy: chemotherapy (eg R-CHOP) and RT
- Rituximab: binds to CB20 on the surface of B cells and causes antibody dependent cell-mediated cytotoxicity, complement mediated lysis of tumour cells and direct apoptosis of target cell
- Assessment of response to treatment: use CT and PET (good for distinguishing between metabolically active disease and fibrosed LNs)
- Prognosis: excellent and over 85% of patients can expect to be cured but treatment causes a long term morbidity burden
- Supportive treatment for acute hyperviscosity syndrome
What is multiple myeloma?
- Neoplastic disease characterised by plasma cell accumulation in BM, presence of monoclonal protein in the serum and/or urine and, in symptomatic patients, related tissue change.
- 90% of cases occur >40 years old with peak age in 70’s.
- Results from expansion of a single clone of immunoglobulin secreting terminally differentiated B cells.
Give some features which can be used to diagnosis myeloma
- Monoclonal protein in serum and/or urine
- Increased clonal plasma cells in BM
- Related organ or tissue impairment (CRAB – Hypercalcaemia, renal impairment, anaemia and bone disease)
What are the clinical features of myeloma?
- Bone pain (esp backache) resulting from vertebral collapse and pathological fractures
- Anaemia: lethargy, weakness, dyspnea, pallor and tachycardia
- Neutropenia: related to deficient antibody production
- Renal failure and/or Hypercalcaemia: polydipsia, polyuria, anorexia, vomiting, constipation and mental disturbance
- Abnormal bleeding: myeloma interferes with platelets
- Amyloidosis
- Hyperviscosity: headaches, strokes, etc
- Spinal cord compression
What investigations should you perform if you suspect myeloma?
- Presence of paraprotein: serum and urine immunoglobulin electrophoresis (IgG in 60% of cases)
- Elevated serum immunoglobulin-free light chains (Kappa or lambda)
- Urine: Bence-Jones protein (not really used anymore)
- Bloods: normochromic, normo or macrocytic anaemia, raised ESR, bone profile (raised serum Calcium), U+Es (raised serum creatinine), LFTs (low albumin in advanced disease)
- Blood Film: Rouleaux (stacking of red cells) caused by hyperviscosity of blood.
- MRI: bone lesions (osteolytic areas eg pepper-pot skull and pathological fractures)
*DO not use PET if suspect myeloma
Myeloma: outline specific and supportive treatment
Specific: mostly incurable but use intensive therapy (eg cytotoxic drugs) to reduce tumour burden, followed by autologous stem cell transplant
- Other: thalidomide, lenalidomide
Supportive:
- Renal failure: dialysis and address hyperkalaemia
- Bone disease and Hypercalcaemia: bisphosphonates
- Acute hypercalcaemia: rehydration (24h)+ diuretic + steroids + bisphosphonates
- Radiotherapy: very effective in treating symptoms of myeloma – bone pain or spinal cord compression
- Thromboprophylaxis
