Haem cancers Flashcards
Define myeloma
Malignant proliferation of plasma cells in the bone marrow. Increased Ig secretion and lytic bone lesions
Pathology of myeloma
- Increased osteoclast activity (next to myeloma cells) - lytic bone lesions and hypercalcaemia
- Ig secretion - renal problems and hyperviscosity
Clinical features of myeloma
CRAB
- Calcium (hypercalcaemia) - stones, groans, bones and psychic groans, thirst constipation, nausea
- Renal impairment - oedema, decreased UO, dehydration
- Anaemia + neutropaenia + thrombocytopaenia - recurrent bacterial infection
- Bone disease - osteolytic bone lesions (backache, fractures), pepperpot skull, OP
Investigations for myeloma
- Bloods - reduced Hb, WCC, increased Ca, U&E, ESR
- Xray (first line) - pepperpot skull, vertebral collapse, lytic punched out lesions
- Serum protein electrophoresis - Ig monoclonal band
- Serum urine electrophoresis - Bence-Jones’ protein
- Bone marrow biopsy - increased plasma cells
- Blood film - rouleaux stacks
Management of myeloma
- Chemo
- CTD (8 cycles) - cyclophosphamide, thalidomide, dexamethasone
- VAD (6 cycles) - velcade, adriamycine, dexamethasone
Management of myeloma complications
- Hypercalcaemia - IV fluids
- AKI - fluids/dialysis
- Hyperviscosity - plasmapheresis
- Spinal cord compression - MRI, dexamethasone
Supportive treatment of myeloma
- Anaemia - transfusion
- Bone pain - analgesia + bisphosphonates
- Infections - Abx, regular Ig infusions
Define Hodgkin’s lymphoma
Malignant proliferation of mature lymphocytes in the lymphatic system. Reed-Stenberg cells.
Epidemiology of Hodgkin’s lymphoma
Peaks in young adults and the elderly
Causes of Hodgkin’s lymphoma
- Primary + secondary immunodeficiency
- Autoimmune
- Infection - EBV
- Obesity, FHx
Staging (Ann Harbour) of Hodgkin’s lymphoma
- Single node or site
- > 2 nodes on the same side of diaphragm
- > 2 nodes on both sides of diaphragm
- Disseminated/diffuse
‘A’ symptoms of Hodgkin’s lymphoma
Enlarged, painless, non-tender rubbery lymph nodes. Worsen with alcohol (can become painful)
B symptoms of Hodgkin’s lymphoma
- Fever
- Night sweats
- Weight loss
- Lethargy/malaise
B symptoms have worse prognosis
Management of Hodgkin’s lymphoma
- 1-2a = brief chemo/radiotherapy
- 2b-4 = longer cycles of chemo
- BMT for relapse
Signs of Hodgkin’s lymphoma
- Anaemia
- Hepatosplenomegaly
- Lymphadenopathy
Investigations of Hodgkin’s lymphoma
- Lymph node biopsy - Reed-Stenberg cells
- FBC
- CT chest, abdo, pelvis for staging
Define non-hodgkin’s lymphoma
Any lymphoma that isn’t hodgkin’s lymphoma
Epidemiology of non-hodgkin’s lymphoma
> 40y
Types of non-hodgkin’s lymphoma
- Low grade : follicular
- High grade : diffuse large cell B lymphoma
- Very high grade : Burkitt’s lymphoma
Pathology of non-hodgkin’s lymphoma
70% B cell. Clonal expansion of lymphocytes
Signs and symptoms of non-hodgkin’s lymphoma
Similar to hodgkin’s lymphoma
- GI involvement (if small bowel lymphoma) - abdo pain, diarrhoea, vomiting
- Skin involvement if T cell lymphoma
Investigations for non-hodgkin’s lymphoma
- Lymph node biopsy
- CT
- FBC
Management of non-hodgkin’s lymphoma
Same as hodgkin’s lymphoma + rituximab + steroids
Define leukaemia
All cancers of white blood cells
4 types of leukaemia
- Acute myeloid leukaemia
- Acute lymphoblastic leukaemia
- Chronic myeloid leukaemia
- Chronic lymphocytic leukaemia
Define ALL
Acute malignant transformation of lymphocytic blast cells (no maturation at all). More common in children.
Pathology of ALL
Uncontrolled proliferation of T/B cells -> takes up space in bone marrow (anaemia, thrombocytopaenia, neutropaenia) + blasts in the peripheral blood and aggregation in liver, spleen, lymph nodes and thymus (T cells)
Causes of ALL
- Philadelphia chromosome t(9:22) translocation
2. 20x higher risk with Down’s syndrome
Symptoms of ALL
- Fatigue
- Bleeding
- Palpitations
- Infections
- Bone pain
- Abdominal fullness (from hepatosplenomegaly)
Signs of ALL
- Hepatosplenomegaly
- Anaemia
- Cranial nerve palsies
- Lymphadenopathy
Investigation of ALL
- Blood smear - blast cells (small)
- Bone marrow biopsy
- Lumbar puncture - CNS involvement
- FBC - low RBC, PLT
- Clotting screen - DIC possibility
Management of ALL
- Chemo - intrathecal (spine)
- Methotrexate
- Steroids for maintenance
- BMT - during first remission
- Supportive - IV fluids/blood/platelets
- Treat infection
Define AML
Acute malignant transformation of myeloid blast cells. More common in older adults/elderly
Causes of AML
- Transform from myelodysplasia
2. Genetic - chromosomal mutations
RF AML
- Radiation
2. Alkylating chemo
Symptoms of AML
- Fatigue
- Bleeding
- Palpitations
- Recurrent infections
Signs of AML
- Hepatosplenomegaly
- Anaemia
- Gum hypertrophy
Investigations for AML
- Blood smear - AUER RODS
- FBC - low RBC, PLT
- Bone marrow biopsy
- Clotting screening - DIC risk
Management of AML
- Chemo (with breaks to allow bone regeneration)
- Steroids - maintenance
- Supportive treatment - IV fluids/blood/platelets
- Treat infection
Define CLL
Chronic lymphocytic leukaemia - chronic malignant transformation of mature lymphocytes that have escaped apoptosis. Most common leukaemia in the west
Pathology of CLL
Uncontrollable proliferation of T/B lymphocytes -> can lead to autoimmune haemolysis.
Signs and symptoms of CLL
- Often asymptomatic -> anaemia, bleeding, infection, sweats, anorexia
- Enlarged rubbery non-tender lymph nodes (lymphadenopathy)
- Hepatosplenomegaly
Investigations for CLL
- FBC: low RBC, high lymphocytes
- Blood smear - smudge cells
- Genetic testing (11q/17p)
Management of CLL
INCURABLE
- Chemo for advanced
- Radiotherapy can help lymphadenopathy + splenomegaly
- Rituximab
- Stem cell transplant
Define CML
Chronic myeloid leukaemia - chronic malignant proliferation of mature myeloid cells. The cells divide too quickly. More common in the elderly
Cause + progression of CML
1. Philadelphia gene -> BCR-ABL fusion gene -> tyrosine kinase activity affected Insidious onset (3-4y) -> blast formation -> AML -> rapid death
Symptoms of CML
- Fever
- Weight loss
- Sweating
- Tiredness
- Bleeding
Signs of CML
- Anaemia
- Bruising
- Infection
- Massive splenomegaly
Investigations of CML
- FBC
- Cytogenetics : karotype, FISH (fluorescent in situ hybridisation), PCR
- Blood smear - granulocytes
Management of CML
- 1st line: imatinib (tyrosine kinase inhibitor)
- In acute stage - chemo - hydroxycarbamide
- Stem cell transplant (only cure, significant mortality, younger patients)