Haem/onc for long case Flashcards
Palliative care related social needs
- Finances not working and access – not being able to drive to chemist, unable to afford medications
- Social isolation – impact on pain and mood overall adherence to therapy, increased risk of readmission
- Drug addicted son – Risk of opioid misuse, disruption of family dynamic, stress on patient and attending son’s care needs, risk of palliate care service seeing patient
- Lack of GP notice – out of loop, may be reluctant to prescribe opioid at doses without proper correspondence
- Driving – a stable dose of opioids given alertness and retained cognition is not a contraindication to drive, but at the discretion of state and territory jurisdiction and GP concerns
Management of Chronic Myelogenous Leukaemia
1) WCCs, bone marrow biopsy (bone marrow hyperplasia), cytogenetic karyotype testing (BCR-ABL t(9:22) philedelphia chromosome)
2) stabilise cell counts with hydroxyurea, prevent secondary gout with hydroxyurea
3) Imatinib - tyrosine kinase inhibitor - monitor with FBE, bone marrow 3 monthly, BCR-ABL PCR
- dasatinib/nilotinib second line if not responsive to imatinib
4) Allograft bone marrow transplant in blast phase not responsive to imatinib
Management of Acute myeloid leukaemia
1) Stratify risk - FBE, coag profile
- Bone marrow biopsy >20% blast cells
- Immunophenotyping - PML-RARA transcript, CBF leukaemia,
- Karyotyping -t(15:17), t(8:12), inv 6 (good prognosis), del 7 or complex karyotypes Poor prognosis) - APML - evidence of DIC
2) Chemotherapy for pt <70 and good performance state
- ICE (idarubicin, cytarabine, etoposide) or fludarabine, cytatabine combinations
3) Allograft stem cell transplant - poor risk and/or good performance status
- Poor prognosis factors (secondary leukaemia, poor performance status, Age > 60, Adverse cytogenetics)
4) acute promyelocytic leukaemia treatment
- All-trans-rentinoic acid and idarubicin
- ATRA, methotrexate and 6-mercaptopurse
Management of Acute lymphoblastic leukaemia
1) chemotherapy - prednisolone, combination chemotherapy, CNS prophylaxis with methotrexate and cranial irradiation
2) Allogenic stem cell transplant
3) poor cytogenetic markers include t(9:22) and t(4:11)
Chronic lymphocytic leukaemia management
1) Stage and prognosticate
- Lymph node count - Stage A <2, stage B>2, stage C - anaemia or thrombocytopaenia
- Indications for therapy: B symptoms, bulky disease/advanced stage, autoimmune phenomena (autoimmune haemolytic anaemia), rapid doubling time <6 months, young age < 40, bone marrow failure
- Cytogenetic and immunophenotyping - CD38, trisomy 12, 11q, del 17p unmutated IgH, ZAP70 (all poor prognosis)
2) Chemotherapy < 70 with good prognosis
- fludarabine/cyclophosphamide and rituximab
- Relapse: alemtuzumab
3) Chemo >70 years and/or poor prognosis
- fludarabine or chlorambucil and rituximab
4) < 50yrs old - allogenic stem cell transplant
Management of Hodgkin’s lymphoma
1) Early stage disease (AA stage - I, IB, IIA, non-bulky disease)
- Good prognostic markers - doxorubicin, bleomycin, vinblastine dacarbazine (ABVD) for 4 months, (> 6 months for poor prognosis)
- Consider omitting bleomycin if DLCO is reduced or consider Mechlorethamine, vincristine, prednisolone, procarbazine (MOPP)
2) Advanced disease (AA stage bulky disease, IIB, III, IV)
- Good prognosis (IPS <4) - 6 months of ABVD followed by radiotherapy to bulky sites
- Poor prognosis (IPS >4) - BEACOP for < 60yrs - bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, prednisolone, procarbazine), ABVD for age> 60yrs
3) Monitor for risk of AML and NHL after 10yrs and solid tumours after 15yrs
Prognostication of Hodgkin’s lymphoma
Serum alb <40
Hb <10.5
Male
Age >45yrs
Stage IV
WCC >15
Lymphocytes <0.6
Prognosis of non-Hodgkin’s lymphoma
Age >60 years
Stage III or IV
LDH >10
Extranodal spread
Poor performace status 2-4
NHLs and treatment
1) Diffuse B cell lymphoma - RCHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone); intrathecal methotrexate if CNS involvement
- Relapse - fludarabine followed by allogenic stem cell transplant, hypomethylating agents, monoclonal antibodies
2) Mantle cell lymphoma - low grade but poor prognosis - R-hyper CVAD (MDACC), R- CHOP, FCR - followed by allogenic stem cell transplant
3) Burkitt’s and lymphoblastic lymphoma - managed as ALL
4) T cell lymphoma - hyperCVAD, allogenic stem cell transplant
5) Follicular lymphoma (indolent) - treat as per CLL
Secondary causes of NHLs
Hepatitis C
HIV and immunosuppression
Sjogrens
Radiation exposure
Coeliac’s disease - gastrointestinal lymphoma
Helicobacter pylori - MALT lymphoma
Examination for haematological malignancy
Plethoric facies, hypertension,
Conjunctival pallor, mucosal bleeding
gum hypertrophy (AML)
Bony tenderness (MM, ALL)
Lymphadenopathy (epitrochlear, axillary, cervical, inguinal)
Hepatosplenomegaly (CML, AML, polycythemia vera)
Arthritis (gout), digital infarcts (erythromelalgia)
Fundus for retinal venous engorgement (PV), retinal ischaemia (hyperleukocytosis syndrome in AML)
Retinal ischaemia, neurological deficit, respiratory distress (hyperleucocytosis syndrome)
Cirrhosis (Budd-Chiari in PV)
Investigation of polycythemia
History of - headache, vertigo, tinnitus, blurred vision, pruritis, tingling in extremities in heat, previous stroke (hyperviscosity syndrome)
Investigation - FBE, red cell mass to exclude erythocytosis in setting of low plasma volume
EPO >4 secondary polycythemia, <4 primary
oxygen sats overnight - exclude sleep apnoea
High uric acid, low iron and raised B12 supports polycythemia vera
Abdo uss - exclude erythropoietin secreting mass lesions
Bone marrow - myeloid cell hyperplasia
JAK2 2 gene mutation 95% sensitivity for PCV
Management of PCV
Raised haematocrit controlled by venesection
Low dose aspirin for thrombosis
Chemotherapy busulfan or P32 for resistant cases
Erythromelalgia successfully managed with salicylate therapy
Hyperuricaemia is managed with allopurinol
Massive splenomegaly with splenectomy
Ann Arbor staging for Hodgkin’s lymphoma
Stage 1 - Single lymph node region
Stage 2 - 2 or more lymph node regions located on same side of diaphragm
Stage 3 - 2 or more lymph node regions on both sides of diaphragm
Stage 4 disseminated disease
B symptoms - night sweats, fevers, weight loss >10% of body in 6 months
History for cancer patient
Initial presenting symptoms
Diagnostic tests carried out
Treatment received so far
Exposure to carcinogens
Past medical history (UC w/ colonic carcinoma, H pylori w/ MALT, HL for AML, CML patients, cirrhosis for hepatic carcinoma, HPV infection for cervical and head and neck Ca)
Exposure to immunosuppressive therapies, chemotherapy, radiation exposure, hormonal treatment
Family history
Social - smoking, alcohol, sexual activity, sun exposure, asbestos
Type and regime of chemotherapy an radiotherapy
Side effects such as nausea, vomiting and oral mucositis, hair loss, sepsis, organ specific - cardiac toxicity, pulmonary toxicity, peripheral neuropathy, bladder toxicity)
Sperm and ova harvesting
Depression
Examination for cancer patients
General - cachexia, alopecia, cushingoid body habitus, vascular access devices, flat affect
Skin - radiation dermatitis, radiation burns, surgical scars, radiotherapy tattoos
Buccal mucosa for pigmented lesions - Peutz-Jegher’s syndrome - colorectal Ca
Organomegaly, hepatomegaly, splenomegaly, bony tenderness
Lymphadenopathy
Define mass lesions by quoting measurements, consistency, fixation/mobility, shape and temp
Evidence of pulmonary toxicity (cyclophosphamide, bisulphan, bleomycin, radiotherapy)
Neurological examination (exclude cord compression, cerebral mets, paraneoplastic phenomenan neurotoxic phenomena)
Evidence of heart failure (treatment with anthracyclines)
Management of Breast Cancer
1) screening - ages 50 to 70 mammogram 2 years
2) mass discovery - FNA or core biopsy; need to check for axillary lymphadenopathy and hepatomegaly; LFTs (raised ALP) and X ray, uss or CT CAP +/- bone scan
3) Early stage disease
- Resection and breast conserving surgery and radiotherapy or modified radical mastectomy
- Adjuvant chemotherapy (hormonal and/or monoclonal antibody depending on hormone receptor positivity, HER2 receptor positivity, menopausal state)
4) Advanced stage disease
- Induction chemotherapy
- Regional therapy with surgery and/or radiotherapy
- oophorectomy or chemical castration with LH in younger hormone receptor positive patients
- Older patient hormone receptor positive - tamoxifen, aromatase inhibitor or single agent chemotherapy
Adjuvant chemotherapy early stage in breast cancer
1) Hormone receptor positive
- Premenopausal - Chemotherapy with taxanes and anthracyclines - Ovarian ablation and tamoxifen for 5 years
- Post menopausal - Aromatase inhibitors and chemotherapy; regular follow up with yearly mammogram
2) Hormone receptor negative
- Chemotherapy +/- radiotherapy
- HER2 receptor positive patients can undergo treatment with trastuzumab
Identifying cancers of unknown primary
1) immunoperoxidase tumour markers
- Mesenchymal cancer - vimentin
- rhabdomyosarcoma - desmin
- breast ca - oestrogen or progesterone
- prostate ca - PSA
- Malignant melanoma - S-100, HMB45
- Squamous cell carcinoma - cytokeratin
2) serum tumour markers
- Testicular - AFP, bHCG
- cholangiocarcinoma - CA19-9
- Ovarian CA125
Management of prostate cancer
1) Early disease
- Radical prostatectomy - complications - impotence (90% patients), urinary incontinence, rectovesical fistula formation
- Radiotherapy - external beam or brachytherapy - bladder irradiation, proctitis, impotence, diarrhoea, dysuria, severe perineal cutaneous reaction
- Surveillence
2) Advanced disease
- Hormonal caustration - for those not suitable for surgery and those with metastatic disease - total androgen blockage wtih goserelin/luprelide with anti-androgen flutamide to prevent paradoxical worsening of bony pain; 3 year expectancy
- Hormonal therapy refractory - antiandrogen withdrawal, antifungal agent ketoconazole, corticosteroids, docetaxel
- Radiotherapy for acute bony pain
Management of non-small cell carcinoma of the lung
1) Non-small-cell lung cancer
- Stages I and II (ipsilateral peribronchial or hilar lymph nodes) - lobectomy; 3 monthly follow up for 2 years, 6 monthly for 5 years
- Stage IIIA (mediastinal or subcarinal lymph node involvement or spread to pleura, pericardium or chest wall) - surgery, radiotherapy and chemotherapy
- Stage IIIB - locally advanced disease - radiotherapy and chemotherapy
- Stage IV - palliation, double or single agent cisplatin or another agent
Chemotherapy includes - cisplatin, taxanes, gemcitabine, vinorelbine, camphothecin
Management of small cell lung cancer
1) Limited disease (limited to single radiation field)
- platinum based etoposide - life expectancy 18months
- mediastinal radiotherapy and prophylactic cranial irradiation
2) Extensive disease
- gentle chemotherapy
- palliation
Paraneoplastic syndrome, LAMS, SIADH, cushing’s syndrome
Management of colorectal Ca
Investigations - ALP, CTCAP colonoscopy/sigmoidoscopy, transrectal ultrasound, MRI and PET
Follow up colonoscopy 1 month following stenosed carcinoma to look for synchronous tumours; 1 yearly colonoscopies following
Dukes A and B - mucosa/submucosa or muscularis- surgery
Dukes C - lymph node involvement - adjuvant chemotherapy with 5-fluorouracil and leucovorin and oxaliplatin
Dukes D - palliative surgery and/or chemotherapy
Rectal carcinoma may benefit from neo-adjuvant chemotherapy (prior to surgery)
Cancer of head and neck
Investigations include CT CAP, endoscopy, PET/MRI for TNM staging
Stage 1 and 2 - Radiotherapy and surgery depending on site and organ function
Stage 3 and 4 - chemo-radiotherapy with cisplatin and 5-FU
1) Cancers that metastasise to brain
2) Cancers that metastasise to bone
3) Cancers that cause DVT
1) Lung breast, kidney, melanoma, colon
2) Breast, lung, thyroid, kidney, prostate
3) Breast, lung, colon, pancreas
Presentations of Multiple Myeloma
Bony pain, pathological fractures
Symptomatic anaemia
Pancytopaenia
Sepsis
Renal failure
Acute symptomatic hypercalcaemia
Hyperviscosity (IgM)
Criteria for Multiple Myeloma
1) Major
2) Minor
1) >30% plasma cells in bone marrow, M band in SPEP (>3.5g/L IgG or >2g/L IgA), 1g/24hr of Bence Jones protein in urine, Plasmacytoma confirmed on Bx
2) 10-30% plasma cells in BM, M band and Bence-Jones at lower levels, Lytic lesions on skeletal survey, low immunoglobulins
1 major and 3 minor criteria present for Dx
Management of Multiple Myeloma
Treat acute complications - Hypercalcaemia (hydration, bisphosphonates), spinal cord compression (radioTx), renal failure (IV fluids, dialysis)
For patients < 70yrs and good prognosis - cytoreductive chemotherapy w/ VAD (vincristine, doxorubicin, dexamethasone)/stem cell collection/ and melphalan followed by stem cell rescue
Second ASCT if good partial response
Maintenance thalidomide +/- steroids if in remission
Thalidomide based therapy +/- Dexamethasone if not suitable
Second line therapy
- Thalidomide based therapy, Bortezomib, Lenalidomide
- Allograft Tx for suitable patients
Supportive therapy
- Bisphosphonates for pathological #
- transfusion
- IV immunoglobulin
- RadioTx for osteolytic lesions
Differential diagnosis for Multiple Myeloma
MGUS
Smouldering Myeloma
Solitary Plasmacytoma
Waldenstrom’s macroglobulinaemia
Primary or light chain amyloidosis
Lymphoma
CLL
Poor prognostic signs in Multiple Myeloma
Cytogenetic abnormalities del 13q, t(4:14), del 17p
Hb <8.5
Hypercalcaemia
High M protein
CRF
Low Alb
Elevated beta 2 microglobulin >5.5
High LDH
Age > 70
Poor performance status
Moderate increase in breast cancer 4%
Family history of breast cancer in 2 family members on same side AND/OR at least one age <50yrs
One family member with breast cancer age < 50yrs
Requires
Biennial referral to family cancer clinic
Annual mammograms, from 40 years if one family member with breast ca <50yrs.
High risk breast cancer
Need annual breast mammogram, MRI and/or USS, family cancer genetic clinic, consideration of chemo-preventative measures
Family member with known high risk gene mutation BRCA
Two first or second degree family members on the same side with breast or ovarian cancer and one or more of:
- additional relatives with breast or ovarian Ca
- breast cancer diagnosed before age 40 years
- bilateral breast cancer
- breast and ovarian cancer in the same woman
- Ashkenazi Jewish ancestry
- breast cancer in male relative
One relative (first or second degree) with breast Ca <45 and another with a sarcoma diagnosis
