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Kate Larard kml20@ic.ac.uk Slides adapted from Isobel Elliott BLOOD CANCERS MedEd Y3 Written Exam Lectures 2023SESSION STRUCTURE Aetiology RFs Presentation Investigations Management = Gold Standard = High YieldSESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMA SBA 1 (question) A 71-year-old man presents to the haematology clinic after having some blood tests at his general practice which showed a raised WCC. He has a 3-month history of cervical lymphadenopathy, but feels well and is otherwise asymptomatic. He has no organomegaly. What is the most likely diagnosis?mudge cells. a) Chronic myeloid leukaemia b) Acute myeloid leukaemia c) Chronic lymphocytic leukaemia d) Acute lymphoblastic leukaemia SBA 2 (question) A 52 year old male patient presents to the general practitioner with a 4 week history of weight loss and malaise. He also reports discomfort in the left upper abdomen and abdominal fullness. There is no past medical history of note. He denies any recent travel. On physical examination there is a mass arising from the left upper quadrant, extending to the right iliac fossa. A FBC showed a low Hb and raised WCC with a raised basophil count. Which of the following blood film findings is consistent with the most likely diagnosis? a) Mix of mature and immature myeloid cells b) Auer rod c) Smudge cells d) Poikilocytes e) Leukaemic lymphoblastic cells SBA 3 (question) A 2 year old girl is brought by her parents to the Accident and Emergency department. They say that she has been unwell for the last couple of days with a cough and a fever and they think she might have a chest infection. On examination she has conjunctival pallor, superficial lymphadenopathy and hepatosplenomegaly. Some bloods are ordered, which show anaemia, thrombocytopenia and neutropenia. She is diagnosed with a pneumonia. What is the most likely underlying diagnosis that put her at risk of developing this pneumonia? a) ALL b) CML c) AML d) CLL e) Myeloma SBA 4 (question) A 75-year-old man presents to his GP with a six week history of generalised weakness and fatigue. He also reports recurrent nosebleeds and bleeding gums. He has urgent blood tests which reveal anaemia, thrombocytopenia and leucocytosis. A diagnosis of leukaemia is suspected. Which of the following is a hallmark of acute myeloid leukaemia (AML)? A. Reed-Sternberg cells B. Auer rods C. Philadelphia chromosome D. Blast cells E. Rouleaux formationSESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMAMyeloid leukaemias Lymphoid leukaemiasLEUKAEMIAS BLOOD CANCERS THAT BEGIN WITH MUTATION OF A BM PROGENITOR CELL AND RESULT IN PRESENCE OF ABNORMAL CELLS IN THE BLOOD ACUTE CHRONIC Rapid and aggressive Slow and can go unnoticed for a while MYELOID LINEAGE LYMPHOID LINEAGEACUTE MYELOID LEUKAEMIA AETIOLOGY • RAPID proliferation of myeloblasts and presence of abnormal myeloblasts in the blood RFs: • Trisomy 21 • Irradiation CLINICAL FEATURES • B symptoms(fever, weight loss) • Featuresof BM failure • Neutropenia → infections • Anaemia → pallor • Thrombocytopenia → bleeding • Tissue infiltration • Swollen gums (monocytic) • Mild splenomegalyACUTE MYELOID LEUKAEMIA INVESTIGATIONS • FBC • High WCC (blasts), low plt, rbc • Blood film - Auerrod cells, blast cells • Bone marrow biopsy MANAGEMENT • Chemotherapy • Supportive • Blood products • Infection prophylaxis • Allopurinol, fluids, electrolytes • Prevent TLSACUTE LYMPHOBLASTIC LEUKAEMIA AETIOLOGY • Rapid proliferation of lymboblasts → out crowding and BM failure • Commonest cancer in children RFs: • Trisomy 21 • Neurofibromatosis CLINICALFEATURES • B symptoms (fever, night sweats, wt loss) • BMfailure • Neutropenia → infections • Anaemia → pallor • Thrombocytopenia → bleeding • Tissue infiltration • Lyphadenopathy • Hepatosplenomagaly • Tender bones • Swollen testesACUTE LYMPHOBLASTIC LEUKAEMIA INVESTIGATIONS • FBC • High WCC (blasts), lowplt, rbc • BM biopsy • >20% lymphoblasts MANAGEMENT • Chemotherapy • Supportive • Blood products • Infection prophylaxis • Allopurinol, fluids, electrolytes • Prevent TLS • Consider stem cell transplantCHRONIC MYELOID LEUKAEMIA AETIOLOGY Proliferation of myeloid stemcells results in increased production of ‘normalish’ myeloid downstream cells RFS • M>F 1.4:1 • 40-60Y Philadelphia Chromosome: t(9;22) BCR-ABL1 fusion gene (80%) CLINICAL FEATURES • 50% asymptomatic • Massive splenomegaly • B-symptoms • Symptoms of BM failure - Increased infections, Fatigue, bleedingCHRONIC MYELOID LEUKAEMIA INVESTIGATIONS • FBC: • pancytopenia, elevated WCC, raised basophil count (very few other causesof this) • BM aspirate: hypercellular with a spectrum of immature and mature myeloid cells • PCR for BCR-ABL1 fusion gene • FISH for BCR-ABL1 fusion gene MANAGAMENT • Tyrosine kinase inhibitors • Imatinib • 95% remission rate Chronic phase → accelerated phase → blast crisis • Stem Cell TransplantCHRONIC LYMPHOID LEUKAEMIA AETIOLOGY Failure of apoptosis resulting in increased number of lymphocytes that aren’t functional RFs: • Age 65-70 • M>F CLINICAL FEATURES: • May be asymptomatic • Symmetricalpainless lymphadenopathy • BM failure • Anaemia, thrombocytopenia, neutropenia • B-symptoms • Can progress to aggressive non hodgkins lymphoma (Richter’s transformation)CHRONIC LYMPHOID LEUKAEMIA Investigations • FBC – WWC ~100, normal Hb and Plt • Lowserumimmunoglobulin • Blood film • SMEAR/SMUDGE CELLS MANAGAMENT • Watchful waiting ifslowly progressive disease • Supportive treatment • RituximabLEUKAEMIA SUMMARY Acute Myeloid Leukaemia • Auer Rods Acute Lymphoblastic Leukaemia • Children <6 years Chronic Myeloid Leukaemia • Philadelphia Chromosome Chronic Lymphocytic Leukaemia • Smudge Cells SBA 1 (question) A 71-year-old man presents to the haematology clinic after having some blood tests at his general practice which showed a raised WCC. He has a 3-month history of cervical lymphadenopathy, but feels well and is otherwise asymptomatic. He has no organomegaly. What is the most likely diagnosis?mudge cells. a) Chronic myeloid leukaemia b) Acute myeloid leukaemia c) Chronic lymphocytic leukaemia d) Acute lymphoblastic leukaemia SBA 1 (answer) A 71-year-old man presents to the haematology clinic after having some blood tests at his general practice which showed a raised WCC. He has a 3-month history of cervical lymphadenopathy, but feels well and is otherwise asymptomatic. He has no organomegaly. What is the most likely diagnosis?mudge cells. a) Chronic myeloid leukaemia b) Acute myeloid leukaemia c) Chronic lymphocytic leukaemia d) Acute lymphoblastic leukaemia SBA 2 (question) A 52 year old male patient presents to the general practitioner with a 4 week history of weight loss and malaise. He also reports discomfort in the left upper abdomen and abdominal fullness. There is no past medical history of note. He denies any recent travel. On physical examination there is a mass arising from the left upper quadrant, extending to the right iliac fossa. A FBC showed a low Hb and raised WCC with a raised basophil count. Which of the following blood film findings is consistent with the most likely diagnosis? a) Mix of mature and immature myeloid cells b) Auer rod c) Smudge cells d) Poikilocytes e) Leukaemic lymphoblastic cells SBA 2 (answer) A 52 year old male patient presents to the general practitioner with a 4 week history of weight loss and malaise. He also reports discomfort in the left upper abdomen and abdominal fullness. There is no past medical history of note. He denies any recent travel. On physical examination there is a mass arising from the left upper quadrant, extending to the right iliac fossa. A FBC showed a low Hb and raised WCC with a raised basophil count. Which of the following blood film findings is consistent with the most likely diagnosis? a) Mix of mature and immature myeloid cells b) Auer rod c) Smudge cells d) Poilikocytes e) Leukaemic lymphoblastic cells SBA 3 (question) A 2 year old girl is brought by her parents to the Accident and Emergency department. They say that she has been unwell for the last couple of days with a cough and a fever and they think she might have a chest infection. On examination she has conjunctival pallor, superficial lymphadenopathy and hepatosplenomegaly. Some bloods are ordered, which show anaemia, thrombocytopenia and neutropenia. She is diagnosed with a pneumonia. What is the most likely underlying diagnosis that put her at risk of developing this pneumonia? a) ALL b) CML c) AML d) CLL e) Myeloma SBA 3 (answer) A 2 year old girl is brought by her parents to the Accident and Emergency department. They say that she has been unwell for the last couple of days with a cough and a fever and they think she might have a chest infection. On examination she has conjunctival pallor, superficial lymphadenopathy and hepatosplenomegaly. Some bloods are ordered, which show anaemia, thrombocytopenia and neutropenia. She is diagnosed with a pneumonia. What is the most likely underlying diagnosis that put her at risk of developing this pneumonia? a) ALL b) CML c) AML d) CLL e) Myeloma SBA 4 (question) A 75-year-old man presents to his GP with a six week history of generalised weakness and fatigue. He also reports recurrent nosebleeds and bleeding gums. He has urgent blood tests which reveal anaemia, thrombocytopenia and leucocytosis. A diagnosis of leukaemia is suspected. Which of the following is a hallmark of acute myeloid leukaemia (AML)? A. Reed-Sternberg cells B. Auer rods C. Philadelphia chromosome D. Blast cells E. Rouleaux formation SBA 4 (answer) A 75-year-old man presents to his GP with a six week history of generalised weakness and fatigue. He also reports recurrent nosebleeds and bleeding gums. He has urgent blood tests which reveal anaemia, thrombocytopenia and leucocytosis. A diagnosis of leukaemia is suspected. Which of the following is a hallmark of acute myeloid leukaemia (AML)? A. Reed-Sternberg cells B. Auer rods C. Philadelphia chromosome D. Blast cells E. Rouleaux formationSESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMA SBA 5 (question) A 25-year-old man has presented to his general practitioner after noticing a lump in his neck a few days ago. He is not sure how long this has been there. It is not currently painful but he reports it became painful when he went out for some alcoholic drinks with his friends last Saturday. He has anterior cervical chain. There is no evidence of any other lymphadenopathy.noted in the right Which of the following is the most likely diagnosis? A. Non-Hodgkin's lymphoma B. Metastatic lymph nodes C. Infectious mononucleosis D. Hodgkin's lymphoma E. Reactive lymphadenopathy T/B- cell lymphomasLYPHOMAS Definition: A group of blood cancers which develop from lymphocytes and the tumours are mainly found in the lymph nodes HODGKINS NON- HODGKINS (20%) (80%) • BURKITTS • DLYMPHOMA CELL • MANTLE CELL • FOLLLICULARHODGKINS LYPHOMA Risk Factors: • Bimodal age distribution – peaks between 20-30 and >60 • M>F • 50% associated with EBV Signs and Symptoms: • Lymphadenopathy - painful afteralcohol • Commonly mediastinal and cervical – contiguous spread • Firm and rubbery • B symptoms • splenomegaly +/- hepatomegalyHODGKINS LYPHOMA Investigations • L– mets)de excision (not lymph node aspirate • Reed-Sternberg cells Management • Chemotherapy • Radiotherapy • Stem cell transplant Prognosis excellent especially in young Binucleated ‘owl eyed’NON-HODGKINS LYPHOMA More common than Hodgkin’s Aggressive (High-Grade) Non-Hodgkin Lymphoma: 85% are B cell, 15%are T cellor NK cell • Diffuse Large B-Cell Lymphoma (DLBCL): • Burkitt Lymphoma: • Mantle Cell Lymphoma (MCL): • Anaplastic Large Cell Lymphoma (ALCL): • Enteropathy-associated intestinal T-cell lymphoma (EATL): celiac disease. Indolent (Low-Grade) Non-Hodgkin Lymphoma: • Follicular Lymphoma: • Marginal Zone B-cell Lymphoma: • Small Lymphocytic Lymphoma (SLL) • MALT Lymphoma: H PyloriNON-HODGKINS LYPHOMA Risk Factors: Depends on type eg. MALToma– H Pylori Incidence increases with age Signs and Symptoms: • Painless enlargingmass inneck, axilla or groin • B symptoms (less common than HL) • Organ involvement– skin rashes,headache, hepatosplenomegaly (more common than HL)NON-HODGKINS LYPHOMA Investigations • Lymph node excision (not lymph node aspirate – mets) • No Reed-Sternberg cells • Abnormal cells specific to type eg. DLBCL have CD20+ and CD79a+ B cells Management • Chemotherapy • Radiotherapy • Stem cell transplant Prognosis varies on subtype Binucleated ‘owl eyed’DIFFERENCES Hodgkin Non-Hodgkin Age Bimodal Elderly Lymph nodespread Contiguous Non-contiguous Histology Reed-Sternberg No Reed sternberg Symptoms Painful lymphadenopathy with alcohol Painless lymphadenopathyBURKITTS LYMPHOMA Risk Factors: • Strong association with EBV +/- HIV • t(8;14) translocationc-myc over expression Presentation: • Rapidly enlarginglymph node in the jaw Investigation: • Lymph node: Starry sky appearance under microscopyLYPHOMA STAGING Ann-Arbor A- without B symptoms B- with B symptoms SBA 5 (question) A 25-year-old man has presented to his general practitioner after noticing a lump in his neck a few days ago. He is not sure how long this has been there. It is not currently painful but he reports it became painful when he went out for some alcoholic drinks with his friends last Saturday. He has anterior cervical chain. There is no evidence of any other lymphadenopathy.noted in the right Which of the following is the most likely diagnosis? A. Non-Hodgkin's lymphoma B. Metastatic lymph nodes C. Infectious mononucleosis D. Hodgkin's lymphoma E. Reactive lymphadenopathy SBA 5 (answer) A 25-year-old man has presented to his general practitioner after noticing a lump in his neck a few days ago. He is not sure how long this has been there. It is not currently painful but he reports it became painful when he went out for some alcoholic drinks with his friends last Saturday. He has anterior cervical chain. There is no evidence of any other lymphadenopathy.noted in the right Which of the following is the most likely diagnosis? A. Non-Hodgkin's lymphoma B. Metastatic lymph nodes C. Infectious mononucleosis D. Hodgkin's lymphoma E. Reactive lymphadenopathy SBA 6 (question) A 45-year-old woman presents to her GP with fatigue, night sweats, and weight loss. On examination, she has palpable non-tender cervical lymphadenopathy. She is urgently referred to haematology, where a blood film confirms the presence of Reed-Sternberg cells. Computed tomography (CT) and positron emission tomography (PET) imaging reveal enlarged lymph nodes in the left axillary and bilateral cervical regions. In addition, disease is found in the left para-aortic lymph nodes. According to the Ann-Arbor staging system, what stage of Hodgkin's lymphoma is the patient suffering from? A. IB B. IIA C. IIIB D. IIIA E. IVB SBA 6 (answer) A 45-year-old woman presents to her GP with fatigue, night sweats, and weight loss. On examination, she has palpable non-tender cervical lymphadenopathy. She is urgently referred to haematology, where a blood film confirms the presence of Reed-Sternberg cells. Computed tomography (CT) and positron emission tomography (PET) imaging reveal enlarged lymph nodes in the left axillary and bilateral cervical regions. In addition, disease is found in the left para-aortic lymph nodes. According to the Ann-Arbor staging system, what stage of Hodgkin's lymphoma is the patient suffering from? A. IB B. IIA C. IIIB D. IIIA E. IVBLYMPHOMA SUMMARY HODGKINS LYMPHOMA • enlarged lymph nodes PAINFUL after alcohol consumption • Reed-Sternberg cells NON-HODGKINS • painless enlarged LN BURKITT'S LYMPHOMA • sub-type of NHL • Rapidly enlarged lymph node under the jaw • starry sky appearance on microscopySESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMA SBA 7 (question) A 66-year-old man presents to the emergency department with nausea, vomiting and lethargy. He had a seizure at home lasting 2 minutes and a further seizure in the emergency department. He has a diagnosis of Burkitt lymphoma and received his first dose of chemotherapy 5 days ago. What is this complication? His blood results are shown below: Haemoglobin (Hb) 110 g/L (130–170 g/L) Mean cell volume (MCV) 92 fL (80–96 fL) White cell count 2.5 × 10 /L (3.0–10.0 × 10 /L) Platelets 110 × 10 /L (150–400 × 10 /L) Lactate dehydrogenase 400 IU/L (70–250 IU/L) A. Hypercalcaemia of malignancy Sodium 142 mmol/L (135–146 mmol/L) B. Tumour lysis syndrome Potassium 6.3 mmol/L (3.5–5.3 mmol/L) Urea 13 mmol/L (2.5–7.8 mmol/L) C. Spinal cord compression Creatinine 167 mmol/L (60–120 mmol/L) D. Chemotherapy side effects Calcium 1.6 mmol/L (2.2–2.6 mmol/L) E. Neutropenic sepsis Phosphate 1.8 mmol/L (0.8–1.5 mmol/L)TUMOR LYSIS SYNDROME ONCOLOGICAL EMERGENCY!! Most common in treatment of haematological cancers → Especially ALL and high-grade NHL (eg. Burkitts) Hyperkalaemia Hyperphosphataemia Hypocalcaemia HyperuricaemiaTUMOR LYSIS SYNDROME K+ K+ LDH 3- PO4 PO43- K+ K+ LDH LDH PO 4- K+ PO 4-TUMOR LYSIS SYNDROME K+ K+ LDH 3- PO4 PO43- K+ K+ LDH LDH PO 4- K+ PO 4-TUMOR LYSIS SYNDROME INSULIN + CALCIUM DEXTROSE GLUCONATE ARRHYTHMIAS K+ 3- 2+ TETANY/ PO 4 Ca SEIZURES AKI Hypoxanthine IV fluids PURINEs & PYRIMIDINES Xanthine ALLOPURINOL RASBURICASE Uric acid GOUTTUMOR LYSIS SYNDROME K+ INSULIN + DEXTROSE PO 43- PHOSPHATE BINDERS IV fluids Ca 2+ CALCIUM GLUCONATE ALLOPURINOL Uric acid RASBURICASE SBA 7 (question) A 66-year-old man presents to the emergency department with nausea, vomiting and lethargy. He had a seizure at home lasting 2 minutes and a further seizure in the emergency department. He has a diagnosis of Burkitt lymphoma and received his first dose of chemotherapy 5 days ago. What is this complication? His blood results are shown below: Haemoglobin (Hb) 110 g/L (130–170 g/L) Mean cell volume (MCV) 92 fL (80–96 fL) White cell count 2.5 × 10 /L (3.0–10.0 × 10 /L) Platelets 110 × 10 /L (150–400 × 10 /L) Lactate dehydrogenase 400 IU/L (70–250 IU/L) A. Hypercalcaemia of malignancy Sodium 142 mmol/L (135–146 mmol/L) B. Tumour lysis syndrome Potassium 6.3 mmol/L (3.5–5.3 mmol/L) Urea 13 mmol/L (2.5–7.8 mmol/L) C. Spinal cord compression Creatinine 167 mmol/L (60–120 mmol/L) D. Chemotherapy side effects Calcium 1.6 mmol/L (2.2–2.6 mmol/L) E. Neutropenic sepsis Phosphate 1.8 mmol/L (0.8–1.5 mmol/L) SBA 7 (answer) A 66-year-old man presents to the emergency department with nausea, vomiting and lethargy. He had a seizure at home lasting 2 minutes and a further seizure in the emergency department. He has a diagnosis of Burkitt lymphoma and received his first dose of chemotherapy 5 days ago. What is this complication? His blood results are shown below: Haemoglobin (Hb) 110 g/L (130–170 g/L) Mean cell volume (MCV) 92 fL (80–96 fL) White cell count 2.5 × 10 /L (3.0–10.0 × 10 /L) Platelets 110 × 10 /L (150–400 × 10 /L) Lactate dehydrogenase 400 IU/L (70–250 IU/L) A. Hypercalcaemia of malignancy Sodium 142 mmol/L (135–146 mmol/L) B. Tumour lysis syndrome Potassium 6.3 mmol/L (3.5–5.3 mmol/L) Urea 13 mmol/L (2.5–7.8 mmol/L) C. Spinal cord compression Creatinine 167 mmol/L (60–120 mmol/L) D. Chemotherapy side effects Calcium 1.6 mmol/L (2.2–2.6 mmol/L) E. Neutropenic sepsis Phosphate 1.8 mmol/L (0.8–1.5 mmol/L)SESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMA SBA 8 (question) An 82-year-old male comes in with progressive generalised weakness, nausea and constipation. The physical exam and vital signs are normal. His blood results are remarkable for a calcium of 4.2 mmol/L and Hb 6g/dL. His admission chest x-ray revealed multiple lytic bone lesions. What is the most likely cause of his hypercalcaemia? A. Prostate cancer B. Osteosarcoma C. Multiple myeloma D. Hyperparathyroidism E. Paget’s diseaseMultiple myelomaMULTIPLE MYELOMA AETIOLOGY Presentation (CRAB) • PROLIFERATION of plasma cells and over pr• PARAPROTEIN → IgG most common C alciumhigh (stones, moans,groans) • Age >70 R enal impairment • Agricultural work : exposure to herbicides etc • Family history A naemia- due to bone marrow crowding • African decent B one lesions- increased osteoclast activation (pain, osteolytic lesions,fractures)MULTIPLE MYELOMA INVESTIGATIONS BLOODS • Low Hb, high Ca, high Cr • Normal ALP BLOOD FILM • Rouleaux formation • Bence jones proteins in urine • Serum electropheresis – paraprotein spike • Bone marrow aspirate - >10% plasma cells BONE SCAN • X-ray skull → pepper pot skull lesionsMULTIPLE MYELOMA MANAGEMENT • Stem Cell Transplant • Chemotherapy/steroid/Monoclonals • Average survival 5-7 years • Supportive for CRAB symptoms • Ca → fluids and bisphosphonates COMPLICATIONS • Hypercalcaemia • Spinal Cord Compression SBA 8 (question) An 82-year-old male comes in with progressive generalised weakness, nausea and constipation. The physical exam and vital signs are normal. His blood results are remarkable for a calcium of 4.2 mmol/L and Hb 6g/dL. His admission chest x-ray revealed multiple lytic bone lesions. What is the most likely cause of his hypercalcaemia? A. Prostate cancer B. Osteosarcoma C. Multiple myeloma D. Hyperparathyroidism E. Paget’s disease SBA 8 (question) An 82-year-old male comes in with progressive generalised weakness, nausea and constipation. The physical exam and vital signs are normal. His blood results are remarkable for a calcium of 4.2 mmol/L and Hb 6g/dL. His admission chest x-ray revealed multiple lytic bone lesions. What is the most likely cause of his hypercalcaemia? A. Prostate cancer B. Osteosarcoma C. Multiple myeloma D. Hyperparathyroidism E. Paget’s disease SBA 9 (question) A 66-year-old man, who has a diagnosis of multiple myeloma, presents to the emergency department with after being found on the floor by his wife. On further questioning he has backpain and he admits to having some urinary incontinence. strength.cal examination of the limbs shows bilateral lower limb weakness but preserved arm What is the most likely cause of this presentation? A. Hypercalcaemia of malignancy B. Tumour lysis syndrome C. Spinal cord compression D. Chemotherapy side effects E. Neutropenic sepsis SBA 9 (answer) A 66-year-old man, who has a diagnosis of multiple myeloma, presents to the emergency department with after being found on the floor by his wife. On further questioning he has backpain and he admits to having some urinary incontinence. strength.cal examination of the limbs shows bilateral lower limb weakness but preserved arm What is the most likely cause of this presentation? A. Hypercalcaemia of malignancy B. Tumour lysis syndrome C. Spinal cord compression D. Chemotherapy side effects E. Neutropenic sepsis SPINAL CORD COMPRESSION Fractures/vertebral collapse can compress the spinal cord Complication ofmultiple myeloma due to osteolytic features Features: • Back pain • Numbness, weakness in limbs • Urinary/ bowel incontinence Diagnosis • Whole body X-ray/ MRI Managament • Analgaesia +/- steroids • Surgical decompressionSESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMA Myeloproliferative/Myelodysplasia Does not make Increase in healthy red blood one/mixture of cell cells lineageMyeloproliferative Syndrome Disease of the elderly Rarely can transform into AML Chronic Myeloid Leukaemia Polycythaemia Vera Essential ThrombocytosisMyeloproliferative neoplasms 1. ESSENTIAL THROMBOCYTHEMIA AETIOLOGY • Mutation in the JAK2 gene 55% • Causes proliferation of megakaryocytes and over production of platelets CLINICAL FEATURES • Thrombus formation • DVT • Leg pain and swelling • PE • SOB, haemoptysis • Hyperviscosity • Headache, dizziness • SplenomegalyMyeloproliferative neoplasms 1. ESSENTIAL THROMBOCYTHEMIA INVESTIGATIONS: Bedside - Examine for DVT - Livedo reticularis Bloods - FBC - Raised Plts (>450-600 x 10^9/L) - CRP/ESR - Rule out infection/ inflammation Special - Gene panel – JAK2mutation Myeloproliferative neoplasms 1. ESSENTIAL THROMBOCYTHEMIA MANAGEMENT: 1. Aspirin (antiplatelet) 2. Hydroxycarbamide • If aspirin CI eg. asthma • High risk pts • Good prognosisMyeloproliferative neoplasms 2.POLYCYTHEMIA RUBRA VERA AETIOLOGY • PRIMARY POLYCYTHEMIA • Mutation in thJAK2 gene (95%) • Causes BM to make too many RBCs CLINICAL FEATURES • Itchiness especially after a warm bath • Tingling/ burning in hands • Burning vision • Headaches • Plethoric (red nose) • Splenomegaly • Thrombosis • GoutMyeloproliferative neoplasms 2.POLYCYTHEMIA RUBRA VERA INVESTIGATIONS: Bloods • FBC • High Hb, Hct >0.52 (M), >0.48(F) • EPO levels (low in PRV, high in EPO secreting tumor) Special • Gene panel- JAK2mutationMyeloproliferative neoplasms 2.POLYCYTHEMIA RUBRA VERA MANAGEMENT • Venesection (maintain Hct <0.45) • Aspirin • Hydroxycarbamide • If high risk of thrombosis • JAK2inhibitorsMyeloproliferative neoplasms 3. MYELOFIBROSIS AETOLOGY • Clonal proliferation of stem cells → cytokine release → fibrosis • Resulting in BM failure • JAK2mutation in 50% CLINICAL FEATURES • Usually elderly • Pancytopenia related Sx • Can have massive splenomegaly Splenomegaly can differentiate from myelodysplasiaMyeloproliferative neoplasms 3. MYELOFIBROSIS INVESTIGATIONS • BM aspiration → Dry tap • Blood film → Tear drop poikilocytes (dacrocytes) • Moleculartests for JAK2 MANAGEMENT • Support with blood products • JAK2inhibitors • Stem cell transplantMYLOPROLIFERATIVE SUMMARY ESSENTIALTHROMBOCYTHAEMIA • High platelets • Thrombosis risk – treat with aspirin POLYCYTHAEMIAVERA • High red blood cells • Itching after hot bath • Thrombosis risk - venesection MYLOFIBROSIS • BM failure and splenomegaly • Dry tap on bone marrow aspirationSESSION CONTENT • LEUKAEMIAS AML ALL CML CLL • MYELOPROLIFERATIVE NEOPLASMS Essential Thrombocythaemia • LYMPHOMAS Polycythaemia Vera Non-Hodgkins Myelofibrosis -Burkitts • TUMOR LYSIS SYNDROME • MULTIPLE MYELOMATHANK YOU FOR COMING! PLEASE, PLEASE, PLEASE FILL IN THE FEEDBACK FORM!