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Haematology Part 2 Myelogenous Leukemias Lymphocytic Leukemias Lymphomas Myeloma Oncological Emergencies 1.5 hours Q U E S T I 1 N A 68 year old man presents to A&E with sudden A CHRONIC MYELOID LEUKAEMIA onset right sided abdominal pain and a distended abdomen. On examination, the liver B HODGKIN'S LYMPHOMA can be felt 5 cm below the costal margin and the patient is visibly tender on palpation of the right upper quadrant. The patient is currently being C NON -HODGKINS LYMPHOMA treated for a haematological malignancy, however as he was on holiday and thus has D ACUTE MYELOID LEUKAEMIA missed a few clinic appointments. Given this presentation, what haematological malignancy E POLYCYTHAEMIA RUBRA VERA does this most patient have? ANSWER ON THE ZOOM POLL Q U E S T I 1 N A 68 year old man presents to A&E with sudden A CHRONIC MYELOID LEUKAEMIA onset right sided abdominal pain and a distended abdomen. On examination, the liver B HODGKIN'S LYMPHOMA can be felt 5 cm below the costal margin and the patient is visibly tender on palpation of the right upper quadrant. The patient is currently being C NON -HOGDKINS LYMPHOMA treated for a haematological malignancy, however as he was on holiday and thus has D ACUTE MYELOID LEUKAEMIA missed a few clinic appointments. Given this presentation, what haematological malignancy E POLYCYTHAEMIA RUBRA VERA does this patient most likely have? ANSWER ON THE ZOOM POLL Polycythaemia Rubra Vera Pathophysiology Symptoms • Uncontrolled proliferation of RBC • May present with stroke, Budd precursors Chiari etc due to high viscosity of • Hyper viscous blood blood • 90% have Jak 2 mutation • Itching after warm bath Investigations Treatment • Regular venesections • Elevated Hb on FBC • Hydroxyurea • Bone Marrow biopsy- hypercellular • aspirin prophylaxis Essential Thrombocytosis Pathophysiology Symptoms • Uncontrolled proliferation of megakaryocytes • Burning sensation in hands • Increased platelets- prothrombotic • Pro-thrombotic!!! • 50% have Jak 2 mutation Investigations Treatment • High platelet count • Bone Marrow biopsy- • Hydroxyurea megakaryocyte proliferation • aspirin prophylaxis Q U E S T I O2N A 74 year old man presents to A&E with A SMEAR CELLS increased shortness of breath, fatigue and dizziness. On examination there is marked B ROULEAUX FORMATION conjunctival pallor, and a large mass if felt on the left upper quadrant of the abdomen. A haematological malignancy is suspected and a C REED STEINBURG CELLS bone marrow biopsy is attempted however is unsuccessful. Given the likely diagnosis, what is D TEAR DROP POIKILOCYTES most likely to be seen on a blood film of this patient? E HEINZ BODIES ANSWER ON THE ZOOM POLL Q U E S T I O2N A 74 year old man presents to A&E with A SMEAR CELLS increased shortness of breath, fatigue and dizziness. On examination there is marked B ROULEAUX FORMATION conjunctival pallor, and a large mass if felt on the left upper quadrant of the abdomen. A haematological malignancy is suspected and a C REED STEINBURG CELLS bone marrow biopsy is attempted however is unsuccessful. Given the likely diagnosis, what is D TEAR DROP POIKILOCYTES most likely to be seen on a blood film of this patient? E HEINIZ BODIES ANSWER ON THE ZOOM POLL Myelofibrosis Pathophysiology Symptoms • Uncontrolled proliferation of abnormal megakaryocytes • Platelet derived factor activates • Anaemia fibroblasts • Loss of appetite • Collagen deposition in the bone marrow Investigations Treatment • FBC • Bone marrow transplant • Trephine biopsy Myelodysplasia Pathophysiology Symptoms • Neoplastic disorder- preleukemia • Anaemia • Bruising may progress onto AML • Recurrent infections Investigations Treatment • FBC • Bone Marrow biopsy • Bone marrow transplant BM Transplants 1 2 3 Allogenic- from another Umbilical Cord- Cells frozen Autologous- patients own person who is genotypically and birth and transplanted bone marrow used similar later • CML • Myelofibrosis Causes of Massive Splenomegaly • Kala Azar • Malaria Q U E S T I 3 N A 74 year old man presents to A&E with A HIGH PHOSPHATE, LOW CALCIUM complaining of palpitations. He has recently been diagnosed with non -Hogdkin’s lymphoma B cell B HIGH SODIUM, HIGH CHLORIDE lymphoma for which he received chemotherapy 4 days ago. An ECG shows flattened P waves, and tall tented T waves. Given the likely condition, C HIGH PHOSPHATE, HIGH CALCIUM what other abnormality may be detected in this patient? D LOW SODIUM, LOW CALCIUM E LOW PHOSPHATE, LOW CALCIUM ANSWER ON THE ZOOM POLL Q U E S T I O N 3 A 74 year old man presents to A&E with A HIGH PHOSPHATE, LOW CALCIUM complaining of palpitations. He has recently been diagnosed with non -Hodgkin’s lymphoma B cell B HIGH SODIUM, HIGH CHLORIDE lymphoma for which he received chemotherapy 4 days ago. An ECG shows flattened P waves, and tall tented T waves. Given the likely condition, C HIGH PHOSPHATE, HIGH CALCIUM what other abnormality may be detected in this patient? D LOW SODIUM, LOW CALCIUM E LOW PHOSPHATE, LOW CALCIUM ANSWER ON THE ZOOM POLL Q U E S T I O4N 48 year old lady who has recently been A MEASURE URINE OUTPUT diagnosed with breast cancer of T3N2M0 staging, undergoes neoadjuvant chemotherapy B FBC with the aim of reducing its size to enable surgical management. She presents to the ED complaining of some chills and shivers. Basic C IV RASBURICASE observations show she has a temperature of 37.9 degrees celcius . Blood pressure is normal. Given D IV ACICLOVIR the likely diagnosis, what is the next most important step in the management of this patient. E IV BROAD SPECTRUM ANTIBIOTICS ANSWER ON THE ZOOM POLL Q U E S T I O4N 48 year old lady who has recently been A MEASURE URINE OUTPUT diagnosed with breast cancer of T3N2M0 staging, undergoes neoadjuvant chemotherapy B FBC with the aim of reducing its size to enable surgical management. She presents to the ED complaining of some chills and shivers. Basic C IV RASBURICASE observations show she has a temperature of 37.9 degrees celcius . Blood pressure is normal. Given D IV ACICLOVIR the likely diagnosis, what is the next most important step in the management of this patient. E IV BROAD SPECTRUM ANTIBIOTICS ANSWER ON THE ZOOM POLLTumour Lysis Syndrome Hypercalcemia Cord Compression High Potassium Can be seen in malignancy MRI spine within 24 hours High Phosphate SCC of the lungs a common cause High Dose dexamethasone Low Calcium Groans, moans, stones bones SVCO: Pemberton’s sign Prevention: IV IV fluids High Dose Dexamethosone allopurinol/rasburicase IV bisphosphonates Why might you see distended Outline the management of veins in the head and neck in hyperkalaemia? What would the ECG show? SVCO? • Any patient at risk of neutropenia e.g post chemotherapy • Usually when Neutrophils < 1 Neutropenic Sepsis • Immediate Broad Spectrum Antibiotics e.g Tazocin • DO NOT WAIT FOR BLOOD CULTURES!!!!!!!! Q U E S T I 5 N A 65 year old Caucasian man comes in with A HYPERSEGMENTED NEUTROPHILS increasing lethargy. His wife complains that he no longer does the dishes or the gardening as he is B MULTIPLE MATURE GRANULOCYTES so tired, and that this has got progressively worse over the past year or so. A look at this past medical history shows that in the past year, C SMEAR CELLS he has been admitted to the hospital several times for chest infections. On examination the D REED-STEINBURG CELLS patient has pale conjunctiva. A FBC shows a low Hb, with a slightly raised mean corpuscular E AUER RODS volume with a high reticulocyte count. Given this presentation, what is most likely to be seen in this ANSWER ON THE ZOOM POLL patient’s blood film. Q U E S T I 5 N A 65 year old Caucasian man comes in with A HYPERSEGMENTED NEUTROPHILS increasing lethargy. His wife complains that he no longer does the dishes or the gardening as he is B MULTIPLE MATURE GRANULOCYTES so tired, and that this has got progressively worse over the past year or so. A look at this past medical history shows that in the past year, C SMEAR CELLS he has been admitted to the hospital several times for chest infections. On examination the D REED-STEINBURG CELLS patient has pale conjunctiva. A FBC shows a low Hb, with a slightly raised mean corpuscular E AUER RODS volume, with a high reticulocyte count. Given this presentation, what is most likely to be seen in this ANSWER ON THE ZOOM POLL patient’s blood film. Q U E S T I O6N A 65 year old Caucasian man comes in with increasing lethargy. His wife complains that he no A BLAST CRISIS longer does the dishes or the gardening as he is so tired, and that this has got progressively B EXACERBATION OF CML worse over the past year or so. A look at this past medical history shows that in the past year, C RICHTER’S TRANSFORMATION he has been admitted to the hospital several times for chest infections. On examination the patient has pale conjunctiva. A FBC shows a low D LEONARD’S TRANSFORMATION Hb, with a slightly raised mean corpuscular volume. This same patient then presents a week E NON-HOGKIN’S LYMPHOMA later with severe night sweats, weight loss and multiple enlarged lymph nodes. What has ANSWER ON THE ZOOM POLL happened to the patient? Q U E S T I O6N A 65 year old Caucasian man comes in with increasing lethargy. His wife complains that he no A BLAST CRISIS longer does the dishes or the gardening as he is so tired, and that this has got progressively B EXACERBATION OF CML worse over the past year or so. A look at this past medical history shows that in the past year, C RICHTER’S TRANSFORMATION he has been admitted to the hospital several times for chest infections. On examination the patient has pale conjunctiva. A FBC shows a low D LEONARD’S TRANSFORMATION Hb, with a slightly raised mean corpuscular volume. This same patient then presents a week E NON-HOGKIN’S LYMPHOMA later with severe night sweats, weight loss and multiple enlarged lymph nodes. What has ANSWER ON THE ZOOM POLL happened to the patient? Lymphoid: Lymphoid ALL progenitor cell Natural Small Killer Cell Lymphocyte T- Cell B cell CLL Plasma Cell Myeloma Acute Lymphoblastic Leukaemia Pathophysiology Symptoms • Uncontrolled proliferation of • Recurrent infections lymphoid progenitor cell • Unexplained bruising/petechiae • No mature B or T lymphocytes • Can affect the CNS Investigations Treatment • FBC • To induce remission: Vincristine, • Blood Film Prednisolone, an anthracycline, • Bone marrow biopsy asparaginase Chronic Lymphoblastic Leukaemia Pathophysiology Symptoms • Uncontrolled proliferation of B cells • Insidious onset • Hypogammaglobinaemia • Anemia • Recurrent infections • Most common in Western world amongst the elderly!! Investigations Treatment • FBC • Blood film- SMEAR CELLS • Early stage: ‘Watch and wait’ • Bone Marrow biopsy • Chemotherapy including rituximab • Transformation of CLL to High grane no hodgkins B cell Lymphoma • Rapid Deterioration Richter’s Transformation • B symptoms, plus lymphadenopathy • Poor Prognosis Q U E S T I O7N A 67 year old male comes in with complaining of A FBC abdominal pain and fatigue. His past medical history shows that he recently had a right ureteric B PERIPHERAL BLOOD FILM stone treated via lithotripsy. An FBC shows a low Hb and low platelets. On U&Es the patient is noted to have a high calcium and the blood film C URINE ELECTROPHORESIS shows a rouleaux formation. Given the likely diagnosis (Dx) what is the best investigation used D SERUM ELECTROPHORESIS to confirm the Dx? E BONE MARROW BIOPSY ANSWER ON THE ZOOM POLL Q U E S T I O7N A 67 year old male comes in with complaining of A FBC abdominal pain and fatigue. His past medical history shows that he recently had a right ureteric B PERIPHERAL BLOOD FILM stone treated via lithotripsy. An FBC shows a low Hb and low platelets. On U&Es the patient is noted to have a high calcium and the blood film C URINE ELECTROPHORESIS shows a rouleaux formation. Given the likely diagnosis (Dx) what is the best investigation to D SERUM ELECTROPHORESIS confirm the Dx? E BONE MARROW BIOPSY ANSWER ON THE ZOOM POLL Myeloma Pathophysiology Symptoms • Uncontrolled proliferation of plasma cells • Symptoms due to bone marrow • Median Age of 70 • CRABBI infiltration and plasma protein deposition Investigations Treatment • Bortezomib + Dexamethasone • FBC (Stem Cell Transplant possible) • Blood Film • Thalidomide + an Alkylating • Urine- Bence Jones Proteins agent + Dexamethasone • Gold Standard: BM Biopsy (Stem Cell Transplant not possible) • Hypercalcaemia • Renal Failure Myeloma • Anaemia • Infections Q U E S T I 8 N A 56 year old male comes with to the GP, with A MYC MUTATION the results of a private peripheral blood film he had when he was away in the USA. The results B BCR- ABL (T(9:22)) state,’ presence of multiple mature granulocytes. On examination, the patient is found to have massive splenomegaly. Given the likely diagnosis C BRCA2 what genetic mutation might this patient have? D KRAS E ADAM 17 ANSWER ON THE ZOOM POLL Q U E S T I 8 N A 56 year old male comes with to the GP, with A MYC MUTATION the results of a private peripheral blood film he had when he was away in the USA. The results B BCR- ABL (T(9:22)) state,’ presence of multiple mature granulocytes. On examination, the patient is found to have massive splenomegaly. Given the likely diagnosis C BRCA2 what genetic mutation might this patient have? D KRAS E ADAM 17 ANSWER ON THE ZOOM POLL Myeloid: Myeloid progenitor AML cell Megakaryocytes Erythrocytes Mast Cell Myeloblasts Platelets Eosinophil Basophil Neutrophil Monocyte Macrophage Acute Myeloid Leukaemia Pathophysiology Symptoms • Uncontrolled proliferation of the • Neutropenia: Recurrent infections myeloid precursor cells • Anaemia: Fatigue, pallor • This fills up the Bone marrow • Typically in the elderly • Thrombocytopenia: Bruising Investigations Treatment • Depends on the stage as well as • FBC the patient. Intensive • Blood Film: Auer Rods • Bone Marrow Biopsy Chemotherapy may be offered ( Cyatarabine, azacytidine) Chronic Myeloid Leukaemia Pathophysiology Symptoms • BCR- ABL mutation, causes uncontrolled tyrosine kinase • Anaemia activity • Night sweats • Increased proliferation of the granulocyte cell line • Splenomegaly- Early satiety Investigations Treatment • FBC • Blood Film: Granulocytes at • Imatinib various stages of differentiation • Gold Standard: BM Biopsy • Hydroxyurea • Cytogenetics ALL AML CML In children Can lead to Blast Crisis: Stroke etc BCR-ABL as its hypercoagulable CNS Involvement Tyrosine kinase inhibitor to treat Auer Rods FBC if in doubt Mature/Maturing Granulocytes Down’s children have a higher risk CLL APML Myeloma Most common in the elderly in the Causes DIC CRABI Western world This is because the promyelocytes release tissue factor in to the Most commonly IgG SMEAR Cells on blood film plasma Plasmacytomas can cause cord compression Q U E S T I O9N A 34 year old male presents to the A BONE MARROW BIOPSY Haematologist complaining of night sweats and fever. He has also lost about 5 stone in the past B TREPHINE BIOPSY month. He also complains that the front of his neck is very painful when he drinks alcohol. On examination the patient is found to have C FINE NEEDLE ASPIRATION asymmetrical lymphadenopathy of the cervical and axillary lymph nodes. Given these findings, D EXCISIONAL BIOPSY what is the gold standard investigation for diagnosis? E FBC ANSWER ON THE ZOOM POLL Q U E S T I O9N A 34 year old male presents to the A BONE MARROW BIOPSY Haematologist complaining of night sweats and fever. He has also lost about 5 stone in the past B TREPHINE BIOPSY month. He also complains that the front of his neck is very painful when he drinks alcohol. On examination the patient is found to have C FINE NEEDLE ASPIRATION asymmetrical lymphadenopathy of the cervical and axillary lymph nodes. Given these findings, D EXCISIONAL BIOPSY what is the gold standard investigation for diagnosis? E FBC ANSWER ON THE ZOOM POLL Hodgkin’ s Lymphoma Pathophysiology Symptoms • Clonal Transformation of cells of • Rubbery Lymphadenopathy B-cell origin, which gives rise to • B symptoms the Reed-Steinburg cells • Pain on Alcohol Consumption Investigations Treatment • FBC • Blood Film • BM Biopsy for advanced stages • ABVD Regimen • Excisional Biopsy Non-Hodgkin’ s Lymphoma Pathophysiology Symptoms • Uncontrolled proliferation of B/T cells within the lymph node- more • Lymphadenopathy: Classically commonly B cells symmetrical • Diffuse B cell Lymphoma is the • B symptoms most common type Investigations Treatment • FBC • Blood Film • BM Biopsy for late stages • R-CHOP • Excisional Biopsy • CT for extra nodal metastasis • I: Single lymph node region • II: 2 or more lymph node regions on the Ann- Arbour same side of the diaphragm Staging • III: Lymph node regions on both sides of the diaphragm B- Presence of B symptoms • IV: Diffuse involvement of extra-nodal tissue PLEASE FILL OUT THE FEEDBACK FORM PLEASE TUNE IN TO OUR REMAINING SESSIONS THIS WEEK! @OSCEazyOfficial @osceazyofficial OSCEazy Osceazy@gmail.com