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Pattern Recognition in Haematology
Laith Tafesh F1
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Aims of this session Focus on MCQs NOT preparation for MOSLERs
Haematology by pattern recognition NOT ins and outs of disease process – though this helps with memory
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MCQ1 A 67 year old gentlemen presents with a five week history of lethargy and lower back pain. In the last three days, his wife has noticed that he has become increasingly confused. Initial bloods on arrival to MAU are as follows: Hb – 75 Ca – 3.5 WCC – 3.5 Creatinine – 220 Platelets - 120 Urea – 14.5 Na – 140 PTH – low K – 4 LFTs – normal Which of the following would be the most appropriate initial investigation considering the most likely diagnosis? Renal biopsy Skeletal survey Serum electrophoresis and free light chains CT chest, abdomen, pelvis Bone marrow biopsy
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MCQ1 A 67 year old gentlemen presents with a five week history of lethargy and lower back pain. In the last three days, his wife has noticed that he has become increasingly confused. Initial bloods on arrival to MAU are as follows: Hb – 75 Ca – 3.5 WCC – 3.5 Creatinine – 220 Platelets - 120 Urea – 14.5 Na – 140 PTH – low K – 4 LFTs – normal Which of the following would be the most appropriate initial investigation considering the most likely diagnosis? Renal biopsy Skeletal survey Serum electrophoresis and free light chains CT chest, abdomen, pelvis Bone marrow biopsy
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Multiple Myeloma - Overview
Plasma cell malignancy Characterised by presence of paraprotein (monoclonal immunoglobulin or light chain) End organ damage
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Multiple Myeloma - CRAB
C – Hypercalcaemia R – Renal impairment A – Anaemia (and pancytopenia) B – Bone pathology (e.g. lytic lesions, bone pain)
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Multiple Myeloma – Key Investigations
Laboratory (Bloods): Serum electrophoresis – monoclonal band (paraprotein) Serum free light chains U/Es – renal impairment Calcium FBC – anaemia/pancytopenia Imaging: Skeletal survey – looking for lytic lesions (e.g. ‘pepper pot’ skull) Diagnostic: bone marrow aspirate + trephine NB: various other Ix, but above are probably most important to know
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MCQ2 A 75 year old lady presents to her GP with a five month history of generalised fatigue and weight loss. On examination, she has cervical and inguinal lymphadenopathy, and she also has significant hepatosplenomegaly. The GP performs a FBC, which reveals the following: Hb – Platelets – 190 WCC – Lymphocyte count – 18 Neutrophil count – 1.5 Which of the following is the most likely diagnosis? Hodgkin’s lymphoma Diffuse large B-cell lymphoma Myelofibrosis Acute lymphoblastic leukaemia Chronic lymphocytic leukaemia
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MCQ2 A 75 year old lady presents to her GP with a five month history of generalised fatigue and weight loss. On examination, she has cervical and inguinal lymphadenopathy, and she also has significant hepatosplenomegaly. The GP performs a FBC, which reveals the following: Hb – Platelets – 190 WCC – Lymphocyte count – 18 Neutrophil count – 1.5 Which of the following is the most likely diagnosis? Hodgkin’s lymphoma Diffuse large B-cell lymphoma Myelofibrosis Acute lymphoblastic leukaemia Chronic lymphocytic leukaemia
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Chronic lymphocytic leukaemia
Failure of apoptosis => accumulation of mature B cells Complications Autoimmune haemolytic anaemia Bone marrow failure (infiltration) Immunodefiency (hypogammaglobulinaemia) => infection
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Significant Lymphocytosis Chronic Lymphocytic Leukaemia
Elderly Lymphadenopathy/ Hepatosplenomegaly Significant Lymphocytosis Chronic Lymphocytic Leukaemia
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MCQ3 A 45 year old gentleman with acute myeloid leukaemia has gone through successful induction chemotherapy and is currently in remission. His haematologist has referred him for stem cell transplantation. His two siblings are being tested to see if they’re a suitable match to serve as stem cell donors. Which of the following is the most important factor in determining donor-recipient compatability? Sex Age HLA typing ABO blood group RhD blood group
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MCQ3 A 45 year old gentleman with acute myeloid leukaemia has gone through successful induction chemotherapy and is currently in remission. His haematologist has referred him for stem cell transplantation. His two siblings are being tested to see if they’re a suitable match to serve as stem cell donors. Which of the following is the most important factor in determining donor-recipient compatability? Sex Age HLA typing ABO blood group RhD blood group
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Haemopoeitic stem cell transplantation
Autologous vs allogeneic MRD vs MUD Benign and malignant disorders Allows intensive chemotherapy HLA typing – 12 alleles Major morbidity and mortality Complications GvHD (acute and chronic) Infection
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MCQ4 A 65 year old, previously fit and well, lady presents to her GP with progressive shortness of breath, lightheadedness and jaundice. Bloods are taken which reveal the following: Blood film reveals spherocytes. Which of the following investigations is most likely to be diagnostic? Direct antiglobulin test Chromium labeling Bone marrow aspirate Bone marrow trephine US liver Hb – 78 LDH – 800 WCC – 5 Bili – 58 Platelets - 230 ALP – 120 Reticulocytes ALT– 25
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MCQ4 A 65 year old, previously fit and well, lady presents to her GP with progressive shortness of breath, lightheadedness and jaundice. Bloods are taken which reveal the following: Blood film reveals spherocytes. Which of the following investigations is most likely to be diagnostic? Direct antiglobulin test Chromium labeling Bone marrow aspirate Bone marrow trephine US liver Hb – 78 LDH – 800 WCC – 5 Bili – 58 Platelets - 230 ALP – 120 Reticulocytes ALT– 25
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Haemolytic anaemia Premature red cell breakdown Acquired vs hereditary
Immune vs non-immune Autoimmune haemolytic anaemia – idiopathic or secondary to lymphoproliferative disorders or autoimmune disease
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Features in investigations
Increased red cell breakdown: anaemia, hyperbilirubinaemia, raised LDH Compensatory increased red cell production: raised reticulocyte count Blood film Spherocytes – hereditary spherocytosis, autoimmune haemolytic anaemia Schistocytes – MAHA Sickle cells – sickle cell disease Direct antiglobulin test (direct Coombs test)
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MCQ5 A 29 year old pregnant lady presents to the emergency department at 29 weeks gestation. She has developed sudden onset abdominal pain with severe vaginal bleeding. Placental abruption is suspected and bloods are taken in the resuscitation room. Hb – 85 APTT - 70 Platelets – 10 PT - 42 WCC – 6 Fibrinogen 0.5 Which of the following investigations would be most useful in confirming the most likely diagnosis? Bleeding time Thrombin time D-dimer Factor XIII assay Factor IX assay
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MCQ5 A 29 year old pregnant lady presents to the emergency department at 29 weeks gestation. She has developed sudden onset abdominal pain with severe vaginal bleeding. Placental abruption is suspected and bloods are taken in the resuscitation room. Hb – 85 APTT - 70 Platelets – 10 PT - 42 WCC – 6 Fibrinogen 0.5 Which of the following investigations would be most useful in confirming the most likely diagnosis? Bleeding time Thrombin time D-dimer Factor XIII assay Factor IX assay
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Coagulation Prolonged PT (extrinsic system)
Warfarin, vitamin K deficiency, liver disease Prolonged APTT (intrinsic system) Haemophilia A and B, lupus anticoagulant, heparin DIC: ↑APTT, ↑PT, ↓Fibrinogen, ↑D-dimer, ↓Platelets
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MCQ6 A 52 year old gentleman presents to his GP with a six month history of abdominal fullness, weight loss and fevers. Examination reveals massive splenomegaly. Blood counts reveal severe leukocytosis (WCC 110) with marked neutrophilia. Hb is 112 and platelet count is 320. Cytogenetics is likely to demonstrate a reciprocal translocation between which two chromosomes? 8 and 15 2 and 21 3 and 14 6 and 16 9 and 22
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MCQ6 A 52 year old gentleman presents to his GP with a six month history of abdominal fullness, weight loss and fevers. Examination reveals massive splenomegaly. Blood counts reveal severe leukocytosis (WCC 110) with marked neutrophilia. Hb is 112 and platelet count is 320. Cytogenetics is likely to demonstrate a reciprocal translocation between which two chromosomes? 8 and 15 2 and 21 3 and 14 6 and 16 9 and 22
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Chronic Myeloid Leukaemia
Myeloproliferative neoplasm Hepatosplenomegaly Usually ↑↑↑WCC with raised neutrophil count Philadelphia chromosome – reciprocal translocation between 9 and 22 BCR/ABL fusion gene Chronic, accelerated, blast transformation TKI, e.g. imatinib
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MCQ7 A 45 year old lady presents to A+E with intractable epistaxis. She has also been feeling gradually more lethargic over the last few weeks. Observations are stable and the patient is haemodynamically stable. The clerking doctor performs bloods, which reveal the following: Hb – 83 MCV – 95 WCC – 0.6 Neutrophils – 0.2 Platelets – 9 Reticulocyte count - 5 Coagulation screen – normal Other than severe neutropenia and thrombocytopenia, the blood film does not reveal anything significant. Which of the following is the most appropriate first line investigation? Bone marrow biopsy B12/folate CT chest, abdomen, pelvis Direct antiglobulin test Platelet function tests
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MCQ7 A 45 year old lady presents to A+E with intractable epistaxis. She has also been feeling gradually more lethargic over the last few weeks. Observations are stable and the patient is haemodynamically stable. The clerking doctor performs bloods, which reveal the following: Hb – 83 MCV – 95 WCC – 0.6 Neutrophils – 0.2 Platelets – 9 Reticulocyte count - 5 Coagulation screen – normal Other than severe neutropenia and thrombocytopenia, the blood film does not reveal anything significant. Which of the following is the most appropriate first line investigation? Bone marrow biopsy B12/folate CT chest, abdomen, pelvis Direct antiglobulin test Platelet function tests
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Pancytopenia Increased peripheral destruction
Hypersplenism, sepsis, autoimmune disease Decreased marrow production, i.e. bone marrow failure ALWAYS RULE OUT B12/FOLATE DEFICIENCY IN PANCYTOPENIA
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Megaloblastic anaemia
Bone marrow failure Hypocellular marrow Aplastic anaemia MDS Hypercellular marrow Infiltration Megaloblastic anaemia
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MCQ8 A 69 year old patient who has renal anaemia and a haemoglobin of 73 is receiving a unit of red blood cells for relief of symptoms of anaemia. An hour into the blood transfusion, the nursing staff call you as he has become pyrexial. All other observations are normal and systemic examination is unremarkable. The patient feels shivery. What type of transfusion reaction does this likely represent? Acute haemolytic reaction Bacterial contamination Non-haemolytic febrile transfusion reaction Mild allergic reaction Anaphylaxis
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MCQ8 A 69 year old patient who has renal anaemia and a haemoglobin of 73 is receiving a unit of red blood cells for relief of symptoms of anaemia. An hour into the blood transfusion, the nursing staff call you as he has become pyrexial. All other observations are normal and systemic examination is unremarkable. The patient feels shivery. What type of transfusion reaction does this likely represent? Acute haemolytic reaction Bacterial contamination Non-haemolytic febrile transfusion reaction Mild allergic reaction Anaphylaxis
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Transfusion reactions
Acute haemolytic transfusion reaction (ABO incompatibility) Bacterial contamination – septic signs Anaphylaxis – ABC problems Allergic reaction – urticaria, itch, low grade pyrexia Febrile reaction – pyrexia, shivery TACO – fluid overload secondary to transfusion TRALI – ARDS secondary to transfusion
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MCQ9 A 24 year old lady with night sweats and lymphadenopathy is referred to the haematology clinic by her GP. The haematologists organise a lymph node biopsy, which reveals Reed-Sternberg cells. What is the most likely diagnosis? Non-Hodgkin’s lymphoma Hodgkin’s lymphoma Chronic lymphocytic leukaemia Acute lymphoblastic leukaemia Glandular fever
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MCQ9 A 24 year old lady with night sweats and lymphadenopathy is referred to the haematology clinic by her GP. The haematologists organise a lymph node biopsy, which reveals Reed-Sternberg cells. What is the most likely diagnosis? Non-Hodgkin’s lymphoma Hodgkin’s lymphoma Chronic lymphocytic leukaemia Acute lymphoblastic leukaemia Glandular fever
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Lymphoma Hodgkin’s lymphoma – Reed-Sternberg cell
Non-Hodgkin’s – main subtypes Highly specialist subject – stick to basics for finals Malignant proliferation of lymphocytes – accumulate in lymphatic system B symptoms – fever >38, drenching night sweats, weight loss >10% in six months
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MCQ10 A 70 year old gentleman is admitted to A+E with a GCS of 9 (M4, V3, E2) following a fall and head injury. The patient is on warfarin for atrial fibrillation. What is the most appropriate first step to take with regards to the management of this patient? Urgent CT head Administer prothrombin complex concentrate Administer IV vitamin K Urgent neurosurgical intervention Administer fresh frozen plasma
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MCQ10 A 70 year old gentleman is admitted to A+E with a GCS of 9 (M4, V3, E2) following a fall and head injury. The patient is on warfarin for atrial fibrillation. What is the most appropriate first step to take with regards to the management of this patient? Urgent CT head Administer prothrombin complex concentrate Administer IV vitamin K Urgent neurosurgical intervention Administer fresh frozen plasma
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Warfarin Vitamin K antagonist
Indications: AF, VTE, mechanical heart valves INR target range – varies depending on indication (usually 2-3) Reversal: vitamin K (delayed onset), prothrombin complex concentrate (rapid onset but temporary effect)
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Reversal - Bleeding Guidelines vary depending on Trust
Life threatening bleed: Beriplex + IV vitamin K Significant bleed (haemodynamically stable): IV vitamin K Minor bleed: oral vitamin K
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Reversal – No bleeding INR >8: oral vitamin K
INR 5-7.9: omit or reduce dose, oral vitamin K if high risk
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Summary Focus on haematology MCQs
Unlikely to get in MOSLERs – except maybe splenomegaly Learn to recognise patterns
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