In a patient with a deep venous thrombosis, for which of the following factors occurring in isolation at the time of the initial thrombotic event is there strongest indication for extending anticoagulation beyond six months?
A. Heterozygosity for factor V Leiden mutation.
B. Presence of a lupus inhibitor/anticoagulant.
C. Absence of a provoking stimulus.
D. Presence of an elevated homocysteine level.
E. Heterozygosity for prothrombin G20210A gene mutation.
Which one of the following statements is the best description of the molecular basis of a chimaeric protein (eg Bcr-Abl)?
A. Formation of disulphide bonds between cytosine residues on different peptides.
B. Post-transcription ligation of non-homologous messenger RNA (mRNA) molecules.
C. Hybrid messenger RNA (mRNA) synthesis from a bi-directional promoter.
D. Deletion of an exon from one gene.
E. In-frame ligation of the 5`-end of one gene to the 3`-end of another.
Give examples of diseases for each RBC-opathy
1. Abnormality of GPI-linked proteins2. Red cell enzyme deficiency3. Haemoglobin abnormality. 4. Red cell membrane abnormality. 5. Haematinic deficiency. B12/Folate
1. Paroxysmal Nocturnal Haemoglobulinemia.
2. G6PD, Pyruvate Kinase deficiency
3. Thalassemia, Sickle cell anaemia
4. Spherocytosis, elliptocytosis
5. B12/Folate/Iron deficiency
Why do you get leg ulcers in haemolytic disorders?
Leg ulcers may develop in patients with sickle cell anemia and other hemolytic disorders, as a result of decreased red blood cell (RBC) deformability and endothelial changes
QUESTION 51 Which of the following best explains the morphological appearance of red cells pictured below?
A. Abnormality of GPI-linked proteins.
B. Red cell enzyme deficiency.
C. Haemoglobin abnormality.
D. Red cell membrane abnormality.
E. Haematinic deficiency.
D. This is elliptocytosis
Of the following antiplatelet actions, which best describes the mechanism of clopidogrel?
A. Phosphodiesterase inhibition.
B. COX1 acetylation.
C. ADP receptor blocking. ADPase on endothelial cells. Granules released from platelets which leads to further platelet aggregation
D. GP IIb/IIIa blockade.
E. Factor Xa inhibition.
Which of the following is most characteristic of a patient with haemophilia A with 8% baseline factor VIII activity?
A. Spontaneous cutaneous purpura.
B. Spontaneous gastrointestinal haemorrhage.
C. Spontaneous deep muscle bleeds.
D. Spontaneous haemarthroses.
E. No spontaneous bleeding.
E. >5% activity = no bleeding
A is platelets not factors.B doesn't happen. (Can get haematoma)
A 69-year-old woman presents with severe pancytopenia and peripheral blood macrocytosis associated with a low serum vitamin B12 level. Which one of the following investigation results most strongly supports a diagnosis of pernicious anaemia? A. Positive anti-parietal cell antibody. B. Positive intrinsic factor antibody. C. Elevated fasting plasma homocysteine level. D. Elevated fasting serum gastrin level. E. Reduced red cell folate level.
Which of the following findings is most specific for the diagnosis of pernicious anaemia?
A. Increased serum homocysteine.
B. Increased serum methylmalonate.
C. Positive gastric parietal cell antibodies.
D. Increased serum gastrin.
E. Positive intrinsic factor antibodies.
E. Highly specific
A 21-year-old Filipino woman has been referred for investigation of anaemia. Her blood counts and iron studies are as follows:
Which of the following is the most likely diagnosis?
A. alpha+-thalassaemia trait (a-/aa).
B. alpha0-thalassaemia trait (--/aa).
C. HbH disease (--/-a).
D. beta0-thalassaemia trait.
E. beta+-thalassaemia trait.
C. HbH disease (--/-a).
A & B only give mild anaemia
In D&E, HbF makes up the majority of Hb with variable amounts of HbA2
A 65-year-old woman, established on haemodialysis for nine months, is maintained on erythropoietin 4,000 units twice a week intravenously. Previously her haemoglobin (Hb) had remained steady in the range of 105 – 115 gm/L. She now presents with a Hb of 89 gm/L on routine testing. There have been no changes to her erythropoietin dose or other medications. Her mean corpuscular volume (MCV) is 84 fL [78-98], her serum iron is 13 μmol/l [13-35], iron saturation is 18% [15-46], serum ferritin is 630 μg/l [20-300] and her reticulocyte count is 30 x 109/L [12-100].
Which of the following is the most likely cause for her erythropoietin resistance?
B. Pure red cell aplasia.
C. Vitamin B12 deficiency.
D. Iron deficiency.
A 78-year-old woman has a history of hypertension on amlodipine, and has been found to have a persistently elevated platelet count for several months. She presents to the emergency department with abdominal pain. Her full blood examination and serum biochemistry are as follows:
Which of the following is the most likely cause of her elevated serum potassium?
B. Renal tubular acidosis.
C. Chronic renal impairment.
D. Haemolytic anaemia.
E. Lactic acidosis.
A previously well 37-year-old man presents with lethargy and easy bruising over several weeks. Physical examination is unremarkable apart from occasional bruises.
His full blood examination shows:
His blood film is shown below.
The most likely diagnosis is:
A. essential thrombocythaemia.
B. occult haemorrhage.
C. primary myelofibrosis.
D. occult carcinoma.
E. chronic myeloid leukaemia.
A 47-year-old woman has coagulation studies performed as part of preoperative assessment prior to elective hysterectomy for menorrhagia.
Which of the following is the most likely interpretation of these results?
A. Tranexamic acid therapy.
B. Disseminated intravascular coagulation.
C. Heparin exposure.
D. Von Willebrand disease.
E. Lupus anticoagulant.
Which of the following is the most common mechanism of a Febrile Non-Haemolytic Transfusion reaction (FNHTR) to transfusion of packed red cells? A. Bacterial contamination of donor product. B. Immediate hypersensitivity to proteins of donor origin. C. Interaction between donor antibodies with recipient granulocytes. D. Interaction between donor granulocytes with recipient antibodies. E. Interaction between donor red cells with recipient red cell antibodies.
QUESTION 29 Which of the following is the greatest cause of mortality occurring more than 100 days following allogeneic peripheral blood cell stem transplantation for acute leukaemia? A. Hepatic veno-occlusive disease. B. Graft-versus-host disease. C. Transplantation-related lung injury. D. Transplantation-related infection. E. Recurrent leukaemia.
A 72-year-old woman who is taking warfarin for an embolic stroke secondary to atrial fibrillation presents to the emergency department after a simple fall. On examination she has a few small bruises on her legs but is otherwise well. Her pulse is 68 bpm and irregular and her blood pressure is 175/95 mmHg. Her full blood examination is normal however her serum creatinine is elevated at 170 mcg/L [50-100 mcg/L] and her INR is elevated at 7.1. In addition to regular monitoring, which of the following is the best management strategy for her elevated international normalised ratio (INR)? A. Withhold warfarin. B. Withhold warfarin and administer Vitamin K1. C. Withhold warfarin and administer Vitamin K1 and prothrombin complex concentrate. D. Withhold warfarin and administer Vitamin K1, prothrombin complex concentrate with fresh plasma. E. Withhold warfarin and administer prothrombin complex concentrate with fresh plasma.
A 58-year-old woman has been unwell for several weeks with back pains. She has no past medical history of note. She is a smoker of 25 pack years. On examination she is tender over the mid thoracic spine and over several ribs. The rest of the examination is unremarkable. Blood tests show:
A CXR shows lytic lesions in several thoracic vertebrae with clear lung fields. Spinal X-rays confirm several lytic vertebral lesions. Immunoglobulins are normal. Serum and urine electrophoresis and immunoelectrophoresis are negative. Mammography is normal. CT scanning of chest and abdomen shows the vertebral lesions but no other masses, no organomegaly and no lymphadenopathy. Thyroid and pelvic ultrasound are normal. A bone scan shows no increased uptake. Which of the following tests should next be performed? A. 24 hour urine calcium. B. Bone marrow examination. C. Biopsy of a bone lesion. D. Vitamin D levels. E. Parathyroid hormone related peptide levels.
B. This woman presents with the CRAB acronym of multiple myeloma - and it's likely nonsecretory. The other option would be serum free light chains which picks up nonsecretory disease
A 26-year-old female has been found to be homozygous for the HFE gene C282Y polymorphism, when screened for haemochromatosis. Her serum ferritin is 18 micrograms/L [15-200 micrograms/L] and her transferrin saturation is 82% [
Which of the following is the most appropriate next step in her management?
C. Liver biopsy.
D. Imaging of liver for iron loading.
Question 51 The following mutations (Cys282Tyr and His63Asp) are associated with hereditary haemochromatosis. Which one of the following genotypes provides the greatest risk for the development of clinical disease? A. Heterozygous Cys282Tyr. B. Heterozygous His63Asp. C. Double-heterozygote for Cys282Tyr and His63Asp. D. Homozygous Cys282Tyr. E. Homozygous His63Asp.
QUESTION 78 Which of the following situations is most likely to result in a false negative FDG-PET (Fluorodeoxyglucose-Positron Emission Tomography) scan when restaging a patient with aggressive lymphoma post-chemotherapy? A. Necrosis at site of previous involvement. B. Fibrosis at site of previous involvement. C. Brown fat. D. Uncontrolled diabetes. E. Recent surgery.
A 67-year-old man with longstanding rheumatoid arthritis treated with methotrexate, prednisolone and celecoxib presents unwell with fatigue and anorexia. On examination his arthritis is quiescent but he has two mouth ulcers. His full blood examination is shown below.
The most appropriate drug to administer is: A. folic acid. B. filgrastim. C. folinic acid. D. cholestyramine. E. prednisolone.
Which of the following transfusion reactions is leucodepletion most effective in preventing?A. Febrile non-haemolytic transfusion reaction.B. Transfusion associated graft versus host disease.C. Anaphylaxis.D. Transfusion related acute lung injury.E. Delayed haemolytic transfusion reaction.
QUESTION 7Hepcidin and ferroportin regulate systemic iron absorption by inhibition of:A. enterocyte brush border iron reduction.B. enterocyte apical iron uptake.C. iron oxidation by hephaestin.D. enterocyte basolateral iron release.E. iron binding to transferrin.
QUESTION 38Other than bacterial infections, opportunistic infection with which of the following is most likely to occur in a patient after induction chemotherapy for acute myeloid leukaemia (AML)?A. Candida albicans.B. Mycobacterium tuberculosis.C. Cytomegalovirus.D. Cryptosporidium.E. Toxoplasma gondii.
A blood film showing acanthocytes and Howell-Jolly bodies is taken from a patient withcoeliac disease.
Which of the following is the most likely explanation for this appearance?
A. Malnutrition.B. Hyposplenism.C. Iron deficiency.D. Hypothyroidism.E. Folate deficiency.
QUESTION 46Some autosomal recessive diseases have high prevalence in particular populations, even though theyare often fatal (e.g. Alpha thalassaemia in South East Asia; G6PD deficiency in the Mediterranean).Which of the following is the most likely explanation?A. Consanguinity.B. High mutation rates in specific populations.C. Survival advantage in heterozygous carriers.D. Survival advantage in wild type homozygotes.E. Founder effect.
A 30-year-old female presents with a painful swollen left calf. Duplex study demonstrates a proximaldeep vein thrombus extending into the ileofemoral venous system. The following laboratory resultsare obtained:Prothrombin time (PT) 16 sec [11 – 18 sec]Activated partial thromboplastin time (APTT) 43 sec [25 – 36 sec]Dilute Russell Viper venom test Lupus anticoagulant detectedAnticardiolipin IgG negativeAnticardiolipin IgM negativeAnti-beta 2 glycoprotein 1 moderate positiveTreatment with anticoagulation with heparin followed by warfarin therapy is commenced. Repeattesting at 12-weeks confirms a positive anti-beta 2 glycoprotein 1. What is the most appropriateduration of anticoagulation?A. 3 months.B. 6 months.C. 12 months.D. 24 months.E. Lifelong.
QUESTION 64A 37-year-old male is found to have some persistently enlarged lymph nodes (<2 cm diameter) in theleft posterior triangle. A biopsy reveals follicular, Grade 1, B-cell non-Hodgkin lymphoma. Stagingprocedures including computed tomography (CT) scan, positron emission tomography (PET) scan and bone marrow biopsy confirm the disease is confined to a single lymph node region in the neck.Which of the following management strategies is most appropriate?A. Watch and wait.B. Oral chlorambucil.C. Radiotherapy.D. Multiagent chemotherapy.E. Combined modality therapy.
QUESTION 68A 29-year-old woman is referred for evaluation after she was found to be heterozygous forfactor V Leiden mutation on screening. This screening was initiated when her great aunt sustained acalf vein thrombosis following hip arthroplasty. She is currently 16-weeks pregnant and has nopersonal history of venous thromboembolic disease.Which of the following should be recommended for the remainder of her pregnancy?A. Low molecular weight heparin.B. Aspirin.C. No thromboprophylaxis.D. Surveillance ultrasonography of the lower limbs.E. Warfarin.