Mock Haematology Module 3

A 50-year-old man is evaluated for erectile dysfunction, progressive loss of libido, personality change and fatigue over 6 months. He is obese and has gained 11 kg in the past year since ceasing smoking (30 pack-years). He snores heavily, sleeps unrefreshed, and has excessive daytime somnolence (falls asleep watching television and at the dinner table). He denies depressed mood.

Examination: BP 140/80 mmHg; HR 92/min; T 37.0°C; weight 120 kg. Resting and post-exertional SpO₂ 92%. Otherwise normal apart from obesity.

• Haemoglobin 185 g/L [130–180]
• Haematocrit 0.55 [0.40–0.54]
• WCC 4.6 ×10⁹/L [4.0–11.0] with normal differential
• Platelets 275 ×10⁹/L [150–400]
• Blood film normal
• Sodium 142 mmol/L [135–145]
• Potassium 4.2 mmol/L [3.5–5.2]
• Creatinine 88 µmol/L [60–110]
• Haemoglobin–oxygen dissociation (P50) normal
• Serum erythropoietin high-normal

Which of the following is the most likely cause of the erythrocytosis?

A 75-year-old woman has been treated for polycythaemia vera for 10 years with phlebotomy and hydroxyurea. She has not required phlebotomy for 4 years, and the hydroxyurea dose was gradually reduced because of cytopenias before being ceased 3 weeks ago. Examination reveals pallor and a spleen palpable 12 cm below the left costal margin.

• Haemoglobin 95 g/L [115–155]
• Haematocrit 0.25 [0.36–0.46]
• White cell count 2.2 ×10⁹/L [4.0–11.0]
• Platelets 550 ×10⁹/L [150–400]
• Sodium 140 mmol/L [135–145]
• Potassium 4.1 mmol/L [3.5–5.2]
• Creatinine 106 µmol/L [45–90]
• Peripheral blood film: teardrop-shaped red cells, nucleated red cells, myelocytes, promyelocytes and 1% myeloblasts

What is the most likely cause of the anaemia, thrombocytopenia and leukopenia?

Which of the following is most suggestive of intravascular haemolysis?

A 36-year-old man presents post-ictally after an observed generalised seizure.

• Haemoglobin 90 g/L [130–180]
• MCV 106 fL [80–100]
• White cell count 12.6 ×10⁹/L [4.0–11.0]
• Neutrophils 10.4 ×10⁹/L [2.0–8.0]
• Lymphocytes 0.8 ×10⁹/L [1.0–4.0]
• Monocytes 1.1 ×10⁹/L [0.2–1.0]
• Eosinophils 0.2 ×10⁹/L [0.0–0.5]
• Basophils 0.1 ×10⁹/L [0.0–0.2]
• Platelets 135 ×10⁹/L [150–400]

Blood film: macrocytic red cells with anisopoikilocytosis; neutrophils are hypersegmented (a single neutrophil with ≥6 lobes, or ≥5% with ≥5 lobes).

The most likely cause of the macrocytosis is:

A 15-year-old girl presents with jaundice and moderate pallor. Examination reveals jaundice, no hepatomegaly, and a spleen palpable 3–4 cm below the costal margin.

• Haemoglobin 75 g/L [115–140]
• MCV 80 fL [75–90]
• Platelets 410 ×10⁹/L [150–600]
• White cell count 10.1 ×10⁹/L [5.0–14.5], normal differential

Peripheral blood film shows spherocytosis.

The next most appropriate test is:

A 20-year-old man presents with pallor, lethargy and tachycardia.

• Haemoglobin 50 g/L [130–180]
• MCV 62 fL [80–100]
• White cell count 4.8 ×10⁹/L [4.0–11.0]
• Platelets 520 ×10⁹/L [150–400]
• Albumin 22 g/L [34–48]

Blood film: microcytic, hypochromic red cells with anisopoikilocytosis and pencil cells.

What is the most likely diagnosis?

A 23-year-old man presents with pallor, dark urine and fever following bloody diarrhoea 7 days earlier. He has a family history of hereditary spherocytosis.

• Haemoglobin 79 g/L [130–180]
• White cell count 23 ×10⁹/L [4.0–11.0]
• Platelets 110 ×10⁹/L [150–400]
• Creatinine 180 µmol/L [60–110]
• Urea 15 mmol/L [3.0–8.0]
• LDH markedly elevated [120–250 U/L]
• Direct antiglobulin test negative

Blood film: schistocytes (fragmented red cells) and helmet cells.

Which of the following is the most likely diagnosis?

A 16-year-old is brought to hospital with a one-day history of fever, vomiting and lethargy. He is pale with mildly icteric sclera. T 37.5°C, HR 110/min, BP 90/60 mmHg, RR 25/min. No hepatosplenomegaly. Urinalysis is positive for blood.

• Haemoglobin 78 g/L [116–136]
• MCV 83 fL [75–85]
• MCH 29 pg [24–30]
• MCHC 345 g/L [310–360]
• Platelets 292 ×10⁹/L [150–450]
• Reticulocytes 154.2 ×10⁹/L [20–105]
• Heinz bodies negative; poikilocytosis +; occasional spherocytes
• White cell count 5.83 ×10⁹/L [6.10–11.00]
• Total bilirubin 84 µmol/L [2–20]; conjugated 8 µmol/L [<10]
• Haptoglobin <0.06 g/L [0.30–2.10]
• DAT (poly / IgG / C3d) all negative

Which diagnosis is most likely?

Which of the following is the most important component in the pathogenesis of β-thalassaemia?

A couple planning a family both have a family history of thalassaemia.

Which combination of parental genotypes would cause the least concern regarding an affected child?

A 24-year-old woman recently arrived from Vietnam presents for evaluation of abnormal menstrual bleeding. Examination is normal.

• Haemoglobin 113 g/L [120–155]
• Red cell count 5.2 ×10¹²/L [3.8–5.2]
• MCV 71 fL [80–95]
• MCH 22.0 pg [27.0–32.5]
• MCHC 310 g/L [320–360]
• White cell count 6.6 ×10⁹/L [4.0–11.0], normal differential
• Platelets 212 ×10⁹/L [150–400]
• Blood film: microcytosis and hypochromasia, otherwise normal
• HbA₂ 2.7% [1.8–3.5]; HbF 0.4% [0–2.0]; no abnormal bands
• HbH preparation: occasional HbH inclusions present
• Iron 8 µmol/L [7–32]; transferrin 3.2 g/L [2.1–3.6]; ferritin 15 µg/L [15–200]

The most likely diagnosis is:

A 28-year-old man presents with a fractured ankle requiring surgery.

• Haemoglobin 115 g/L [130–180]
• Red cell count 5.6 ×10¹²/L [4.0–5.7]
• MCV 62 fL [80–100]
• White cell count 12.5 ×10⁹/L [4.0–11.0] (mild neutrophilia)
• Platelets 390 ×10⁹/L [150–400]
• Ferritin 95 µg/L [30–300]
• HbA₂ 5.2% [1.8–3.5]; HbF 1.2% [0–2.0]
• HbH preparation: no HbH bodies; electrophoresis: no abnormal bands

The most likely explanation for his anaemia is:

A 21-year-old Filipino woman is referred for investigation of anaemia.

• Haemoglobin 81 g/L [113–159]
• Haematocrit 0.27 [0.36–0.46]
• Red cell count 4.58 ×10¹²/L [3.60–5.30]
• White cell count 4.60 ×10⁹/L [4.0–11.0]
• Platelets 421 ×10⁹/L [150–400]
• MCV 59 fL [80–100]
• MCH 17.7 pg [28.0–34.0]
• MCHC 300 g/L [320–360]
• Reticulocytes 142 ×10⁹/L [20–105]
• Ferritin 482 µg/L [15–200]
• HbA₂ 1.4% [1.8–3.5]; HbF 0.3% [0–2.0]

Which of the following is the most likely diagnosis?

Which of these haemoglobins will be synthesised at a reduced rate in α-thalassaemia?

Which of these haemoglobins will be synthesised at a reduced rate in β-thalassaemia?

One parent has α-thalassaemia trait with two deletions in cis (–/αα); the other is a silent carrier with a single deletion (-α/αα).

What is the likelihood (%) of a child having HbH disease?

A 69-year-old woman on a chronic transfusion programme for myelodysplasia experiences a non-haemolytic febrile transfusion reaction during each of two red-cell transfusions four weeks apart.

Filtered (leucodepleted) blood would reduce her risk of further reaction by reducing exposure to:

A 23-year-old woman presents with several days of petechiae of the skin and oral mucosa. No infection or medication other than the oral contraceptive pill. She is well; examination is unremarkable apart from petechiae.

• Haemoglobin 125 g/L [120–155]
• Haematocrit 0.34 [0.36–0.46]
• MCV 83 fL [80–95]
• White cell count 7.5 ×10⁹/L [4.0–11.0], normal differential
• Platelets 11 ×10⁹/L [150–400]
• Prothrombin time normal; APTT normal
• ANA negative; HIV serology negative

Which of the following is the most appropriate treatment?

A 78-year-old man presents with progressive lethargy over several months and recent bruising, epistaxis, headaches and visual deterioration.

He is anaemic with scattered ecchymoses; visual acuity is reduced bilaterally and fundoscopy shows retinal venous distension with numerous retinal haemorrhages.

• Haemoglobin 72 g/L [135–170]
• MCV 101 fL [80–95]
• White cell count 4.0 ×10⁹/L [4.0–11.0]
• Neutrophils 1.2 ×10⁹/L [2.0–8.0]
• Platelets 90 ×10⁹/L [150–400]
• PT-INR 1.5 [0.9–1.3]; APTT 45 s [26–38]
• Urea 12.5 mmol/L [3.0–8.0]; creatinine 150 µmol/L [60–110]
• Liver function and calcium normal
• Protein electrophoresis: hypogammaglobulinaemia with raised IgM-κ paraprotein 60 g/L

What is the most appropriate initial therapy?

Which of the following would you expect to find in a patient with pure red cell aplasia?

What would you expect to see on the peripheral blood smear of a patient with autoimmune haemolytic anaemia?

A 30-year-old asymptomatic woman is found to have a platelet count of 750 ×10⁹/L on routine testing. She is otherwise fit and well, and no secondary cause for the thrombocytosis has been identified after appropriate investigation.

Which of the following is the most appropriate management?

A 30-year-old woman undergoing induction chemotherapy for AML has epistaxis that will not stop with local measures.

• Haemoglobin 89 g/L [115–155]
• Platelets 12 ×10⁹/L [150–400]
• INR 1.2 [0.9–1.3]
• APTT 45 s [26–38]
• Thrombin clotting time 32 s [<24]
• Fibrinogen 5.2 g/L [2.0–4.0]

Which of the following is the most appropriate management?

Cryoprecipitate, when used as a first-line replacement product, is most useful in the treatment of which of the following?

Within two hours of vigorous exercise, a 20-year-old man presents with sudden severe left lateral lower chest pain, dyspnoea and anxiety. He is febrile and anaemic with local tenderness over the left lower ribs.

• Haemoglobin 75 g/L [130–180]
• MCV 81 fL [80–100]
• White cell count 13.6 ×10⁹/L [4.0–11.0]
• Neutrophils 11.2 ×10⁹/L [2.0–8.0]
• Platelets 180 ×10⁹/L [150–400]
• Reticulocytes 200 ×10⁹/L [9–116]

Blood film: sickle-shaped red cells.

The most likely diagnosis is: