Haematology

Question 1

A 22 year old lady presenting with fatigue, menorrhagia and a microcytic anaemia.

Answer 1

Iron deficiency anaemia

Question 2

A 77 year old asymptomatic man is found to have a lymphocytosis.

Answer 2

The only option above that is associated with a lymphocytosis is chronic lymphocytic leukaemia, that is a clonal (neoplastic) disorder of B cells. Many patients will have asymptomatic disease.

Question 3

A 67 year old man admitted to hospital with back pain, hypercalcemia and renal failure is found to have significant Bence Jones proteinuria.

Answer 3

Bence Jones proteins are found in up to 75% of patients with myeloma and in the urine, reflect the excretion of (clonal ie kappa or lambda only)immunoglobulin light chains.

Question 4

Uncontrolled production of essentially normally functioning blood cells

Answer 4

Polycythaemia rubra vera

The red cells production is uncontrolled (unlike D where it is controlled by high epo driven by the secondary cause eg hypoxia). The result is an excess of normally functioning red cells. In E CLL cells have no useful function.

Question 5

2. Uncontrolled production of immature blood cells in the bone marrow

Answer 5

Acute myeloid leukaemia; AML is a bone marrow based malignancy where there is a block in differentiation and an excess of primitive cells accumulate rapidly.

Question 6

3. Clonal B cell disorder usually resulting in a large number of circulating malignant cells

Answer 6

CLL is a chronic leukaemia so unlike acute leukaemia there is not a block on early differentiation but failure of cell death and so a steady accumulation of cells over time. They are produced in bone marrow and seen circulating as an excess of small mature lymphocytes in the blood (often with smear cells seen on a blood film). Diagnosis is confirmed by peripheral blood immunophenotyping.

Question 7

A 28 year old woman attending the antenatal clinic has a blood count performed with the results as follows: Haemoglobin low , MCV normal fl, white cell count normal neutrophil count normal and platelet count norml Haemoglobin analysis identifies a high level of Hb S without detectable HbA.

Answer 7

The detection of HbS (glutamic acid to valine substitution in the beta chain of haemoglobin) indicates that the patient has sickle cell anaemia (homozygous). HbS would also have been detected if the patient had sickle trait (heterozygous), but HbA would also have been present. Also, the patient would not be expected to be anaemic on account of sickle trait.

Question 8

A 28 year old man has a blood count performed with the results as follows: Haemoglobin 152g/L, MCV 65 fl, white cell count 6.4 x 109/l, neutrophil count 3.8 x 109/l and platelet count 270 x 109/l. Haemoglobin analysis identifies the presence of raised Hb A2.

Question 9

An 18 year old man with fatigue and a family history of a haemolytic disorder has a blood count performed with the results as follows: Haemoglobin 82g/L, MCV 94 fl, white cell count 5.9 x 109/l, neutrophil count 3.2 x 109/l and platelet count 170 x 109/l. Blood film shows polychromasia and red cells with loss of central pallor.

Question 10

A 58 year old lady with a family history of hypothyroidism and atrophic gastritis presents with fatigue, macrocytosis and pancytopenia.

Question 11

A 25 year old male has recurrent admissions to hospitals with pain in his legs and chest wall. On one occasion, he became extremely breathless and required a red cell exchange transfusion.

Question 12

A 21 year old girl has a history of heavy periods and investigations indicate a defect in primary haemostasis. Her blood count is normal.

Question 13

A 52 year old lady with non-Hodgkin’s lymphoma is treated with Rituximab.

Question 14

A 77 year old man with chronic myeloid leukaemia is treated with Imatinib

Question 15

A 67 year old man with chest pain and ST-elevated myocardial infarction is treated with aspirin in the ambulance on the way to hospital.

Question 16

1. Sickle cell disease

Question 17

Myelodysplasia

Question 18

Hereditary Spherocytosis

Question 19

Defective hexose monophosphate shunt pathway

Question 20

A Cytosolic defect reducing haemoglobin synthesis

Question 21

Failure of globin synthesis

Question 22

A 68 year old woman with fatigue has a blood count performed and the results are as follows: Haemoglobin 87g/L, MCV 110 fl, white cell count 2.4 x 109/l, neutrophil count 1 x 109/l and platelet count 100 x 109/l. The blood film shows macroovalocytes and hypersegmented neutrophils.

Question 23

A 58 year old man with fatigue has a blood count performed and the results are as follows: Haemoglobin 77g/L, MCV 90 fl, white cell count 22.4 x 109/l, neutrophil count 0.1 x 109/l and platelet count 30 x 109/l. The blood film shows an excess of blasts with Auer rods.

Question 24

A 52 year old man with a stroke has a blood count performed and the results are as follows: Haemoglobin 140g/L, MCV 90 fl, white cell count 10.4 x 109/l, neutrophil count 6.8 x 109/l and platelet count 930 x 109/l. The blood film shows an excess of platelets with some giant forms.

Question 25

Evan’s syndrome

Question 26

Fanconi’s anaemia

Question 27

Autoimmune thrombocytopenia

Question 28

Which of the following statements regarding Vitamin K is true Question 10Select one: a. Vitamin K is water soluble, absorbed in the upper intestine, responsible for bile salt absorption, carboxylates specific preformed clotting factors b. Vitamin K is water soluble, absorbed in the upper intestine, responsible for bile salt absorption, is antagonised by warfarin c. Vitamin K is water soluble, absorbed in the upper intestine, carboxylates specific preformed clotting factors, is antagonised by warfarin d. Vitamin K is water soluble, responsible for bile salt absorption, carboxylates specific preformed clotting factors, is antagonised by warfarin e. Vitamin K is absorbed in the upper intestine, responsible for bile salt absorption, carboxylates specific preformed clotting factors, is antagonised by warfarin

Question 29

A 28 year old woman attending the antenatal clinic has a blood count performed with the results as follows: Haemoglobin 112g/L, MCV 65 fl, MCH 20 pg, white cell count 6.4 x 109/l, neutrophil count 3.8 x 109/l and platelet count 170 x 109/l. The serum ferritin is within the normal range.

Question 30

A 52 year-old asymptomatic, fit man with no significant medical history has a blood count performed with the results as follows: Haemoglobin 200g/L, MCV 81 fl, white cell count 11 x 109/l, neutrophil count 7.2 x 109/l and platelet count 402 x 109/l. Blood film confirms the blood count abnormalities.

Question 31

A 21 year-old man with fatigue and easy bruising has a blood count performed with the results as follows: Haemoglobin 8.0g/L, MCV 92 fl, white cell count 50 x 109/l, neutrophil count 0.2 x 109/l and platelet count 40 x 109/l. The blood film shows an excess of blasts.

Question 32

Normal prothrombin time, prolonged partial thromboplastin time, normal platelet count, normal fibrinogen.

Question 33

Prolonged prothrombin time, normal partial thromboplastin time, normal platelet count, normal fibrinogen.

Question 34

Prolonged prothrombin time, prolonged partial thromboplastin time, low platelet count, low fibrinogen.

Question 35

1. It is an ADP antagonist

Question 36

2. It irreversibly inactivates cyclooxygenase 1

Question 37

3. It is a highly selective direct inhibitor of activated factor X

Question 38

Warfarin

Question 39

Dipyridamole

Question 40

Abciximab

Question 41

Dabigitran

Question 42

1. Temperature 37.8C pulse 90 bpm, two hours after starting a blood transfusion. Otherwise well with normal blood pressure.

Question 43

2. 92 year old woman who is breathless after receiving a 4 unit red cell transfusion for chronic anaemia.

Question 44

3. 48 year old man becoming breathlessness two hours after an infusion of fresh frozen plasma.

Question 45

Which of the following are causes of hereditary thrombophilia Question 15Select one: a. Factor V Leiden, antiphospholipid syndrome, antithrombin deficiency, protein C deficiency b. Factor V Leiden, antiphospholipid syndrome, antithrombin deficiency, protein S deficiency c. Factor V Leiden, antiphospholipid syndrome, protein C deficiency, protein S deficiency d. Factor V Leiden, antithrombin deficiency, protein C deficiency, protein S deficiency e. Antiphospholipid syndrome, antithrombin deficiency, protein C deficiency, protein S deficiency

Question 46

An 18 year old woman with heavy periods has a blood count performed and the results are as follows: Haemoglobin 67g/L, MCV 69 fl, white cell count 6.4 x 109/l, neutrophil count 4.2 x 109/l and platelet count 495 x 109/l. Serum ferritin is low. She is relatively asymptomatic from her anaemia.

Question 47

A 52 year old man has a blood count performed for investigation of mild fatigue: Haemoglobin 100g/L, MCV 94 fl, white cell count 6.4 x 109/l, neutrophil count 4.2 x 109/l and platelet count 295 x 109/l. There is a reticulocytosis and serum lactate dehydrogenase is raised. Direct antiglobulin test (Coombs’ test) is strongly positive.

Question 48

A 67 year old lady has recently completed intensive chemotherapy for acute myeloid leukaemia. She is fatigued and short of breath on minimal exertion. Haemoglobin 78g/L, MCV 94 fl, white cell count 0.2 x 109/l, neutrophil count 0.1 x 109/l and platelet count 25 x 109/l.