Haematology Module 4

Which of the following is NOT part of the intrinsic pathway in the coagulation cascade?

Factor VII

Correct Incorrect Correct answer

Factor VIII

Correct Incorrect Correct answer

Factor IX

Correct Incorrect Correct answer

Factor XI

Correct Incorrect Correct answer

Factor XII

Correct Incorrect Correct answer

A 16 year old girl receiving chemotherapy for acute myeloid leukaemia presents in the early hours of Sunday morning with fever, bruising, petechiae and oral mucosal bleeding. Her platelet count is 4 x 109/L [150-400] and her blood group is Group A Rh negative. Platelet transfusion is indicated.

The transfusion service does not have irradiated Group A Rh negative platelets available.

Which of the following products would be most appropriate to request as an alternative?

Irradiated Group O Rh negative single donor platelets

Correct Incorrect Correct answer

Irradiated Group A Rh positive pooled donor platelets

Correct Incorrect Correct answer

Irradiated Group A Rh positive single donor platelets

Correct Incorrect Correct answer

Irradiated Group O Rh negative pooled donor platelets.

Correct Incorrect Correct answer

Unirradiated Group A Rh negative single donor platelets

Correct Incorrect Correct answer

Clotting factor deficiencies are most likely to result in spontaneous hemorrhage into which tissue?

joint

Correct Incorrect Correct answer

skin

Correct Incorrect Correct answer

nasal

Correct Incorrect Correct answer

muscle

Correct Incorrect Correct answer

biliary

Correct Incorrect Correct answer

Which one of the following cytotoxic agents acts by inhibiting dihydrofolate reductase and thymidylate synthesis?

Methotrexate

Correct Incorrect Correct answer

Vincristine

Correct Incorrect Correct answer

Bleomycin

Correct Incorrect Correct answer

Cyclophosphamide

Correct Incorrect Correct answer

Doxorubicin

Correct Incorrect Correct answer

An 18 year old man is admitted to hospital after developing a haemarthrosis in his right knee whilst playing football in the garden. The following blood results are obtained:

Platelets	220 * 10⁹/l
PT	11 secs
APTT	76 secs
Factor VIIIc activity	Normal

What is the most likely diagnosis?

Haemophilia B

Correct Incorrect Correct answer

Antithrombin III deficiency

Correct Incorrect Correct answer

Von Willebrand's disease

Correct Incorrect Correct answer

Antiphospholipid syndrome

Correct Incorrect Correct answer

Haemophilia A

Correct Incorrect Correct answer

A 62-year-old male attends the Emergency Department with a severe nose bleed. He is known to have alcoholic cirrhosis. His investigations reveal:

haemoglobin 10.9 g/dL (13.0-18.0)
white cell count 5 x 109/L (4-11)
platelet count 60 x 109/L ( 150-400)
prothrombin time 17.5s (11.5-15.5)
APPT 42s (30-40)
fibrinogen 0.7 g/L (1.8-5.4)

What is the most appropriate blood product for this patient?

Cryoprecipitate

Correct Incorrect Correct answer

Platelet transfusion

Correct Incorrect Correct answer

Factor VIII

Correct Incorrect Correct answer

Prothrombin complex concentrate

Correct Incorrect Correct answer

Red blood cell transfusion

Correct Incorrect Correct answer

A surgical registrar rings you for an opinion regarding bridging anticoagulation therapy for a patient with atrial fibrillation (CHADS2 score of 2) who is about to undergo an elective bowel surgery tomorrow. The patient’s warfarin has been withheld for 5 days. What would you advise them to do?

No bridging anti-coagulation is required.

Correct Incorrect Correct answer

Start IV Heparin and stop 2 hours prior to surgery.

Correct Incorrect Correct answer

Start a LMWH (Dalteparin) and stop 6 hours prior to surgery.

Correct Incorrect Correct answer

Start LMWH (Dalteparin) 24 hours prior to surgery and stop immediately preoperatively.

Correct Incorrect Correct answer

Start IV Heparin and stop 30 minutes prior to surgery

Correct Incorrect Correct answer

Which one of the following is least likely to cause a warm autoimmune haemolytic anaemia?

Mycoplasma infection

Correct Incorrect Correct answer

Methyldopa

Correct Incorrect Correct answer

Chronic lymphocytic leukaemia

Correct Incorrect Correct answer

Lymphoma

Correct Incorrect Correct answer

Systemic lupus erythematous

Correct Incorrect Correct answer

A 33-year-old male patient with a history of recurrent nose bleeds, iron deficiency anaemia and dyspnoea is found to have a pulmonary AV malformation on pulmonary angiography. What is the likely underlying diagnosis?

Hereditary haemorrhagic telangiectasia

Correct Incorrect Correct answer

Haemophilia A

Correct Incorrect Correct answer

Mantle cell lymphoma

Correct Incorrect Correct answer

Wegener's granulomatosis

Correct Incorrect Correct answer

Down's syndrome

Correct Incorrect Correct answer

A blood film is reported as follows:

Howell-Jolly bodies, target cells and occasional Pappenheimer bodies are seen.

What is the most likely underlying cause?

Post-splenectomy

Correct Incorrect Correct answer

Iron-deficiency anaemia

Correct Incorrect Correct answer

Lead poisoning

Correct Incorrect Correct answer

Myelofibrosis

Correct Incorrect Correct answer

Sideroblastic anaemia

Correct Incorrect Correct answer

A 22-year-old man with sickle cell anaemia presents with pallor, lethargy and a headache. Blood results are as follows:

Hb	46 g/l
Reticulocytes	3%

Infection with a parvovirus is suspected. What is the likely diagnosis?

Aplastic crisis

Correct Incorrect Correct answer

Thrombotic crisis

Correct Incorrect Correct answer

Sequestration crisis

Correct Incorrect Correct answer

Transformation to myelodysplasia

Correct Incorrect Correct answer

Haemolytic crisis

Correct Incorrect Correct answer

Which one of the following is NOT a feature of paroxysmal nocturnal haemoglobinuria?

Haemarthrosis

Correct Incorrect Correct answer

Haemolytic anaemia

Correct Incorrect Correct answer

Esophageal spasm

Correct Incorrect Correct answer

Haemoglobinuria

Correct Incorrect Correct answer

Dark urine

Correct Incorrect Correct answer

A 21-year-old man comes for review. He recently had an abdominal ultrasound for episodic right upper quadrant pain which demonstrated gallstones. A full blood count was also ordered which was reported as follows:

Hb	98 g/l
MCV	91 fl
Plt	177 x 10⁹/l
WBC	5.3 x 10⁹/l

The patient also mentions that his father had a splenectomy at the age of 30 years.

Which one of the following tests is most likely to be diagnostic?

Osmotic fragility test

Correct Incorrect Correct answer

Ham's test

Correct Incorrect Correct answer

PAS staining of erythrocytes

Correct Incorrect Correct answer

Glucose-6-phoshate dehydrogenase levels

Correct Incorrect Correct answer

Direct Coombs' test

Correct Incorrect Correct answer

A 42-year-old female is noted to have a Hb of 178 g/L. Which one of the following is least likely to be the cause?

Haemochromatosis

Correct Incorrect Correct answer

Polycythaemia rubra vera

Correct Incorrect Correct answer

Chronic obstructive pulmonary disease

Correct Incorrect Correct answer

Hypernephroma

Correct Incorrect Correct answer

Dehydration

Correct Incorrect Correct answer

Which one of the following is considered a high risk feature in acute lymphoblastic leukaemia?

Philadelphia chromosome positive

Correct Incorrect Correct answer

Pre-B phenotype

Correct Incorrect Correct answer

Presentation in childhood

Correct Incorrect Correct answer

Initial white cell count of 18 x 10^9/l

Correct Incorrect Correct answer

Female sex

Correct Incorrect Correct answer

A 34-year-old man who is HIV positive is starting treatment for Burkitt’s lymphoma. His chemotherapy regime includes cyclophosphamide, vincristine, methotrexate and prednisolone. Around 24 hours after starting chemotherapy he becomes confused and complains of muscle cramps in his legs. Which one of the following is most likely to have occurred?

Tumour lysis syndrome

Correct Incorrect Correct answer

Prednisolone-induced psychosis

Correct Incorrect Correct answer

Hypercalcaemia

Correct Incorrect Correct answer

Methotrexate pneumonitis leading to hypoxia

Correct Incorrect Correct answer

Haemorrhagic cystitis leading to acute renal failure

Correct Incorrect Correct answer

A 66-year-old woman with a history of chronic kidney disease stage disease 4 metastatic breast cancer is admitted with a swollen right calf. Investigations confirm a deep vein thrombosis and she is started on treatment dose dalteparin. As she has a significant degree of renal impairment it is decided to monitor her response to dalteparin. What is the most appropriate blood test to perform?

Anti-Factor Xa levels

Correct Incorrect Correct answer

Antithrombin III levels

Correct Incorrect Correct answer

Anti-Factor VIIIa levels

Correct Incorrect Correct answer

Protrombin time (PT)

Correct Incorrect Correct answer

Activated Partial Thromboplastin Time (APTT)

Correct Incorrect Correct answer

A 72-year-old woman is found to have a marked lymphocytosis associated with smudge cells on the blood film. A diagnosis of chronic lymphocytic leukaemia is suspected. Which one of the following is the investigation of choice?

Immunophenotyping

Correct Incorrect Correct answer

Bone marrow aspiration

Correct Incorrect Correct answer

Protein electrophoresis

Correct Incorrect Correct answer

White cell scan

Correct Incorrect Correct answer

Bone marrow trephine

Correct Incorrect Correct answer

A 15-year-old man is noted to bleed excessively during an elective dental extraction. Following the procedure, examination reveals petechial skin haemorrhages. Blood results show:

Hb	123 g/l
Plt	255 x 10⁹/l
WBC	7.9 x 10⁹/l
PT	13.3 secs
APTT	39 secs
Factor VIII activity	87%

What is the most likely diagnosis?

Von Willebrand's disease

Correct Incorrect Correct answer

Disseminated intravascular coagulation

Correct Incorrect Correct answer

Idiopathic thrombocytopenic purpura

Correct Incorrect Correct answer

Haemophilia A

Correct Incorrect Correct answer

Haemophilia B

Correct Incorrect Correct answer

A 52-year-old woman with a history of hypothyroidism presents with lethargy and a sore tongue. Blood tests are reported as follows:

Hb	107 g/l
MCV	121 fl
Plt	177 x 10⁹/l
WBC	5.4 x 10⁹/l

Further tests are ordered:

Vitamin B12	64 ng/l (200-900 ng/l)
Folic acid	7.2 nmol/l (> 3.0 nmol/l)

What is the most appropriate management?

1000 mcg IM hydroxocobolamin administered one to three times per week or daily, followed by once weekly

Correct Incorrect Correct answer

1000 mcg of IM hydroxocobalamin once every 3 months

Correct Incorrect Correct answer

1000 mcg of IM hydroxocobalamin once every 2 months + folic acid 5mg od

Correct Incorrect Correct answer

Give folic acid 5mg od one week then recheck bloods

Correct Incorrect Correct answer

1000 mcg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months

Correct Incorrect Correct answer

A patient with a history of recurrent thromboembolic events develops a deep vein thrombosis despite full anticoagulation with heparin. Which one of the following causes of thrombophilia is associated with resistance to heparin?

Antithrombin III deficiency

Correct Incorrect Correct answer

Protein S deficiency

Correct Incorrect Correct answer

Protein C deficiency

Correct Incorrect Correct answer

Lupus anticoagulant

Correct Incorrect Correct answer

Activated protein C resistance

Correct Incorrect Correct answer

What is the mechanism of action of DDAVP in von Willebrand’s disease?

Induces release of von Willebrand's factor from endothelial cells

Correct Incorrect Correct answer

Prevents renal excretion of von Willebrand's factor

Correct Incorrect Correct answer

Promotes breakdown of large multimers

Correct Incorrect Correct answer

Inhibits breakdown of von Willebrand's factor

Correct Incorrect Correct answer

Acts as substitute carrier molecule for factor VIII

Correct Incorrect Correct answer

Which one of the following statements regarding the aetiology of venous thromboembolism (VTE) is correct?

Tamoxifen therapy increases the risk of VTE

Correct Incorrect Correct answer

Third generation combined oral contraceptive pills are safer than second generation ones

Correct Incorrect Correct answer

VTE develops in around 5% of patients with Goodpasture's syndrome

Correct Incorrect Correct answer

Female gender is a risk factor recurrent VTE

Correct Incorrect Correct answer

The second trimester of pregnancy is associated with a greater risk than the puerperium

Correct Incorrect Correct answer

A 72-year-old man is admitted with a deep vein thrombosis. He is normally fit and well but has recently lost weight. Blood tests reveal the following:

IgG	889 mg/dl (600-1300 mg/dl)
IgM	1674 mg/dl (50-330 mg/dl)
IgA	131 mg/dl (60-300 mg/dl)

What is the most likely diagnosis?

Waldenstrom's macroglobulinaemia

Correct Incorrect Correct answer

IgM paraproteinemia

Correct Incorrect Correct answer

Acute promyelocytic leukaemia

Correct Incorrect Correct answer

Antiphospholipid syndrome

Correct Incorrect Correct answer

Multiple myeloma

Correct Incorrect Correct answer

Which of the following is a cause of extravascular haemolysis?

Hereditary spherocytosis

Correct Incorrect Correct answer

Paroxysmal nocturnal haemoglobinuria

Correct Incorrect Correct answer

Disseminated intravascular coagulation

Correct Incorrect Correct answer

Mismatched blood transfusion

Correct Incorrect Correct answer

A 67-year-old man is diagnosed with myelofibrosis. What is the most common presenting symptom of myelofibrosis?

Severe fatigue

Correct Incorrect Correct answer

Anorexia and weight loss

Correct Incorrect Correct answer

Night sweats

Correct Incorrect Correct answer

Easy bruising

Correct Incorrect Correct answer

Splenomegaly

Correct Incorrect Correct answer

Which one of the following malignancies may be associated with Human T-cell lymphotropic virus type 1 (HTLV-1)?

Adult T-cell leukaemia

Correct Incorrect Correct answer

Colorectal cancer

Correct Incorrect Correct answer

Burkitt's lymphoma

Correct Incorrect Correct answer

Medullary thyroid cancer

Correct Incorrect Correct answer

Breast cancer

Correct Incorrect Correct answer

A 51-year-old female is referred to the haematology clinic with a haemoglobin of 192 g/l. She is a non-smoker. Her oxygen saturations on room air are 98% and she is noted to have mass in the left upper quadrant.

What is the most useful test to establish whether she has polycythaemia rubra vera?

JAK2 mutation screen

Correct Incorrect Correct answer

Bone marrow aspiration

Correct Incorrect Correct answer

Blood film

Correct Incorrect Correct answer

Red cell mass

Correct Incorrect Correct answer

Transferrin saturation

Correct Incorrect Correct answer

Haematology Module 4

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