Haematology Module 2

A 54-year-old male develops acute onset of hypoxemia and non-cardiogenic pulmonary edema within six hours after receiving a transfusion. A diagnosis of transfusion-related acute lung injury (TRALI) is suspected.

Which of the following reasons best explains why the incidence of TRALI is highest with plasma transfusions compared to red blood cells (RBCs) and platelets?

Plasma contains a greater volume of antileukocyte antibodies compared to RBCs and platelets.

Correct Incorrect Correct answer

Plasma has a higher concentration of potassium compared to RBCs and platelets.

Correct Incorrect Correct answer

Plasma contains higher levels of clotting factors, which contribute to pulmonary inflammation.

Correct Incorrect Correct answer

Plasma is more likely to be stored at colder temperatures, leading to increased risk of TRALI.

Correct Incorrect Correct answer

Plasma transfusions are more frequently administered in larger volumes than RBCs or platelets.

Correct Incorrect Correct answer

A 65-year-old male patient presents with fatigue, weight loss, and bone pain. Laboratory results show anemia, hypercalcemia, and renal impairment.

Serum protein electrophoresis (SPEP) is negative for monoclonal protein, but urine protein electrophoresis (UPEP) reveals a large amount of kappa light chains.

The patient’s urine albumin to creatinine ratio (ACR) is 30 mg/g, but the urine protein to creatinine ratio (PCR) is 300 mg/g.

Which of the following tests would be most useful to confirm the diagnosis of light chain myeloma?

Serum free light chain (FLC) assay

Correct Incorrect Correct answer

Bone marrow biopsy with immunohistochemistry

Correct Incorrect Correct answer

Kidney biopsy to confirm light chain cast nephropathy

Correct Incorrect Correct answer

Serum electrophoresis with immunofixation (IFE)

Correct Incorrect Correct answer

24-hour urine collection for protein quantification

Correct Incorrect Correct answer

A 62-year-old man with a history of heavy chain myeloma presents to the emergency department with a 2-day history of progressive abdominal pain, nausea, vomiting, and inability to pass stool or flatus. He has no prior abdominal surgeries.

On examination, he is afebrile, with a distended abdomen and high-pitched bowel sounds. Laboratory studies show mild anemia and normal renal function.

Abdominal CT reveals a segment of small bowel with circumferential wall thickening and luminal narrowing, but no evidence of external compression or adhesions.

Which of the following is the most appropriate next step in the diagnostic approach for this patient?

Consider direct small bowel involvement by plasma cell neoplasm as a potential cause of obstruction and pursue tissue diagnosis to confirm etiology

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Assume the obstruction is due to adhesions and manage conservatively

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Schedule urgent exploratory laparotomy without further evaluation

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Attribute symptoms to medication side effects and discharge the patient

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Initiate empiric antibiotics for presumed infectious enteritis

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How is multiple myeloma diagnosed?

Presence of at least 10% clonal bone marrow plasma cells and serum or urinary monoclonal protein + myeloma related organ dysfunction.

Correct Incorrect Correct answer

Presence of at least 10% clonal bone marrow plasma cells and serum or urinary monoclonal protein

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Presence of renal failure and bone pain

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Presence of at least 30% clonal bone marrow plasma cells and serum or urinary monoclonal protein + myeloma related organ dysfunction.

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Presence of punched out lesions on skeletal survey

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A 63 year old man has been newly diagnosed with multiple myeloma. Unfortunately, he is not a candidate for autologous stem-cell transplant. The hematologist plans to start him on a combination of Bortezomib, Melphalan, and Prednisone (BMP). Which of the following medication could be added to his treatment in order to lower the risk of disease progression?

Daratumumab

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Ipilimumab

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Natalizumab

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Crizotinib

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Venetoclax

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A 71-year-old woman who is known to have multiple myeloma is admitted with confusion. Blood tests show the following:

Corrected calcium

2.91 mmol/l

Which one of the following is the most significant impact of her raised calcium level?

Increased osteoclastic activation

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Adverse effects of standard treatment

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Impaired renal function

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Increased renal tubular calcium reabsorption

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Elevated PTH-rP levels

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Which one of the following feature is least recognized in myeloma?

Night sweats

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Bone pain

Correct Incorrect Correct answer

Renal dysfunction

Correct Incorrect Correct answer

Anaemia

Correct Incorrect Correct answer

Hypercalcemia

Correct Incorrect Correct answer

Which transcription factors belonging to the Ikaros family are associated with the pathogenesis of multiple myeloma?

Ikaros (IKZF1) and Aiolos (IKZF3)

Correct Incorrect Correct answer

ETS1 and ETS2

Correct Incorrect Correct answer

FOXO1 and FOXO3

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IRF4 and IRF8

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At the time multiple myeloma is diagnosed, what is the most common presentation?

Anemia

Correct Incorrect Correct answer

bony lesions

Correct Incorrect Correct answer

bone pain

Correct Incorrect Correct answer

Renal impairment

Correct Incorrect Correct answer

infection

Correct Incorrect Correct answer

What is the most useful marker of prognosis in myeloma?

B2-microglobulin

Correct Incorrect Correct answer

Calcium level

Correct Incorrect Correct answer

Urine Bence-Jones protein levels

Correct Incorrect Correct answer

Alkaline phosphotase

Correct Incorrect Correct answer

ESR

Correct Incorrect Correct answer

A 30-year-old man is investigated for enlarged, painless cervical lymph nodes. A biopsy is taken and a diagnosis of Hodgkin’s lymphoma is made. Which one of the following types of Hodgkin’s lymphoma carries the worst prognosis?

Lymphocyte depleted

Correct Incorrect Correct answer

Lymphocyte predominant

Correct Incorrect Correct answer

Mixed cellularity

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Nodular sclerosing

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Hairy cell

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An 18-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 (10⁹/l)
WBC 7.9 (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

Transfusion Associated Circulatory Overload (TACO) and Transfusion Related Acute Lung Injury (TRALI) are both rare complications that could occur during blood transfusion but carries a significant mortality risk. However, in both situation, patient may present with similar signs and symptoms such as shortness of breath, increased respiratory rate and hypoxemia.

Which of the following features would be more in keeping with TACO rather than TRALI?

Good response to diuresis

Correct Incorrect Correct answer

Moderate hypotension

Correct Incorrect Correct answer

Onset within 6 hours of transfusion

Correct Incorrect Correct answer

Hypoxemia on arterial blood gas

Correct Incorrect Correct answer

Low BNP <250 pg/ml

Correct Incorrect Correct answer

Which one of the following would most suggest a leukaemoid reaction rather than chronic myeloid leukaemia?

Dohle bodies in the white cells

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Raised packed cell volume

Correct Incorrect Correct answer

Right shift of neutrophils

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A low leucocyte alkaline phosphatase score

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Positive osmotic fragility test

Correct Incorrect Correct answer

A patient presents with nephrotic syndrome, postural hypotension, raised INR and macroglossia.

What is the diagnosis?

Light-chain Amyloidosis

Correct Incorrect Correct answer

Multiple myeloma

Correct Incorrect Correct answer

MGUS

Correct Incorrect Correct answer

POEMS syndrome

Correct Incorrect Correct answer

Waldentrom's gammaglobulinemia.

Correct Incorrect Correct answer

Which of the following is NOT involved in fibrinolysis?

Prothrombinase complex

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Plasmin

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tPA

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Plasminogen

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Plasminogen activator inhibitor (PAI)-1

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A 60 year old man is referred by a surgeon for assessment of a prolonged APTT that was detected at a pre-operative assessment for elective CABG. His past medical history includes rheumatoid arthritis and benign prostatic hypertrophy. There is no past history of bleeding. His coagulation profile as listed below:

APTT 45secs (22-38secs)
PT 14 (13-17secs)
Thrombin clotting time 12secs (11-18 secs)
APTT mixing studies 34 secs

What should you advise the surgeon to do?

Suggest check factor VIII levels

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No further investigation required and patient may proceed with surgery

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Suggest check factor VII levels

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Suggest check factor X levels

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Repeat the test as there is likely a heparin contamination.

Correct Incorrect Correct answer

What is the role of DDAVP in patients with von Willebrands disease?

Increased vWF and FVIII levels

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Reduces the prothrombin time

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inhibits conversion of plasminogen to plasmin.

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reduces the renal clearance of clotting factors

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initiates coagulation by increasing tissue factor release

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A 20 year old woman has steroid refractory immune thrombocytopenia (ITP) relapsing after splenectomy with a platelet count below 20.She is commenced on a thrombopoeitin receptor agonist (romiplostim or eltrombopag) and her platelets rise rapidly to 220.

Which of the following is the most likely reason for this?

Increased platelet production in the marrow

Correct Incorrect Correct answer

Depletion of autoreactive B cells

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Prolonged platelet survival

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Inhibition of platelet phagocytosis by macrophages

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Reduced levels of circulating antiplatelet antibodies

Correct Incorrect Correct answer

Which of the following medication is most likely to enhance the effect of warfarin?

Erythromycin

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Carbamazepine

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Phenytoin

Correct Incorrect Correct answer

Vitamin K

Correct Incorrect Correct answer

Oral contraceptive.

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In a woman with antiphospholipid syndrome and recurrent pregnancy loss, what is the most appropriate treatment that will increase the chance for her of having a successful pregnancy?

Aspirin and low molecular weight heparin (LMWH)

Correct Incorrect Correct answer

Aspirin only

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Warfarin

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Aspirin and warfarin

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Aspirin and corticosteroid.

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A patient is found to have persistently elevated antiphospholipid antibodies (aPL) but is otherwise asymptomatic with no previous history of thrombosis and no concerning risk factors for thrombosis either.

What medication should be used for prophylaxis against primary thrombosis?

No prophylaxis required

Correct Incorrect Correct answer

Low dose aspirin

Correct Incorrect Correct answer

Warfarin

Correct Incorrect Correct answer

Low molecular weight heparin

Correct Incorrect Correct answer

Aspirin and warfarin

Correct Incorrect Correct answer

Which of the following agent is most effective in the treatment of myelodysplastic syndrome with chromosome 5q syndrome?

Lenalidomide

Correct Incorrect Correct answer

Thalidomide

Correct Incorrect Correct answer

High dose prednisone

Correct Incorrect Correct answer

Intravenous Immunoglobulin

Correct Incorrect Correct answer

Imatinib

Correct Incorrect Correct answer

What is the most common cause of familial thrombophilia?

Factor V leiden

Correct Incorrect Correct answer

Anti-thrombin III deficiency

Correct Incorrect Correct answer

Protein C deficiency

Correct Incorrect Correct answer

Protein S deficiency

Correct Incorrect Correct answer

Prothrombin gene mutation

Correct Incorrect Correct answer

A 42 year old female has just been diagnosed with unprovoked right leg deep venous thrombosis (DVT). Which of the following condition (if present), would require you to provide her with lifelong anti-coagulation?

Presence of antiphospholipid syndrome

Correct Incorrect Correct answer

Presence of factor V leiden mutation

Correct Incorrect Correct answer

Presence of von Willebrands disease

Correct Incorrect Correct answer

Presence of homocystenemia

Correct Incorrect Correct answer

Presence of prothrombin gene mutation

Correct Incorrect Correct answer

All of the following intervention are appropriate for patients who are deemed to have lower-risk myelodysplastic syndrome (MDS) except?

Lenalidomide

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Luspatercept

Correct Incorrect Correct answer

Azacitidine

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Red cell transfusions

Correct Incorrect Correct answer

Allogeneic hematopoietic cell transplantation (HCT)

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Which of the following coagulation abnormalities is resistant to the effect of Heparin?

Anti-thrombin deficiency

Correct Incorrect Correct answer

Factor V leiden

Correct Incorrect Correct answer

Prothrombin gene mutation

Correct Incorrect Correct answer

Protein C deficiency

Correct Incorrect Correct answer

Protein S deficiency

Correct Incorrect Correct answer

All the following are vitamin K dependent coagulation factors except:

factor VIII

Correct Incorrect Correct answer

factor X

Correct Incorrect Correct answer

factor VII

Correct Incorrect Correct answer

protein C

Correct Incorrect Correct answer

protein S

Correct Incorrect Correct answer

A 60-year-old man presents to the emergency department with chest pain and hypotension.

His full blood examination shows:

haemoglobin 125 g/L [128-175]
white cell count 10.2 x 10⁹/l [3.9-12.7] with normal white cell differential.
platelets 100 x 10⁹/l [150-396]
Coagulation investigations show:
APTT 100 seconds [26-38]
APTT 1:1 mix (patient: normal plasma) 52 seconds
prothrombin time-international normalised ratio (PT-INR) 1.3 [1.0-1.3]
thrombin clotting time (TCT) 200 seconds [<24]
fibrinogen 0.9 g/L [2.0-4.0]
D-dimer 5000 ng/ml

What is the most likely diagnosis?

administration of unfractionated heparin.

Correct Incorrect Correct answer

disseminated intravascular coagulation

Correct Incorrect Correct answer

administration of low-molecular weight heparin.

Correct Incorrect Correct answer

administration of thrombolytic agent.

Correct Incorrect Correct answer

lupus inhibitor

Correct Incorrect Correct answer

A 67 year old man undergoes aortic valve replacement and is commenced on warfarin in the post operative period. Five days after commencing warfarin he develops a painful burning rash over his buttocks with evidence of skin necrosis.

Which of the following disorder is he likely to have?

Protein C deficiency

Correct Incorrect Correct answer

Anti-thrombin III deficiency

Correct Incorrect Correct answer

Hyperviscosity syndrome

Correct Incorrect Correct answer

Protein S deficiency

Correct Incorrect Correct answer

Vitamin K deficiency

Correct Incorrect Correct answer

A 21-year-old woman presents with recent-onset of right leg swelling and pleuritic chest pain and is found to have iliofemoral thrombosis on Doppler ultrasound examination.

Results of a ventilation-perfusion lung scan indicate a high probability for pulmonary emboli.

She had been taking the oral contraceptive pill for the last three years but has now ceased. She is a non-smoker. There is no significant medical history and no known family history of venous thromboembolism.

A panel of thrombophilia screen is performed.

In the search for a hypercoagulable state, which of the following will most likely be affected given the presence of extensive thrombosis?

Antithrombin level.

Correct Incorrect Correct answer

Antiphospholipid antibody screen

Correct Incorrect Correct answer

Factor V and prothrombin genotype assessment.

Correct Incorrect Correct answer

Activated partial thromboplastin time (APTT).

Correct Incorrect Correct answer

Full blood count including blood film.

Correct Incorrect Correct answer

Besides its anti-coagulant effect, Warfarin also exerts anti-thrombotic effects via depletion of which of the following?

Factor II

Correct Incorrect Correct answer

Factor VII

Correct Incorrect Correct answer

Factor IX

Correct Incorrect Correct answer

Factor X

Correct Incorrect Correct answer

Factor XI

Correct Incorrect Correct answer

A 50-year-old businessman presents to your office for an insurance examination. He smokes a pack of cigarettes a day, is moderately overweight and has a history of hypertension treated with hydrochlorothiazide. Except for some job-related stresses, he feels well. Systems review is normal.

Findings on physical exam are as follows: BP, 155/90; P, 92; T, 37.0 C; wt, 195 lb.

Plethoric facies are noted, but examination of the heart, lungs, and abdomen is normal.

Laboratory results are as follows:

Hb 185
HCT 55
WBC 6.8 x 10⁹/l with normal differential
peripheral blood smear, normal
Na+, 140
K+, 3.2
Cr, 1.5
COHb, 4.2
RBC volume 31 (upper limits of normal)
plasma volume 36 (low normal)
Pao2, 94
Paco2, 42
pH, 7.41
HCO3, 24
Hb P50, normal
erythropoietin, 25.

Which of the following is the most likely diagnosis?

Relative polycythemia

Correct Incorrect Correct answer

Polycythemia vera

Correct Incorrect Correct answer

Renal cell carcinoma

Correct Incorrect Correct answer

Obstructive sleep apnea

Correct Incorrect Correct answer

High-affinity hemoglobinopathy

Correct Incorrect Correct answer

A 78-year-old man presents for evaluation of progressive headache, dizziness, and weakness of 6 months’ duration.

Hot showers cause generalized pruritus. The past medical history and systems review are otherwise normal. He had a normal health maintenance examination 5 years ago, including routine laboratory studies.

Physical examination shows the following: BP, 130/70; P, 70; T, 37.0 C; wt, 165 lb. The skin of the face is plethoric and dusky red. Heart and lungs are normal. A spleen tip is palpable 2 cm below the costal margin.

There is no lymphadenopathy. The rest of the examination is normal.

Laboratory results are as follows:

Hb 195
HCT 0.65
WBC, 12.8 with normal differential
platelets, 525 x 10⁹/l
peripheral blood smear, increased basophils, myelocytes, and metamyelocytes
Na+, 140
K+, 4.0
Cr, 1.0
uric acid, 10.5
Pao2, 94
Paco2, 42
pH, 7.41
HCO3, 24
erythropoietin 5

Which of the following is the most likely diagnosis?

Polycythemia vera

Correct Incorrect Correct answer

Relative polycythemia

Correct Incorrect Correct answer

Renal cell carcinoma

Correct Incorrect Correct answer

Hypoxic polycythemia

Correct Incorrect Correct answer

High-affinity hemoglobinopathy

Correct Incorrect Correct answer

Haematology Module 2

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