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?

How is multiple myeloma diagnosed?

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?

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

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

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

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

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

What is the most useful marker of prognosis in myeloma?

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?

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?

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?

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

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

What is the diagnosis?

Which of the following is NOT involved in fibrinolysis?

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?

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

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?

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

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?

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?

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

What is the most common cause of familial thrombophilia?

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?

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

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

All the following are vitamin K dependent coagulation factors except:

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?

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?

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?

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

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?

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?