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DFT - EMREE - HEMATOLOGY - 03-10-2025

Total questions: 10

Worksheet time: 10mins

Name
Class
Date
1.

An 82-year-old male with a history of chronic kidney disease and heart failure is admitted for a lower gastrointestinal bleed. His haemoglobin is 6.8 g/dL, and a transfusion of two units of packed red blood cells is initiated. Towards the end of the second unit, the nurse calls you to the bedside because the patient has developed acute shortness of breath. His oxygen saturation has dropped from 96% to 88% on room air. Physical examination reveals bilateral basal crackles, which were not present previously. His blood pressure is 170/95 mmHg, heart rate is 110/min, and his jugular venous pressure is visibly elevated. Which of the following laboratory findings would be the most specific indicator for confirming the suspected diagnosis?

a)

Elevated D-dimer

b)

Markedly elevated Brain Natriuretic Peptide (BNP)

c)

Presence of anti-HLA antibodies in donor plasma

d)

Decreased peripheral neutrophil count

e)

Positive direct Coombs test

2.

A 29-year-old G2P1 woman with a known O-negative blood type delivers a male infant at 37 weeks of gestation via spontaneous vaginal delivery. Her first pregnancy two years ago was uncomplicated, and she does not recall receiving any injections after that delivery. Within 12 hours of birth, the neonate is noted to be lethargic with significant jaundice and pallor. Physical examination reveals hepatosplenomegaly. Initial laboratory results show a haemoglobin of 9.5 g/dL, a total bilirubin of 22 mg/dL (1.5 mg/dL direct), and a peripheral smear showing numerous spherocytes and nucleated red blood cells. The neonate's direct antiglobulin test (DAT) is strongly positive. The hemolysis observed in this neonate is most likely mediated by which of the following maternal antibodies that have crossed the placenta?

a)

Anti-A

b)

Anti-B

c)

Anti-D

d)

Anti-Lewis

e)

Anti-Kell

3.

A 28-year-old man of Southeast Asian origin undergoes a health check-up. He is found to have mild anaemia with a haemoglobin of 11.2 g/dL and a markedly low mean corpuscular volume (MCV) of 68 fL. His red cell distribution width (RDW) is normal at 13.0%. A full iron panel is ordered, which reveals a serum ferritin of 300 µg/L and a transferrin saturation of 45%. The physician suspects a hemoglobinopathy. Which of the following is the most appropriate next test to confirm the suspected diagnosis?

a)

Bone marrow biopsy

b)

Haemoglobin electrophoresis

c)

Serum vitamin B12 and folate levels

d)

Reticulocyte count

4.

A 12-hour-old neonate, born to an O-negative mother, is noted to have rapidly progressing jaundice and lethargy in the NICU. His blood type is confirmed to be O-positive, and his direct antiglobulin test (DAT) is positive. His total serum bilirubin is 28 mg/dL (480 µmol/L) with a direct bilirubin of 1.0 mg/dL. His haemoglobin has dropped to 7.0 g/dL. Despite being under intensive phototherapy for the past 4 hours, his bilirubin level continues to rise. What is the most appropriate next step in the management of this neonate?

a)

Administer a high dose of intravenous immunoglobulin (IVIG).

b)

Perform a simple packed red blood cell transfusion.

c)

Initiate double volume exchange transfusion.

d)

Administer phenobarbital to induce hepatic enzyme activity.

e)

Switch to triple bank phototherapy and recheck levels in 2 hours.

5.

A 47-year-old man, diagnosed with Chronic Myeloid leukaemia (CML) two years ago, is being followed up in the haematology clinic. He has been well-controlled on imatinib and is currently asymptomatic. A routine surveillance bone marrow aspirate and biopsy are performed. The biopsy confirms the presence of CML in the chronic phase with BCR-ABL1 positivity. However, it also reveals a distinct population of 40% mature-appearing lymphocytes. Flow cytometry of the aspirate shows these lymphocytes are monoclonal and express CD5, CD19, CD20, and CD23. Which of the following is the most likely additional diagnosis in this patient?

a)

Blast crisis of CML

b)

Prolymphocytic leukaemia

c)

Chronic Lymphocytic Leukaemia (CLL)

d)

Hairy cell leukaemia

e)

Reactive lymphocytosis

6.

A 25-year-old male of Mediterranean descent presents to the clinic with a lifelong history of fatigue and pallor. On examination, he has scleral icterus and a spleen palpable 6 cm below the left costal margin. Initial laboratory investigations reveal a haemoglobin of 8.5 g/dL, a Mean Corpuscular Volume (MCV) of 65 fL, and a Red Cell Distribution Width (RDW) of 13% (normal range 12-15%). A peripheral blood smear demonstrates hypochromia, microcytosis, target cells, and coarse basophilic stippling. Iron studies show a serum iron of 190 mcg/dL (high), ferritin of 800 ng/mL (high), and transferrin saturation of 75% (high). Which of the following investigations is the most specific next step to establish the definitive diagnosis?

a)

Bone marrow biopsy with Prussian blue stain

b)

Serum lead level

c)

Haemoglobin electrophoresis

d)

Coomb's test

e)

Reticulocyte count

7.

A 34-year-old woman presents to the clinic for advice regarding her risk of thrombosis. Her 58-year-old father and 38-year-old brother both have a history of unprovoked deep vein thrombosis (DVT). The patient herself is asymptomatic and has no significant past medical history. She is considering starting oral contraceptive pills for family planning. A thorough review of her family history reveals no other chronic illnesses, malignancies, or autoimmune disorders. She is concerned about her own risk and asks about potential underlying inherited conditions. What is the most common hereditary thrombophilia she is at risk for?

a)

Antithrombin III deficiency

b)

Protein C deficiency

c)

Factor V Leiden mutation

d)

Prothrombin G20210A gene mutation

e)

Antiphospholipid syndrome

8.

A 62-year-old female is diagnosed with Primary Myelofibrosis. She is experiencing debilitating fatigue, drenching night sweats, and symptomatic splenomegaly that causes significant abdominal discomfort and early satiety. Her risk stratification using the Dynamic International Prognostic Scoring System (DIPSS) places her in the intermediate-2 risk category. Her molecular testing is positive for a JAK2 V617F mutation. The decision is made to initiate therapy aimed at controlling her constitutional symptoms and reducing the size of her spleen. What is the most appropriate therapeutic agent to initiate for this patient's symptomatic disease?

a)

Hydroxyurea

b)

Imatinib

c)

Ruxolitinib

d)

Lenalidomide

e)

Allogeneic stem cell transplant

9.

A 31-year-old woman presents with persistent weakness. Her past medical history is significant for pulmonary tuberculosis treated six months ago. She is not taking any current medications. Laboratory results show a haemoglobin of 10.1 g/dL and an MCV of 72 fL. Iron studies are ordered and reveal a serum iron of 190 µg/dL, a TIBC of 200 µg/dL, and a serum ferritin of 800 ng/mL. A review of her peripheral blood smear confirms microcytic anaemia with target cells and coarse basophilic stippling. Sideroblastic anaemia is suspected. Which of the following investigations is the most specific for confirming the suspected diagnosis?

a)

Haemoglobin electrophoresis

b)

Serum vitamin B6 level

c)

Serum lead level

d)

Bone marrow biopsy with Prussian blue staining

e)

Reticulocyte count

10.

An 18-month-old boy is brought to the primary care clinic by his mother for a routine check-up. She expresses concern that he has been more irritable and less active than usual. His diet consists primarily of whole cow's milk (approximately 1 litre/day). On examination, he is pale. Initial lab results show a haemoglobin of 9.2 g/dL, an MCV of 68 fL, a high RDW of 18.0%, and a normal reticulocyte count. Iron deficiency anaemia is strongly suspected. Which of the following laboratory tests is the most specific for confirming the suspected diagnosis?

a)

Serum iron level

b)

Serum ferritin level

c)

Total iron-binding capacity (TIBC)

d)

Haemoglobin electrophoresis

e)

Peripheral blood smear