Hematology Practice Questions

Q: All-trans-retinoic acid is used in the treatment of:

Acute promyelocytic leukemia (APL). ***Acute promyelocytic leukemia (APL)*** - **All-trans-retinoic acid (ATRA)** is a cornerstone of APL treatment, targeting the specific **PML-RARα fusion protein** responsible for the disease [1]. - ATRA induces the differentiation of immature promyelocytes into mature granulocytes, thereby resolving the **leukemic block** in differentiation [1]. *Acute lymphoblastic leukemia (ALL)* - While ALL involves immature lymphocytes, ANTRA has no significant role in its treatment, which typically involves **multi-agent chemotherapy**, **corticosteroids**, and sometimes **targeted therapies** like tyrosine kinase inhibitors (for Ph+ ALL) [1]. - The mechanism of action of ATRA is specific to the myeloid lineage and the **PML-RARα fusion** [1]. *Chronic myeloid leukemia (CML)* - CML is characterized by the **BCR-ABL1 fusion gene** and is primarily treated with **tyrosine kinase inhibitors (TKIs)** like imatinib, nilotinib, or dasatinib. - ATRA does not target the molecular pathogenesis of CML and is not used in its standard treatment. *Transient myeloproliferative disorder (TMD)* - TMD, also known as transient abnormal myelopoiesis, is a myeloproliferative disorder seen in infants with **Down syndrome** and usually resolves spontaneously. - While it has myeloid features, ATRA is not a standard treatment; management is often **supportive** or involves low-dose chemotherapy if severe.

Q: An elderly patient is receiving a blood transfusion for anemia due to myelodysplastic syndrome (MDS). He was diagnosed with MDS two years ago and has required blood transfusions every six weeks for symptomatic anemia over the past six months. His past medical history also includes hypertension, type 2 diabetes, and coronary artery disease. Halfway through the transfusion of the second unit of packed red blood cells, he develops tachypnea, lumbar pain, tachycardia, and nausea. Which of the following is the most likely explanation?

hemolysis. ***hemolysis*** * The sudden onset of **tachypnea**, **lumbar pain**, and **tachycardia** during a blood transfusion is highly suggestive of an acute **hemolytic transfusion reaction**. Lumbar pain is a classic symptom due to **hemoglobinuria** and acute kidney injury [1]. * Patients with a history of frequent transfusions, like this patient with **myelodysplastic syndrome (MDS)**, are at increased risk for developing **alloantibodies** that can cause such reactions [1]. *anxiety* * While anxiety can cause **tachycardia** and **tachypnea**, it typically does not present with **lumbar pain** or **nausea** specifically during a transfusion. * Anxiety is a less specific diagnosis given the constellation of sudden, severe symptoms occurring precisely mid-transfusion. *fluid overload* * **Fluid overload** (TRALI) typically presents with signs of respiratory distress, such as **dyspnea**, **cough**, and **pulmonary edema**, but not typically with severe **lumbar pain**. * While a patient with underlying cardiovascular disease is at risk, the sudden onset of lumbar pain points away from isolated fluid overload. *pulmonary embolism* * **Pulmonary embolism (PE)** can cause tachypnea and tachycardia, but the presence of **lumbar pain** and **nausea** during a transfusion makes it a less likely primary diagnosis. * PE symptoms are generally more acutely focused on respiratory and cardiovascular compromise, often without the systemic symptoms seen here.

Q: Haemochromatosis affects all of the following organs except:

Salivary glands. ***Salivary glands*** - Haemochromatosis primarily affects **organs involved in iron metabolism**, such as the liver and pancreas, but has **no direct pathological involvement of salivary glands**. - Salivary glands are not known to accumulate iron significantly in haemochromatosis, thus they do not exhibit the same dysfunction seen in affected organs. *Heart* - The heart can be affected by **restrictive cardiomyopathy** due to iron overload, leading to heart failure or arrhythmias. - It may also exhibit **iron deposition**, which can adversely impact cardiac function in patients with haemochromatosis. *Liver* - The liver is the primary organ affected by haemochromatosis, leading to **cirrhosis**, **fibrosis**, and **hepatocellular carcinoma** due to excessive iron accumulation [1]. - Elevated liver enzymes and the risk of liver disease are hallmarks of this condition, making it a critical organ of concern [1]. *Pancreas* - The pancreas can suffer from **diabetes mellitus** due to iron deposition affecting insulin-producing cells, leading to **endocrine dysfunction** [1]. - Additionally, it can experience **exocrine insufficiency**, which is also associated with excessive iron levels in the body.

Q: A 60-year-old man presented to the emergency department with sudden-onset cough, yellow sputum production, and dyspnea, while taking amlodipine for hypertension. Chest X-ray revealed a left upper lobe alveolar infiltrate, and laboratory investigations showed Hb of 6 g/dL, BUN of 60 mg/dL, Creatinine of 2.8 mg/dL, Calcium of 12.3 mg/dL, total protein of 9 g/dL, and albumin of 4.2 g/dL. Echocardiogram revealed a dilated heart, and peripheral neuropathy was also present. What is the most common cause of death in patients with multiple myeloma?

Kidney failure. ***Kidney failure*** - **Renal impairment** is common in multiple myeloma due to **Bence-Jones proteinuria**, hypercalcemia, and amyloidosis, significantly contributing to morbidity and mortality. - The presented labs show elevated **BUN** (60 mg/dL) and **creatinine** (2.8 mg/dL), indicating significant kidney dysfunction, making it a highly likely cause of death in this context. *Infection* - While **infection** is a significant cause of death in multiple myeloma patients due to **immunodeficiency**, the immediate lab findings point more strongly to acute renal failure. - The patient's **cough** with **yellow sputum** and **alveolar infiltrate** suggest pneumonia, but other presented lab values are more indicative of advanced kidney damage. *Bleeding* - **Bleeding diathesis** can occur in multiple myeloma due to **platelet dysfunction** and **coagulation factor deficiencies**, but there is no direct evidence of severe bleeding in the provided case details. - The symptoms and lab findings provided do not directly support bleeding as the primary or most common cause of death in this patient's presentation. *CHF* - **Congestive heart failure (CHF)** can develop in multiple myeloma due to **amyloidosis** affecting the heart or due to severe anemia, which is present (Hb 6 g/dL). - While the echocardiogram revealed a **dilated heart**, the pronounced **renal failure** indicated by BUN and creatinine levels is a more direct and common cause of mortality in multiple myeloma patients.

Q: Which of the following is false about monoclonal gammopathy of unknown significance (MGUS)?

Does not progress to multiple myeloma. ***Does not progress to multiple myeloma*** - This statement is **false**, as Monoclonal Gammopathy of Unknown Significance (MGUS) is a **precursor condition** that can progress to multiple myeloma [1] or other plasma cell disorders in a small percentage of patients. - MGUS is characterized by the presence of a **monoclonal protein** without evidence of end-organ damage, but its malignant potential is why regular monitoring is crucial. *No evidence of Bence-Jones proteinuria* - This statement is **true** for MGUS, as the presence of significant **Bence-Jones proteinuria** (light chain spilling into urine) often indicates progression to multiple myeloma or other plasma cell dyscrasias [2]. - In MGUS, the amount of monoclonal protein is generally low, and **significant renal involvement** or light chain production is typically absent. *Bone marrow plasma cells less than 10%* - This statement is **true** for MGUS; the bone marrow must contain **less than 10% plasma cells** to meet the diagnostic criteria for MGUS [2]. - If plasma cells exceed 10% in the bone marrow, the diagnosis may shift towards **smoldering multiple myeloma** or **active multiple myeloma**, depending on other clinical findings. *Less than 3g/dL of monoclonal protein* - This statement is **true** for MGUS, as the serum **monoclonal protein (M-protein) concentration** must be **less than 3 g/dL** [2]. - A higher M-protein level, particularly above 3 g/dL, is a diagnostic criterion for **smoldering multiple myeloma** or **active multiple myeloma**.

Q: A 9-year-old boy presents with elevated PT and APTT. What is the most likely underlying condition?

Liver disease. ***Defect in common pathway*** - Elevation in both PT (Prothrombin Time) and APTT (Activated Partial Thromboplastin Time) indicates a dysfunction affecting the **common coagulation pathway** [1]. - Conditions such as Vitamin K deficiency or certain coagulation factor deficiencies (like factor II, V, X) would result in **both PT and APTT prolongation** [1]. *Defect in intrinsic pathway* - A defect in the intrinsic pathway typically leads to **prolonged APTT** while PT remains normal, which does not match this case [1]. - Intrinsic pathway defects involve factors such as **VIII, IX, XI**, and they wouldn't elevate PT. *Platelet function defect* - Platelet function defects mainly cause **bleeding disorders** but do not typically prolong PT or APTT; they result in **normal coagulation screening tests** [2]. - Symptoms usually involve **petechiae, purpura**, or excessive bleeding, distinct from coagulation pathway issues [2]. *Defect in extrinsic pathway* - A defect in the extrinsic pathway would primarily lead to a **prolonged PT**, with APTT remaining normal, which is contrary to the findings here [1]. - This pathway defect is usually related to **factor VII** and does not explain the elevation in both tests.

Q: Which of the following does not cause deep venous thrombosis (DVT)?

Subungual hematoma. ***Subungual hematoma*** - A **subungual hematoma** is a collection of blood under the fingernail or toenail, usually caused by trauma. - It is a localized injury that **does not affect systemic coagulation** or venous blood flow, thus not increasing DVT risk. *Lower limb trauma* - **Trauma to the lower limb**, especially involving fractures or significant soft tissue damage, can lead to **venous stasis** due to immobility and direct vessel injury [1]. - This immobility and vessel damage activate the **coagulation cascade**, significantly increasing the risk of DVT [1]. *Cushing's syndrome* - **Cushing's syndrome** is characterized by **hypercortisolism**, which leads to a **hypercoagulable state**. - **Elevated cortisol levels** increase circulating procoagulant factors and decrease fibrinolytic activity, predisposing patients to DVT. *Hip & pelvic surgeries* - **Major surgeries**, particularly those involving the **hip and pelvis**, frequently cause **endothelial injury**, blood stasis, and activate the coagulation system [1]. - Patients undergoing these procedures are at a **very high risk for DVT** due to prolonged immobility and surgical trauma [1].

Q: An obese patient undergoes a gastric bypass procedure to lose weight but never returns for follow-up or continuing care. Three years later, he presents to an emergency room with fatigue, a glossy tongue, and a macrocytic and hyperchromic anemia. Which one of the following is deficient or malfunctioning in this patient, leading to this anemia?

Intrinsic factor. ***Intrinsic factor*** - This patient's symptoms (fatigue, glossitis, macrocytic, and hyperchromic anemia) strongly suggest **vitamin B12 deficiency**, which often results from insufficient intrinsic factor. [1] - **Gastric bypass surgery** can lead to reduced gastric acid secretion and a decreased production of intrinsic factor, both of which are crucial for vitamin B12 absorption in the terminal ileum. [1] *Gastrin* - **Gastrin** primarily regulates gastric acid secretion and mucosal growth, but its deficiency is not a typical direct cause of macrocytic anemia. - While gastrin production can be altered in certain gastric conditions, it's not the primary factor in **vitamin B12 malabsorption** post-gastric bypass. *Iron* - An **iron deficiency** would typically present as **microcytic, hypochromic anemia**, not macrocytic and hyperchromic anemia. [2] - The symptoms described (glossy tongue, specific type of anemia) are inconsistent with isolated iron deficiency. [3] *Lead* - **Lead poisoning** can cause anemia (often microcytic or normocytic, and sometimes with **basophilic stippling**), but it does not lead to a **glossy tongue** or **macrocytic, hyperchromic anemia**. - The clinical presentation is not suggestive of lead toxicity.

Q: In the evaluation of a newly diagnosed case of acute lymphoid leukemia (ALL), which of the following tests is not routinely included?

Plasma viscosity. ### Plasma viscosity - **Plasma viscosity** measurement is not a standard diagnostic or staging test for **acute lymphoid leukemia (ALL)**. [1] - While it can be elevated in conditions with high protein levels or hypergammaglobulinemia, it does not provide specific information relevant to ALL diagnosis or treatment planning. [1] *Bone marrow biopsy* - A **bone marrow biopsy** is crucial for diagnosing ALL, confirming the presence of **lymphoblasts**, and assessing disease burden. [2] - It also helps in identifying cytogenetic and molecular abnormalities. [2] *Cell surface phenotyping* - **Cell surface phenotyping** (immunophenotyping) via **flow cytometry** is essential for classifying the subtype of ALL (e.g., B-ALL, T-ALL) and identifying specific markers. [2] - This information guides treatment protocols and predicts prognosis. [2] *Complete metabolic panel* - A **complete metabolic panel (CMP)** assesses organ function (e.g., kidney, liver), **electrolyte balance**, and levels of substances like **uric acid** and **lactate dehydrogenase (LDH)**. - These measurements are vital for identifying complications, assessing baseline health, and monitoring for **tumor lysis syndrome** which can be seen in ALL.

Q: Which is not seen in Polycythemia Vera?

Increased erythropoietin level. ***Increased erythropoietin level*** - In polycythemia vera, there is typically a **decrease in erythropoietin levels** due to the autonomous production of red blood cells. - The overproduction of red cells occurs independently of erythropoietin stimulation, distinguishing this condition from secondary causes of erythrocytosis. *Ocular congestion* - Ocular congestion can occur due to **increased blood volume** and vascular stasis associated with polycythemia vera. - It is often a result of elevated hematocrit levels leading to **erythromelalgia** and **hyperviscosity symptoms**. *Increase RBC count* - A hallmark of polycythemia vera is a **significant increase in red blood cell (RBC) count**, which is diagnostic for the condition. - This increase in RBCs contributes to symptoms such as **headaches, dizziness**, and ruddy complexion. *Increased Vit B12 binding capacity* - Polycythemia vera is associated with **increased vitamin B12 levels** due to elevated levels of transcobalamin, resulting in high binding capacity. - This phenomenon helps differentiate polycythemia vera from other forms of polycythemia. [1]

Question 1

All-trans-retinoic acid is used in the treatment of:

Accepted Answer

Acute promyelocytic leukemia (APL)

Answer

Acute lymphoblastic leukemia (ALL)

Answer

Chronic myeloid leukemia (CML)

Answer

Transient myeloproliferative disorder (TMD)

Question 2

An elderly patient is receiving a blood transfusion for anemia due to myelodysplastic syndrome (MDS). He was diagnosed with MDS two years ago and has required blood transfusions every six weeks for symptomatic anemia over the past six months. His past medical history also includes hypertension, type 2 diabetes, and coronary artery disease. Halfway through the transfusion of the second unit of packed red blood cells, he develops tachypnea, lumbar pain, tachycardia, and nausea. Which of the following is the most likely explanation?

Accepted Answer

hemolysis

Answer

fluid overload

Answer

pulmonary embolism

Answer

anxiety

Question 3

Haemochromatosis affects all of the following organs except:

Accepted Answer

Salivary glands

Answer

Liver

Answer

Pancreas

Answer

Heart

Question 4

A 60-year-old man presented to the emergency department with sudden-onset cough, yellow sputum production, and dyspnea, while taking amlodipine for hypertension. Chest X-ray revealed a left upper lobe alveolar infiltrate, and laboratory investigations showed Hb of 6 g/dL, BUN of 60 mg/dL, Creatinine of 2.8 mg/dL, Calcium of 12.3 mg/dL, total protein of 9 g/dL, and albumin of 4.2 g/dL. Echocardiogram revealed a dilated heart, and peripheral neuropathy was also present. What is the most common cause of death in patients with multiple myeloma?

Accepted Answer

Kidney failure

Answer

Bleeding

Answer

CHF

Answer

Infection

Question 5

Which of the following is false about monoclonal gammopathy of unknown significance (MGUS)?

Accepted Answer

Does not progress to multiple myeloma

Answer

No evidence of Bence-Jones proteinuria

Answer

Bone marrow plasma cells less than 10%

Answer

Less than 3g/dL of monoclonal protein

Question 6

A 9-year-old boy presents with elevated PT and APTT. What is the most likely underlying condition?

Accepted Answer

Liver disease

Answer

Vitamin K deficiency

Answer

Hemophilia A

Answer

Defect in common pathway

Question 7

Which of the following does not cause deep venous thrombosis (DVT)?

Accepted Answer

Subungual hematoma

Answer

Lower limb trauma

Answer

Hip & pelvic surgeries

Answer

Cushing's syndrome

Question 8

An obese patient undergoes a gastric bypass procedure to lose weight but never returns for follow-up or continuing care. Three years later, he presents to an emergency room with fatigue, a glossy tongue, and a macrocytic and hyperchromic anemia. Which one of the following is deficient or malfunctioning in this patient, leading to this anemia?

Accepted Answer

Intrinsic factor

Answer

Gastrin

Answer

Iron

Answer

Lead

Question 9

In the evaluation of a newly diagnosed case of acute lymphoid leukemia (ALL), which of the following tests is not routinely included?

Accepted Answer

Plasma viscosity

Answer

Bone marrow biopsy

Answer

Complete metabolic panel

Answer

Cell surface phenotyping

Question 10

Which is not seen in Polycythemia Vera?

Accepted Answer

Increased erythropoietin level

Answer

Increased RBC count

Answer

Ocular congestion

Answer

Increased Vitamin B12 binding capacity

Hematology — MCQs

Hematology — MCQs

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