Hematology Practice Questions

Q: Thrombotic event is seen in all of the following except:

Idiopathic thrombocytopenic purpura. ***Immune thrombocytopenic purpura*** - This condition primarily results in **thrombocytopenia**, leading to increased bleeding rather than thrombotic events [1][3]. - Patients typically present with **petechiae** and **purpura**, without the thrombotic complications seen in other conditions [1][3]. *Paroxysmal nocturnal hemoglobinuria* - Characterized by **hemolysis** and a risk of **thrombosis**, particularly in large veins. - Associated with **bone marrow** dysregulation, leading to increased blood viscosity and thrombotic events. *Heparin induced thrombocytopenia* - This condition leads to **thrombocytopenia** and paradoxically **increased thrombosis**, not bleeding. - It is caused by an immune response to heparin, resulting in the activation of platelets. *Disseminated intravascular coagulation* - Involves excessive clotting followed by a risk of bleeding due to **consumption of clotting factors** [1][2]. - Thrombotic events are common, with widespread small thrombi leading to organ dysfunction [2].

Q: Macrocytic anaemia is due to a deficiency of:

Vitamin B12. ***Vitamin B12*** - A deficiency in **Vitamin B12** (cobalamin) impairs DNA synthesis, leading to **megaloblastic erythropoiesis** where red blood cells are larger than normal [1]. - This results in **macrocytic anemia**, characterized by an elevated **mean corpuscular volume (MCV)** [1]. *Vitamin B1* - A deficiency in **Vitamin B1** (thiamine) primarily causes **Beriberi**, affecting the nervous and cardiovascular systems. - It is not directly associated with the development of **macrocytic anemia**. *Vitamin B2* - A deficiency in **Vitamin B2** (riboflavin) can cause **normocytic, normochromic anemia**, but not typically macrocytic anemia. - It also presents with symptoms like **cheilosis**, **glossitis**, and **seborrheic dermatitis**. *Vitamin B6* - A deficiency in **Vitamin B6** (pyridoxine) can lead to **sideroblastic anemia**, which is typically **microcytic** and **hypochromic**, not macrocytic [2]. - It impairs heme synthesis, causing iron accumulation in red blood cell precursors.

Q: Diagnosis of beta thalassemia is established by what?

Hb electrophoresis. Hb electrophoresis - Hemoglobin electrophoresis directly measures the relative proportions of different hemoglobin types (HbA, HbA2, HbF), which is crucial for identifying the characteristic reduction in HbA and elevation of HbA2 and HbF in beta thalassemia. [1] - This method provides a definitive diagnostic profile by separating hemoglobin based on their electrical charge and size, allowing for quantification of abnormal hemoglobin variants. [1] *NESTROFT screening test* - The NESTROFT (Naked Eye Single Tube Red cell Osmotic Fragility Test) is a screening tool used to identify individuals with thalassemia traits and is not a definitive diagnostic test. - While useful for mass screening due to its simplicity and cost-effectiveness, it requires confirmation with more specific tests like hemoglobin electrophoresis. [1] *Hemoglobin A1c test* - The Hemoglobin A1c (HbA1c) test is primarily used to monitor long-term blood glucose control in individuals with diabetes. [2] - It measures the percentage of hemoglobin glycated over a period of 2-3 months and has no direct diagnostic utility for thalassemia. [2] *Presence of target cells in blood smear* - The presence of target cells in a blood smear is a non-specific finding that can be observed in various conditions, including iron deficiency anemia, liver disease, and other hemoglobinopathies, in addition to thalassemia. - While suggestive of a thalassemic disorder, it is not a conclusive diagnostic criterion and requires further investigation with specific diagnostic tests.

Q: Which of the following is not a typical finding in sickle cell anemia?

Splenomegaly usually seen. ***Splenomegaly usually seen*** - In sickle cell anemia, the spleen often becomes **infarcted** due to sickled cells, leading to functional **asplenia** rather than splenomegaly [1]. - **Spleen dysfunction** increases susceptibility to infections, notably from encapsulated organisms [1]. *Enlarged heart* - While **cardiac enlargement** can occur in chronic anemia, it is not a direct and characteristic finding specifically associated with sickle cell anemia. - Other heart findings might include **hypertrophy** due to increased cardiac workload rather than generalized enlargement. *Leukocytosis* - Patients with sickle cell anemia commonly have **leukopenia** due to splenic dysfunction and the sequestration of white blood cells. - Although some acute episodes may cause transient leukocytosis, it is not typical for the disease as a chronic finding. *Fish vertebra* - The term **fish vertebra** refers to the appearance of the spine in conditions like sickle cell anemia due to **vertebral body changes** from infarction. - This feature is associated with the disease [1], contrasting with the correct answer regarding splenomegaly.

Q: Coomb's positive hemolytic anemia is associated with:

Systemic Lupus Erythematosus (SLE). ***Systemic Lupus Erythematosus (SLE)*** - **Autoimmune hemolytic anemia (AIHA)**, including cases with a positive Coombs test, is a common hematologic manifestation of **SLE**, occurring in up to 10% of patients [1]. - In SLE-associated AIHA, autoantibodies—often immunoglobulin G (IgG)—attack red blood cell surfaces, leading to their premature destruction and a **positive Coombs test** [1]. *Polyarteritis Nodosa (PAN)* - PAN is a **necrotizing vasculitis** of small and medium-sized arteries, primarily affecting the kidneys, gut, and nerves. - While it can cause anemia of chronic disease or related to gastrointestinal bleeding, it is not typically associated with **Coombs-positive hemolytic anemia**. *Drug-induced hemolytic anemia* - This condition is caused by **certain medications** (e.g., penicillin, methyldopa) that trigger antibody production against red blood cells or modify their surface to be recognized as foreign. - While it results in a positive Coombs test, it is a **drug-specific reaction** and not inherently associated with a systemic autoimmune disease like those listed. *Thrombotic Thrombocytopenic Purpura (TTP)* - TTP is characterized by a **microangiopathic hemolytic anemia** and **thrombocytopenia**, caused by a deficiency of the ADAMTS13 enzyme. - The hemolysis in TTP is mechanical due to red blood cell fragmentation in tiny clots, resulting in a **negative Coombs test**.

Q: In the context of Fanconi's anemia, which investigation is least relevant?

Echocardiography. ***Echocardiography*** - While Fanconi's anemia can rarely be associated with **cardiac anomalies**, these are not primary or consistent features, making routine echocardiography **less relevant** for diagnosis or monitoring compared to other mentioned investigations. - The core pathology involves **bone marrow failure** and genomic instability, which are better assessed by hematological and genetic tests. *Bone marrow examination* - This is a crucial investigation for Fanconi's anemia, as it directly assesses the bone marrow for **hypoplasia**, **dysplasia**, and changes consistent with impending **myelodysplastic syndrome (MDS)** or acute myeloid leukemia (AML) [1]. - It helps confirm the presence and severity of the **pancytopenia** and bone marrow failure [1]. *Platelet count* - **Thrombocytopenia** (low platelet count) is a common manifestation of Fanconi's anemia, reflecting the progressive **bone marrow failure**. - Monitoring platelet counts is essential for assessing disease progression and guiding **transfusion support** to manage bleeding risk. *Karyotyping* - **Karyotyping** (especially chromosomal breakage studies with mitomycin C or diepoxybutane) is the **gold standard diagnostic test** for Fanconi's anemia. - It identifies the characteristic **chromosomal instability** and breaks, confirming the underlying genetic defect.

Q: A patient develops pulmonary thromboembolism most commonly due to what?

Deep Vein Thrombosis (DVT). ***Deep Vein Thrombosis (DVT)*** - **Deep vein thrombosis (DVT)** is the most common cause of pulmonary thromboembolism, as clots from deep veins, typically in the legs, travel to the lungs [1]. - The initial clot formation in DVT is often multifactorial, involving elements of **Virchow's triad** (venous stasis, endothelial injury, and hypercoagulability) [1]. *Chronic Venous Hypertension* - **Chronic venous hypertension** results from sustained high pressure in leg veins, leading to symptoms like edema, skin changes, and ulcers, but does not directly cause emboli. - It's a consequence of venous insufficiency and doesn't involve the formation of typical occlusive thrombi that can embolize to the pulmonary arteries. *Disseminated intravascular coagulation (DIC)* - **Disseminated intravascular coagulation (DIC)** is a severe, systemic condition characterized by widespread activation of coagulation, leading to microthrombi formation and consumption of clotting factors, often resulting in bleeding. - While small thrombi can form, the primary manifestation is diffuse bleeding, and the thrombi are usually diffuse microvascular clots rather than large, embolizing thrombi to the pulmonary arteries. *Inherited Thrombophilia* - **Inherited thrombophilias** are genetic predispositions to excessive clotting and are risk factors for DVT, but they are not the direct cause of pulmonary embolism. - They increase the likelihood of developing DVT, which then *in turn* can lead to pulmonary embolism [1].

Q: A 60-year-old female with a history of 8 blood transfusions in 2 years, presenting with a hemoglobin level of 60 g/L, total leukocyte count of 5800, platelet count of 3.4 lakhs, mean corpuscular volume of 60, and red blood cell count of 2.11 lakhs/mm3, is diagnosed with hypochromic microcytic anemia. Which investigation is least likely to provide useful information?

Evaluation for pulmonary hemosiderosis. ***Evaluation for pulmonary hemosiderosis*** - This condition involves **iron deposition in the lungs** and is not part of the standard workup for **iron deficiency anemia**. - **Pulmonary hemosiderosis** is a rare condition seen in diseases like Goodpasture syndrome, making it the least relevant investigation here. *Urinary hemosiderin* - Can detect **intravascular hemolysis**, which may be relevant given the patient's transfusion history and ongoing iron deficiency [3]. - Helps identify if **hemolysis** is contributing to iron loss despite multiple transfusions. *Bone marrow examination* - Provides definitive assessment of **iron stores** and can confirm iron deficiency when other tests are inconclusive [3]. - Useful in complex cases where **iron deficiency** persists despite transfusions, indicating massive ongoing blood loss. *GI endoscopy* - Essential for identifying **gastrointestinal bleeding** [2], which could explain iron deficiency despite multiple transfusions. - Helps detect **ulcers**, **tumors**, or other lesions causing chronic blood loss that exceeds iron replacement from transfusions [1], [2].

Q: Haemoglobin F is raised in which condition?

Juvenile chronic myeloid leukemia. ***Juvenile chronic myeloid leukemia*** - This condition is characterized by a high proportion of **fetal hemoglobin (HbF)**, often exceeding 50%, alongside other typical myeloproliferative features. - The elevated HbF is a distinguishing feature of **juvenile CML** from adult CML, which typically presents with normal or only slightly elevated HbF levels. *Beta-thalassemia major* - While patients with **beta-thalassemia major** can have elevated HbF, it is typically in response to a severe deficiency in beta-globin chain production, leading to compensatory gamma-chain synthesis. - However, the primary genetic defect lies in the beta-globin genes, and the HbF increase is usually not as universally high or definitive as in HPFH or juvenile CML. *Sickle cell disease* - Patients with **sickle cell disease** can have variable levels of HbF, and higher levels are associated with a milder disease course [1]. - HbF acts as a protective factor by inhibiting hemoglobin S polymerization, but the presence of high HbF is not a diagnostic marker in the same way it is for HPFH or juvenile CML [1]. *Hereditary persistence of fetal hemoglobin (HPFH)* - This is a benign condition characterized by the **continued production of high levels of HbF into adulthood** due to genetic mutations that prevent the normal developmental switch from gamma-globin to beta-globin synthesis. - While it features significantly raised HbF, HPFH is typically **asymptomatic** and does not present with the myeloproliferative features seen in juvenile CML.

Q: A patient presents with symptoms including gum hyperplasia and hepatosplenomegaly. Which subtype of acute myeloid leukemia (AML) is most likely?

Acute Myeloid Leukemia (M4). ***M4*** - Acute myeloid leukemia (AML) M4, known as **myelomonocytic leukemia**, often presents with **gum infiltration** and **hepatosplenomegaly**, indicating an aggressive disease course [1]. - The presence of monocytes leads to **extramedullary hematopoiesis**, accounting for the enlargement of organs and gum involvement. *ALL* - Acute lymphoblastic leukemia (ALL) primarily affects lymphoid tissues and typically presents with **lymphadenopathy** and **bone pain**, rather than gum infiltration. - **Hepatosplenomegaly** can occur but is not a primary feature as seen in M4. *M3* - Acute promyelocytic leukemia (M3) is characterized by the presence of **promyelocytes** and is associated with a distinct clinical spectrum, primarily related to **coagulopathy** and not gum infiltration. - While it may present with **hematological abnormalities**, gum infiltration is not a common finding. *M2* - AML M2 is classified as **acute myeloblastic leukemia** and has less frequent **gum infiltration** compared to M4 [1]. - It can also cause **hepatosplenomegaly**, but this is more common in other variants like M4 with its characteristic features.

Question 1

Thrombotic event is seen in all of the following except:

Accepted Answer

Idiopathic thrombocytopenic purpura

Answer

Heparin induced thrombocytopenia

Answer

Disseminated intravascular coagulation

Answer

Paroxysmal nocturnal hemoglobinuria

Question 2

Macrocytic anaemia is due to a deficiency of:

Accepted Answer

Vitamin B12

Answer

Vitamin B1

Answer

Vitamin B2

Answer

Vitamin B6

Question 3

Diagnosis of beta thalassemia is established by what?

Accepted Answer

Hb electrophoresis

Answer

NESTROFT screening test

Answer

Hemoglobin A1c test

Answer

Presence of target cells in blood smear

Question 4

Which of the following is not a typical finding in sickle cell anemia?

Accepted Answer

Splenomegaly usually seen

Answer

Enlarged heart

Answer

Leukocytosis

Answer

Fish vertebra

Question 5

Coomb's positive hemolytic anemia is associated with:

Accepted Answer

Systemic Lupus Erythematosus (SLE)

Answer

Drug-induced hemolytic anemia

Answer

Polyarteritis Nodosa (PAN)

Answer

Thrombotic Thrombocytopenic Purpura (TTP)

Question 6

In the context of Fanconi's anemia, which investigation is least relevant?

Accepted Answer

Echocardiography

Answer

Bone marrow examination

Answer

Platelet count

Answer

Karyotyping

Question 7

A patient develops pulmonary thromboembolism most commonly due to what?

Accepted Answer

Deep Vein Thrombosis (DVT)

Answer

Chronic Venous Hypertension

Answer

Disseminated intravascular coagulation (DIC)

Answer

Inherited Thrombophilia

Question 8

A 60-year-old female with a history of 8 blood transfusions in 2 years, presenting with a hemoglobin level of 60 g/L, total leukocyte count of 5800, platelet count of 3.4 lakhs, mean corpuscular volume of 60, and red blood cell count of 2.11 lakhs/mm3, is diagnosed with hypochromic microcytic anemia. Which investigation is least likely to provide useful information?

Accepted Answer

Evaluation for pulmonary hemosiderosis

Answer

Bone marrow examination

Answer

GI endoscopy

Answer

Urinary hemosiderin

Question 9

Haemoglobin F is raised in which condition?

Accepted Answer

Juvenile chronic myeloid leukemia

Answer

Hereditary persistence of fetal hemoglobin (HPFH)

Answer

Beta-thalassemia major

Answer

Sickle cell disease

Question 10

A patient presents with symptoms including gum hyperplasia and hepatosplenomegaly. Which subtype of acute myeloid leukemia (AML) is most likely?

Accepted Answer

Acute Myeloid Leukemia (M4)

Answer

Acute Promyelocytic Leukemia (M3)

Answer

Acute Myeloid Leukemia (M2)

Answer

Acute Myeloid Leukemia (M1)

Hematology — MCQs

Hematology — MCQs

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