Hematology Practice Questions

Q: Bone marrow biopsy is absolutely indicated in which of the hematological conditions?

Hairy cell leukemia. ***Hairy cell leukemia*** - A **bone marrow biopsy** is absolutely indicated for diagnosis due to the characteristic **fibrosis** that often prevents a diagnostic aspirate. - The biopsy can reveal infiltration by "hairy cells" and assess the extent of disease and **myelofibrosis**. *Acute leukemia* - While bone marrow examination (aspirate and biopsy) is crucial for diagnosing **acute leukemia**, an aspirate is often sufficient for initial diagnosis and classification. - Biopsy provides additional information on cellularity and architecture, but the diagnosis can often be made on the aspirate. *Megaloblastic anemia* - Diagnosis primarily relies on **peripheral blood smears** showing macro-ovalocytes and hypersegmented neutrophils, along with **serum vitamin B12** and **folate levels**. - A bone marrow aspirate can show **megaloblastic erythroid and myeloid hyperplasia**, but it is generally not an absolute indication for diagnosis. *Thalassemia* - Diagnosis of **thalassemia** is primarily based on **hemoglobin electrophoresis**, genetic testing, and peripheral blood indices (microcytic, hypochromic anemia). - Bone marrow examination is usually not required for diagnosis, though it may show erythroid hyperplasia.

Q: Which of the following is NOT a treatment option for acute lymphoblastic leukemia?

Rituximab. ***Rituximab*** - **Rituximab** is a monoclonal antibody targeting the **CD20 antigen** found on B-lymphocytes, commonly used in **B-cell non-Hodgkin lymphoma** and **chronic lymphocytic leukemia** [1]. - **Acute lymphoblastic leukemia (ALL)** typically involves T-cell or pre-B-cell lineages and generally **does not express CD20**, making rituximab ineffective as a primary treatment [1].*Vincristine* - **Vincristine** is a **vinca alkaloid** that inhibits microtubule formation, crucial for cell division. - It is a cornerstone of **ALL induction and maintenance chemotherapy regimens**.*L-asparaginase* - **L-asparaginase** depletes asparagine, an essential amino acid for leukemic cells that cannot synthesize it themselves. - It is a vital component of **ALL treatment protocols**, particularly in pediatric ALL.*Prednisolone* - **Prednisolone** is a corticosteroid that induces apoptosis in lymphoid cells. - It is an important agent in the **induction phase of ALL treatment** and also used for supportive care to manage symptoms.

Q: A middle-aged male presented with a history of fatigue and tiredness. On investigation, he had a hemoglobin level of 8 g/dL and a mean corpuscular volume (MCV) of 110 fl, with the peripheral smear showing macrocytes and hypersegmented neutrophils. Which of the following is the most likely cause of these findings in this patient?

Chronic alcohol use. ***Chronic alcoholism*** - **Macrocytic anemia** with a **hemoglobin level of 8 g/dL** is commonly seen in chronic alcoholism due to impaired erythropoiesis and folate deficiency. [1] - The **peripheral smear** showing **hypersegmented neutrophils** is indicative of megaloblastic anemia, often linked to **alcoholism**. [1] *Chronic renal failure* - Typically causes **normocytic anemia** due to inadequate erythropoietin production, not macrocytic anemia. [2] - While uremic effects can impact erythropoiesis, they usually do not produce **hypersegmented neutrophils** found in megaloblastic anemia. *Colon cancer* - More commonly causes **microcytic anemia** due to **iron deficiency** from chronic blood loss rather than macrocytic anemia. [2] - **Macrocytic anemia** with hypersegmented neutrophils would not be expected in most cases of colon cancer. *Ancylostoma duodenale infestation* - This hookworm infection typically leads to **microcytic anemia** due to **iron deficiency**, not macrocytic. [2] - The presence of **macrocytes** and **hypersegmented neutrophils** does not correlate with this type of anemia.

Q: Which of the following conditions can present with mildly elevated bilirubin and normal liver enzymes?

G-6 PD deficiency. ***G-6 PD deficiency*** - **G-6 PD deficiency** can cause **hemolysis** [2], leading to an increase in unconjugated bilirubin, but it does not directly affect liver enzyme production or hepatocyte function. [3] - The liver's excretory capacity is intact, hence liver enzymes remain normal while bilirubin is mildly elevated due to increased pigment load from **red blood cell destruction**. [1] *Malaria* - While malaria can cause **hemolysis** and elevated bilirubin [3], severe cases can also lead to **hepatic dysfunction** and abnormal liver enzymes due to parasitic infection and inflammation. - Complicated malaria, particularly **cerebral malaria**, is associated with features like **hepatic steatosis** and changes to liver cells, which could impact enzyme levels. *Thalassemia* - **Thalassemia** is a chronic hemolytic anemia, causing elevated unconjugated bilirubin. However, long-term iron overload from frequent transfusions (in severe cases) can lead to **hemochromatosis** and eventual **liver damage**, affecting liver enzymes. - In thalassemia patients, chronic hemolysis itself might also indirectly contribute to some liver stress over time, though acute elevations in liver enzymes are not typical unless complications arise. *All of the options* - This option is incorrect because while all three conditions can cause **elevated bilirubin** due to hemolysis, malaria and severe thalassemia can also involve **liver enzyme abnormalities** secondary to disease complications or chronic effects, unlike the typical presentation of G-6 PD deficiency which isolates the bilirubin elevation to hemolysis.

Q: Most significant complication of polycythemia rubra vera is:

Thrombosis. ***Thrombosis*** - **Thrombosis** is the most significant and frequent complication, leading to **morbidity and mortality** in patients with polycythemia vera. - The elevated red blood cell mass and increased blood viscosity significantly raise the risk of both **arterial and venous clots**. *Transformation into leukemia* - While polycythemia vera can transform into **acute myeloid leukemia**, this is a less common complication compared to thrombosis. - Leukemic transformation is often associated with prior exposure to **myelosuppressive therapies**. *Secondary bone marrow failure* - **Bone marrow failure** can occur in the late stages of myeloproliferative neoplasms, but it is less common than thrombotic events during the active phase of the disease. [1] - This typically manifests as **myelofibrosis and pancytopenia**. [1] *Hypersplenism* - **Splenomegaly** is common in polycythemia vera, and **hypersplenism** can occur causing cytopenias. - However, while hypersplenism can cause complications like anemia or thrombocytopenia, it is generally less life-threatening than acute thrombotic events.

Q: Bone marrow transplantation as a treatment modality can be advised in all of the following cases of newly diagnosed conditions, except:

CML. ***CML*** - For **newly diagnosed chronic myeloid leukemia (CML)**, the primary treatment is typically **tyrosine kinase inhibitors (TKIs)**, such as imatinib, which have significantly improved prognosis. - **Bone marrow transplantation (BMT)** is generally reserved for CML patients who are **resistant to TKI therapy** or are in an accelerated or blast phase, making it less likely to be advised as a first-line treatment for a *newly diagnosed* case. *Combined immunodeficiency* - **Severe combined immunodeficiency (SCID)** is a life-threatening condition where the immune system is severely compromised, and **bone marrow transplantation (BMT)** is a curative treatment option as it replaces the defective hematopoietic stem cells [2]. - BMT can restore immune function by providing healthy donor cells that differentiate into various immune cell types, making it a viable and often necessary intervention for newly diagnosed cases [2]. *Aplastic anemia* - For cases of **severe aplastic anemia**, especially in younger patients with an **HLA-matched sibling donor**, **allogeneic hematopoietic stem cell transplantation (HSCT)** is a highly effective and often curative treatment option [1]. - HSCT aims to replace the damaged bone marrow with healthy donor stem cells to restore normal hematopoiesis. *All of the options* - As **CML** is typically managed first with tyrosine kinase inhibitors (TKIs) and bone marrow transplantation is reserved for refractory or advanced cases, it is not appropriate to select "All of the options" as the answer. - The other conditions listed (combined immunodeficiency and aplastic anemia) are indeed strong indications for bone marrow transplantation in newly diagnosed cases [2].

Q: Which antibody is commonly elevated in Waldenström macroglobulinemia?

IgM. ***IgM*** - Waldenström macroglobulinemia is characterized by elevated levels of **IgM**, which is produced by malignant plasma cells [1]. - This condition is associated with **hyperviscosity syndrome** due to the high concentration of IgM in the serum [1]. *IgD* - Typically, **IgD** levels are not significantly altered in Waldenström macroglobulinemia and do not correlate with the disease. - It plays a minor role in the immune response and is not a marker for this condition. *IgG* - Although IgG can be elevated in various disorders, it is not the primary antibody of concern in Waldenström macroglobulinemia. - Patients exhibit increased **IgM** levels rather than IgG involvement, making this option incorrect. *IgA* - Elevated **IgA** levels are more commonly associated with different conditions like chronic infections or autoimmune diseases, not specifically with Waldenström macroglobulinemia. - The hallmark of this disease is the high level of **IgM**, differentiating it from other immunoglobulin disorders [2].

Q: Before the advent of tyrosine kinase inhibitors, the most effective treatment of chronic myeloid leukemia was:

Allogeneic bone marrow transplant. ***Allogeneic bone marrow transplant*** - Before the advent of TKIs, **allogeneic hematopoietic stem cell transplantation (HSCT)** was the only curative treatment for CML [1]. - It involved replacing the patient's diseased bone marrow with healthy stem cells from a genetically matched donor, thereby eradicating the **Philadelphia chromosome-positive clone** [1]. *Haploidentical bone marrow transplant* - While a form of HSCT, **haploidentical transplants** were typically used as a backup option when a fully matched donor was unavailable due to higher risks of **graft-versus-host disease (GVHD)** and rejection. - It was not considered the most effective or preferred treatment before TKIs, being reserved for specific challenging cases. *Chemotherapy* - **Conventional chemotherapy** for CML, such as busulfan or hydroxyurea, primarily aimed at reducing the white blood cell count and controlling symptoms. - It was **palliative** and did not offer a cure or significantly prolong survival in the long term, unlike allogeneic HSCT [1]. *Hydroxyurea and interferon* - **Hydroxyurea** is a cytoreductive agent, and **interferon-alpha** was used to induce hematologic and cytogenetic responses in CML patients. - Although they provided some benefit in controlling the disease and improving survival compared to no treatment, they rarely achieved a cure and were associated with significant side effects, making them less effective than allogeneic HSCT [1].

Q: The most classic dietary trigger for acute hemolytic anemia in G6PD deficiency is which of the following?

Fava beans. **Fava beans** - **Fava beans** contain high concentrations of **vicine and convicine**, which are converted into **divicine and isouramil**. These compounds produce **reactive oxygen species** that overwhelm the erythrocyte's antioxidant defense mechanisms, especially in individuals with **G6PD deficiency** [2]. - In individuals with **G6PD deficiency**, the inability to produce sufficient **NADPH** results in inadequate levels of **reduced glutathione**, making red blood cells vulnerable to **oxidative stress** induced by these fava bean metabolites, leading to **hemolysis** [1, 3]. *Viral upper respiratory infections only* - While **infections (bacterial or viral)** can trigger **hemolytic crises** in G6PD deficient individuals by causing **oxidative stress** and inflammation, they are not a dietary trigger [3, 4]. - The question specifically asks for a **dietary trigger**, which excludes infections as the primary cause. *Vitamin C supplements* - While **large doses of vitamin C (ascorbic acid)** can act as a **pro-oxidant** and potentially induce some oxidative stress, it is generally not considered a classic or strong trigger for acute hemolytic anemia in G6PD deficiency at typical supplement doses. - **Fava beans** are far more potent and well-documented as a dietary trigger for this condition [2]. *Folic acid deficiency* - **Folic acid deficiency** does not directly cause acute hemolytic anemia in G6PD deficiency; it typically leads to **megaloblastic anemia** due to impaired DNA synthesis [4]. - While folic acid supplementation is often given to G6PD deficient patients with chronic hemolysis to support increased red blood cell production, its deficiency does not induce oxidative hemolysis.

Q: Type I cryoglobulinemia is associated with all of the following, except:

Strongly Positive Rheumatoid factor. Type I cryoglobulinemia is associated with all of the following, except: ***Strongly Positive Rheumatoid factor*** - **Type I cryoglobulinemia** involves a **monoclonal immunoglobulin** (usually IgG or IgM) that self-aggregates in the cold. It does not typically involve the autoantibody activity of rheumatoid factor. - While other types of cryoglobulinemia (Type II and III) can be associated with rheumatoid factor activity, it is not a characteristic feature of Type I. *IgM monoclonal paraprotein* - **Type I cryoglobulinemia** involves a single **monoclonal immunoglobulin**, which can be an IgM paraprotein [1]. - This monoclonal protein precipitates at cold temperatures, contributing to the clinical manifestations. *Hyperviscosity* - The presence of large amounts of **monoclonal immunoglobulins** in **Type I cryoglobulinemia** can significantly increase blood viscosity [1]. - This can lead to symptoms such as headaches, visual disturbances, and neurological deficits [1]. *Normal complement levels* - **Type I cryoglobulinemia** typically involves the precipitation of a monoclonal immunoglobulin without significant immune complex formation or complement activation. - Therefore, **complement levels** (C3, C4) usually remain normal, unlike in Type II and III cryoglobulinemias where complement is often consumed.

Question 1

Bone marrow biopsy is absolutely indicated in which of the hematological conditions?

Accepted Answer

Hairy cell leukemia

Answer

Acute leukemia

Answer

Megaloblastic anemia

Answer

Thalassemia

Question 2

Which of the following is NOT a treatment option for acute lymphoblastic leukemia?

Accepted Answer

Rituximab

Answer

L-asparaginase

Answer

Vincristine

Answer

Prednisolone

Question 3

A middle-aged male presented with a history of fatigue and tiredness. On investigation, he had a hemoglobin level of 8 g/dL and a mean corpuscular volume (MCV) of 110 fl, with the peripheral smear showing macrocytes and hypersegmented neutrophils. Which of the following is the most likely cause of these findings in this patient?

Accepted Answer

Chronic alcohol use

Answer

Ancylostoma duodenale infection

Answer

Chronic kidney disease

Answer

Colorectal cancer

Question 4

Which of the following conditions can present with mildly elevated bilirubin and normal liver enzymes?

Accepted Answer

G-6 PD deficiency

Answer

All of the options

Answer

Malaria

Answer

Thalassemia

Question 5

Most significant complication of polycythemia rubra vera is:

Accepted Answer

Thrombosis

Answer

Transformation into leukemia

Answer

Secondary bone marrow failure

Answer

Hypersplenism

Question 6

Bone marrow transplantation as a treatment modality can be advised in all of the following cases of newly diagnosed conditions, except:

Accepted Answer

CML

Answer

Aplastic anemia

Answer

Combined immunodeficiency

Answer

All of the options

Question 7

Which antibody is commonly elevated in Waldenström macroglobulinemia?

Accepted Answer

IgM

Answer

IgG

Answer

IgA

Answer

IgD

Question 8

Before the advent of tyrosine kinase inhibitors, the most effective treatment of chronic myeloid leukemia was:

Accepted Answer

Allogeneic bone marrow transplant

Answer

Chemotherapy

Answer

Hydroxyurea and interferon

Answer

Haploidentical bone marrow transplant

Question 9

The most classic dietary trigger for acute hemolytic anemia in G6PD deficiency is which of the following?

Accepted Answer

Fava beans

Answer

Viral upper respiratory infections only

Answer

Vitamin C supplements

Answer

Folic acid deficiency

Question 10

Type I cryoglobulinemia is associated with all of the following, except:

Accepted Answer

Strongly Positive Rheumatoid factor

Answer

IgM monoclonal paraprotein

Answer

Hyperviscosity

Answer

Normal complement levels

Hematology — MCQs

Hematology — MCQs

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