Hematology Practice Questions

Q: A 32-year-old woman presents with fever, fatigue, jaundice, and anemia. Laboratory results show hemoglobin of 7.5, a high reticulocyte count, and elevated indirect bilirubin. The peripheral smear reveals spherocytes. What is the definitive treatment?

Corticosteroids. Corticosteroids - The presented symptoms (fever, fatigue, jaundice, anemia with high reticulocytes, elevated indirect bilirubin, and spherocytes) are highly suggestive of warm autoimmune hemolytic anemia (AIHA) [1]. - Corticosteroids are the first-line and often definitive treatment, suppressing the immune system and reducing antibody production against red blood cells. Splenectomy - Splenectomy is considered a second-line treatment option if corticosteroids fail or if the patient requires unacceptably high doses for maintenance. - It removes the primary site of red blood cell destruction but is not the initial definitive treatment. Blood transfusion - Blood transfusion is a supportive measure used to manage severe anemia and stabilize the patient, but it does not address the underlying autoimmune process. - While helping with symptoms, it is not a definitive treatment for the autoimmune destruction of red blood cells. Immunosuppressive therapy - While corticosteroids are a type of immunosuppressive therapy, other forms of immunosuppressive therapy (e.g., rituximab, azathioprine, cyclophosphamide) are typically reserved for cases refractory to corticosteroids and splenectomy [2]. - They are not considered the initial definitive treatment for warm AIHA.

Q: In which condition are multiple lytic lesions typically seen?

Multiple myeloma. ***Multiple myeloma*** - Characterized by **solitary lytic bone lesions** [1], often seen on X-rays, indicating areas of bone destruction [1]. - It is associated with **monoclonal proliferation** of plasma cells [1], leading to both osteolytic lesions and hypercalcemia. *Osteoblast* - Involvement of osteoblasts typically leads to **bone formation**, not lytic lesions. - Conditions like **osteosarcoma** may show lytic lesions, but not from osteoblast activity directly. *Mitral stenosis* - Primarily a **valvular heart disease**, leading to **blood flow obstruction**, not associated with lytic bone lesions. - Symptoms are predominantly related to **cardiac function** rather than bone pathology. *Atherosclerosis* - A vascular condition characterized by **plaque formation** within arteries, affecting blood flow but not leading to bone lesions. - It manifests as cardiovascular diseases, not as **osseous lesions**.

Q: Choose the best method of diagnosis for the clinical sign represented in the image.

Serum ceruloplasmin. ***Serum ceruloplasmin*** - The image shows a **Kayser-Fleischer ring**, a greenish-brown discoloration in the periphery of the cornea, which is pathognomonic for **Wilson's disease**. - **Wilson's disease** is a genetic disorder of copper metabolism characterized by **low serum ceruloplasmin** levels (the primary copper-carrying protein in the blood) and increased copper deposition in various tissues. *Serum copper* - While Wilson's disease involves copper accumulation, **total serum copper** can be normal or even elevated due to widespread tissue damage releasing copper into the circulation, making it an unreliable diagnostic marker on its own. - A low serum copper level can be seen, but it is not as specific as low ceruloplasmin, as much of the copper in serum is bound to ceruloplasmin. *Karyotyping* - **Karyotyping** is used to analyze the number and structure of chromosomes and is primarily indicated for diagnosing chromosomal abnormalities, such as Down syndrome or Turner syndrome. - It is not relevant for diagnosing metabolic disorders like Wilson's disease, which is caused by a mutation in a single gene (ATP7B), not a chromosomal aberration. *PCR* - **PCR (Polymerase Chain Reaction)** is a technique used to amplify DNA sequences and can be used for genetic testing to identify specific mutations. - While genetic testing for the **ATP7B gene** mutation is a confirmatory test for Wilson's disease, it is not the primary or best method for initial diagnosis, especially when classic clinical signs and biochemical markers (like low ceruloplasmin) are present.

Q: What is the first-line treatment for idiopathic thrombocytopenia purpura (ITP)?

Corticosteroids. ***Corticosteroids*** - **Corticosteroids**, such as prednisone or dexamethasone, are the **first-line treatment** for ITP due to their ability to quickly reduce antibody production and improve platelet count by immunosuppression. - They work by **decreasing platelet destruction** in the spleen and improving the integrity of blood vessels. *Blood transfusion* - **Blood transfusions** are not a primary treatment for ITP as they do not address the underlying autoimmune destruction of platelets and donated platelets are rapidly destroyed. - They are reserved for **life-threatening hemorrhage** as a temporary measure to increase platelet count in severe bleeding episodes. *Intravenous immunoglobulins (IVIG)* - **IVIG** is typically used in ITP patients who have **severe bleeding**, are unresponsive to corticosteroids, or require a rapid increase in platelet count for emergent situations. - It works by **blocking Fc receptors** on macrophages, thereby reducing platelet destruction, but is not the initial first-line therapy. *Splenectomy* - **Splenectomy** is considered a **second-line treatment** for ITP, reserved for patients who are refractory to medical therapy or require long-term remission [1]. - It removes the primary site of platelet destruction and antibody production but carries **surgical risks** and is not the initial approach [1].

Q: Which of the following is typically not associated with secondary Idiopathic thrombocytopenic purpura?

Rheumatoid arthritis. ***Rheumatoid arthritis*** - While rheumatoid arthritis is an **autoimmune disease**, its association with secondary ITP is statistically **less common** compared to other systemic autoimmune conditions or infections. - ITP is primarily associated with conditions that directly lead to the production of **anti-platelet antibodies** or impair platelet production/survival more significantly. *Systemic lupus erythematosus* - **SLE** is a well-established cause of **secondary ITP** due to its widespread autoimmune activity, including the production of autoantibodies against platelets [1]. - Thrombocytopenia is a common hematologic manifestation of lupus. *Hepatitis C infection* - **Hepatitis C virus (HCV) infection** is strongly associated with secondary ITP, often through immune complex formation and direct effects on platelet survival and production. - HCV can induce the production of **anti-platelet antibodies**. *HIV infection* - **HIV infection** is a frequent cause of secondary ITP, with thrombocytopenia often being one of the earliest hematologic abnormalities. - Mechanisms include **direct viral effects on megakaryocytes**, immune-mediated destruction of platelets, and increased platelet consumption.

Q: What is the earliest hematological change following splenectomy?

Leukocytosis and thrombocytosis. Detailed explanation of hematological changes after splenectomy: ***Leukocytosis and thrombocytosis*** - Following splenectomy, there is an increase in **white blood cells (leukocytosis)** and **platelets (thrombocytosis)**, which is the earliest hematological change observed. - This is due to the loss of splenic function, which normally clears excess blood cells from circulation. *Poikilocytosis* - Poikilocytosis refers to the presence of abnormally shaped red blood cells, which typically indicates an underlying **hemolytic anemia** or **nutritional deficiency** [1]. - This change is **not specific** to splenectomy and may take longer to occur post-surgery. *Presence of Heinz bodies* - Heinz bodies indicate the presence of **denatured hemoglobin** often found in conditions like **G6PD deficiency** or **oxidative stress**. - They are **not a direct consequence** of splenectomy and do not appear as an early hematological change. *Evidence of Howell Jolly bodies* - Howell Jolly bodies are remnants of nuclear material in red blood cells, typically seen after splenectomy due to the spleen's role in **removing such bodies**. - However, their appearance is **later on** compared to leukocytosis and thrombocytosis following surgery.

Q: Which of the following statements is false regarding beta thalassemia major?

Bone marrow iron is depleted. ***Bone marrow iron is depleted*** - In beta thalassemia major, patients typically experience **iron overload** due to repeated blood transfusions, leading to **depleted iron stores** in the bone marrow. - This finding contrasts with conditions where iron stores are preserved or increased, highlighting the pathology of thalassemia. *Growth and development is impaired* - While children with beta thalassemia major may experience growth delay, this statement is **not universally true** for all patients. - Factors such as treatment and iron overload management can influence growth and development outcomes. *Red cell count <4 x 10'2/L* - Normal red cell counts can occur in some patients, particularly in **transfusion-dependent cases**. - Thus, it is not a definitive characteristic of beta thalassemia major, as levels can vary. *Levels of HbA2 < 3.5%* - In beta thalassemia major, HbA2 levels are often **increased**, usually exceeding 3.5% [1]. - This distinguishes it from beta thalassemia trait, where HbA2 is elevated but not to the same extent as in major [1].

Q: Treatment of chronic phase of CML in pregnant women is -

Interferon therapy. ***Interferon therapy*** - **Interferon-alpha** is the preferred treatment for chronic phase **CML** in pregnant women because it is not associated with teratogenic effects. - It works by modulating the **immune system** and inhibiting cell proliferation [1]. *Imatinib* - **Imatinib** is a **tyrosine kinase inhibitor (TKI)** that is highly effective for CML [1], [2]. - However, TKIs are generally **contraindicated in pregnancy** due to potential **teratogenic effects** on the developing fetus. *Leukapheresis* - **Leukapheresis** is a procedure used to reduce the **white blood cell count** significantly and rapidly, often in cases of **hyperleukocytosis**. - It is a supportive measure for managing very high cell counts but not a definitive long-term treatment for CML itself, especially in the chronic phase. *Spleenectomy* - **Spleenectomy** may be considered in CML for specific complications such as massive **splenomegaly**, **splenic infarction**, or severe **thrombocytopenia** secondary to splenic sequestration. - It is not a primary first-line treatment for chronic phase CML, particularly in pregnant women, as it doesn't target the underlying **Philadelphia chromosome abnormality**.

Q: Multiple episodes of acute chest syndrome are associated with which of the following conditions?

Sickle Cell Disease. ***Sickle Cell Disease*** - **Acute chest syndrome** is a leading cause of morbidity and mortality in patients with **sickle cell disease (SCD)**. - It involves new pulmonary infiltrates with fever, chest pain, or respiratory symptoms, often triggered by fat embolism, infection, or vaso-occlusion. *SLE* - While **Systemic Lupus Erythematosus (SLE)** can affect the lungs (e.g., **lupus pneumonitis**, **pulmonary hemorrhage**), it does not typically manifest as recurrent "acute chest syndrome" in the same distinct pattern as SCD [1]. - Lung involvement in SLE is usually due to inflammation or vasculitis, not vaso-occlusive crises [1]. *Sjogren's syndrome* - **Sjogren's syndrome** is primarily an autoimmune disease affecting exocrine glands, leading to dry eyes and mouth. - Pulmonary involvement in Sjogren's, such as **interstitial lung disease** or **bronchiolitis**, is less common and differs from acute chest syndrome. *Bronchiectasis* - **Bronchiectasis** is characterized by permanent dilation and damage to the airways, leading to chronic cough and recurrent infections. - While it involves recurrent lung problems, its pathology and clinical presentation are distinct from the acute, crisis-driven nature of acute chest syndrome.

Q: All of the following statements about Burkitt's lymphoma are true, Except:

Radiotherapy is the treatment of choice. ***Radiotherapy is the treatment of choice*** - Treatment for **Burkitt's lymphoma** primarily involves **chemotherapy**, not radiotherapy, due to the aggressive nature of the disease. - While radiotherapy may be used in some cases, it is **not a standard treatment** for this highly aggressive B-cell lymphoma [1]. *Can present as an abdominal mass* - Burkitt's lymphoma can indeed present as an **abdominal mass**, especially in pediatric cases involving the **ileocecal region**. - The **extranodal** involvement is common, contributing to the mass effect in the abdomen [1]. *B cell lymphoma* - Burkitt's lymphoma is classified as a **B-cell lymphoma** arising from the germinal center of B cells [1]. - It is characterized by high proliferation rates and mutations in B cell-related genes, confirming it as a B-cell malignancy [1]. *8, 14 translocation* - A hallmark of Burkitt's lymphoma is the **translocation of the MYC gene** on chromosome 8 and chromosome 14, leading to abnormal cell proliferation. - This genetic alteration is fundamental in the cancer's pathogenesis and is a criterion for diagnosis.

Question 1

A 32-year-old woman presents with fever, fatigue, jaundice, and anemia. Laboratory results show hemoglobin of 7.5, a high reticulocyte count, and elevated indirect bilirubin. The peripheral smear reveals spherocytes. What is the definitive treatment?

Accepted Answer

Corticosteroids

Answer

Splenectomy

Answer

Blood transfusion

Answer

Immunosuppressive therapy

Question 2

In which condition are multiple lytic lesions typically seen?

Accepted Answer

Multiple myeloma

Answer

Mitral stenosis

Answer

Atherosclerosis

Answer

Osteoporosis

Question 3

Choose the best method of diagnosis for the clinical sign represented in the image.

Accepted Answer

Serum ceruloplasmin

Answer

Serum copper

Answer

Karyotyping

Answer

PCR

Question 4

What is the first-line treatment for idiopathic thrombocytopenia purpura (ITP)?

Accepted Answer

Corticosteroids

Answer

Splenectomy

Answer

Intravenous immunoglobulins (IVIG)

Answer

Blood transfusion

Question 5

Which of the following is typically not associated with secondary Idiopathic thrombocytopenic purpura?

Accepted Answer

Rheumatoid arthritis

Answer

HIV infection

Answer

Systemic lupus erythmatosus

Answer

Hepatitis C infection

Question 6

What is the earliest hematological change following splenectomy?

Accepted Answer

Leukocytosis and thrombocytosis

Answer

Presence of Heinz bodies

Answer

Evidence of Howell Jolly bodies

Answer

Poikilocytosis

Question 7

Which of the following statements is false regarding beta thalassemia major?

Accepted Answer

Bone marrow iron is depleted

Answer

Growth and development is impaired

Answer

Red cell count is typically low

Answer

Levels of HbA2 are typically increased

Question 8

Treatment of chronic phase of CML in pregnant women is -

Accepted Answer

Interferon therapy

Answer

Leukapheresis

Answer

Imatinib

Answer

Spleenectomy

Question 9

Multiple episodes of acute chest syndrome are associated with which of the following conditions?

Accepted Answer

Sickle Cell Disease

Answer

SLE

Answer

Sjogren's syndrome

Answer

Bronchiectasis

Question 10

All of the following statements about Burkitt's lymphoma are true, Except:

Accepted Answer

Radiotherapy is the treatment of choice

Answer

B cell lymphoma

Answer

8, 14 translocation

Answer

Intensive chemotherapy is the treatment of choice

Hematology — MCQs

Hematology — MCQs

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