Hematology Practice Questions

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: 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 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 percentage of patients with multiple myeloma that exhibit vertebral involvement?

88% of patients. ***66%*** - Approximately **66% of patients** with multiple myeloma exhibit some degree of **vertebral involvement**, primarily due to osteolytic lesions. - These lesions lead to **vertebral compression fractures** and pain, making vertebrae a common site of affection in this condition [1]. *22%* - This figure is significantly lower than the established data indicating **higher vertebral involvement** in multiple myeloma patients. - Such a low percentage does not reflect the **typical clinical realities** associated with this disease. *88%* - While vertebral involvement is common, **88%** is an overestimation and not supported by clinical evidence. - The risk of **spinal lesions** generally peaks around **66%**, indicating a lower frequency than suggested here. *44%* - This percentage underrepresents the true prevalence of vertebral involvement in multiple myeloma, which is higher. - Clinical findings consistently show that most patients demonstrate at least **some level of vertebral affection**.

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: 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: In which of the following conditions is Bendamustine most commonly used for treatment?

Non-Hodgkin lymphoma (NHL). ***Non-Hodgkin lymphoma (NHL)*** - **Bendamustine** is an **alkylating agent** frequently used in the treatment of various subtypes of **Non-Hodgkin lymphoma (NHL)**, particularly in relapsed or refractory settings, or as first-line therapy [1]. - It is known for its efficacy with a relatively manageable toxicity profile compared to other alkylating agents in NHL [1]. *Colon carcinoma* - **Bendamustine** is **not a standard treatment** for **colon carcinoma**. - Treatment regimens for colon carcinoma commonly involve agents like **5-fluorouracil**, **oxaliplatin**, and **irinotecan**. *Breast carcinoma* - **Bendamustine** is **not a primary chemotherapy agent** for **breast carcinoma**. - Standard chemotherapy for breast cancer often includes **anthracyclines**, **taxanes**, and **cyclophosphamide**. *Chronic lymphocytic leukemia (CLL)* - While **Bendamustine** is also approved and used for **Chronic lymphocytic leukemia (CLL)**, especially in combination with rituximab, it is arguably **most commonly associated with NHL** due to its broader application across various NHL subtypes and different lines of therapy [1]. - For CLL, it is an important option, but its use in NHL is generally more encompassing [1].

Q: Which of the following is the MOST characteristic feature of Hemolytic Uremic Syndrome (HUS)?

Microangiopathic hemolytic anemia. ### a and c - Hemolytic Uremic Syndrome (HUS) is characterized by **microangiopathic hemolytic anemia** along with **thrombocytopenia**, making this correct [1][2]. - It typically results from an **infection**, often associated with **E. coli**, particularly in children [2]. *Positive Coomb's test* - A positive Coomb's test suggests **autoimmune hemolytic anemia**, not HUS. - HUS generally presents with **negative Coomb's test**, indicating hemolysis mechanism is different from autoimmune causes. *Microangiopathic haemolytic anaemia* - While HUS does involve **microangiopathic hemolytic anemia** [2], this alone does not encompass all its characteristics necessary for diagnosis. - HUS must also include **thrombocytopenia**, which is not mentioned in this option [1]. *Thrombocytopenia* - Thrombocytopenia is indeed a feature of HUS, but presenting it alone lacks the context of the complete syndrome [1]. - HUS must also show **microangiopathic hemolytic anemia** to align with the complete clinical picture [2], which is missing here.

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.

Q: Hypercoagulability due to a defective factor V gene is known as which of the following?

Leiden mutation. ***Leiden mutation*** - The **factor V Leiden mutation** leads to a hypercoagulable state by causing resistance to activated protein C, increasing the risk of venous thrombosis [1]. - It is the most common inherited cause of venous thromboembolism and directly associates with the defective factor V gene [1]. *Antiphospholipid syndrome* - This syndrome is characterized by the presence of **antiphospholipid antibodies** leading to recurrent thrombosis but is not directly related to the factor V gene. - It often presents with symptoms such as **venous and arterial thrombosis**, but its mechanism differs from that of factor V Leiden [2]. *Inducible thrombocytopenia syndrome* - This condition involves **thrombocytopenia** (low platelet count) often due to medication, which is unrelated to the hypercoagulable state from factor V mutation. - It does not involve the **factor V gene** nor lead to an increased risk of clotting as seen in the Leiden mutation. *Lisbon mutation* - The Lisbon mutation is a rare variant of factor V but is not the primary mutation associated with the hypercoagulable state in most cases. - It does not have the same wide clinical implications or prevalence as the **factor V Leiden mutation**.

Question 1

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

Accepted Answer

Corticosteroids

Answer

Splenectomy

Answer

Intravenous immunoglobulins (IVIG)

Answer

Blood transfusion

Question 2

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 3

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 4

What is the percentage of patients with multiple myeloma that exhibit vertebral involvement?

Accepted Answer

88% of patients

Answer

66% of patients

Answer

22% of patients

Answer

44% of patients

Question 5

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 6

Treatment of chronic phase of CML in pregnant women is -

Accepted Answer

Interferon therapy

Answer

Leukapheresis

Answer

Imatinib

Answer

Spleenectomy

Question 7

In which of the following conditions is Bendamustine most commonly used for treatment?

Accepted Answer

Non-Hodgkin lymphoma (NHL)

Answer

Colon carcinoma

Answer

Breast carcinoma

Answer

Chronic lymphocytic leukemia (CLL)

Question 8

Which of the following is the MOST characteristic feature of Hemolytic Uremic Syndrome (HUS)?

Accepted Answer

Microangiopathic hemolytic anemia

Answer

Thrombocytopenia

Answer

Positive Coomb's test

Answer

All of the options

Question 9

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

Question 10

Hypercoagulability due to a defective factor V gene is known as which of the following?

Accepted Answer

Leiden mutation

Answer

Antiphospholipid syndrome

Answer

Prothrombin G20210A mutation

Answer

Protein C deficiency

Hematology — MCQs

Hematology — MCQs

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