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 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: 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: 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: 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: 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: 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**.

Q: The most common organ to be involved in the acute phase of Graft vs host disease is?

Skin. ***Skin*** - The **skin** is the **most frequently affected organ** in acute graft-versus-host disease (GVHD), with nearly all patients exhibiting some dermatologic manifestations. - Initial symptoms often include a **maculopapular rash** that can progress to erythroderma or bullous lesions. *Bone marrow* - While the bone marrow is the site of the **allogeneic stem cell transplant**, it is not typically considered an **acute target organ** for GVHD symptoms in the same way as the skin, gut, or liver. - Although the donor immune cells originate here, the manifestations of GVHD primarily affect other host tissues. *Liver* - The **liver** is a common organ involved in acute GVHD, often presenting with **elevated bilirubin** and alkaline phosphatase levels. - However, **liver involvement is less common** than skin involvement and typically occurs alongside or after skin manifestations. *Gut* - The **gastrointestinal tract** (gut) is frequently affected in acute GVHD, leading to symptoms like **diarrhea, nausea, vomiting**, and abdominal pain. - Similar to liver involvement, gut involvement is common but **occurs less frequently than skin involvement** as the primary acute manifestation.

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: 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

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 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 3

Treatment of chronic phase of CML in pregnant women is -

Accepted Answer

Interferon therapy

Answer

Leukapheresis

Answer

Imatinib

Answer

Spleenectomy

Question 4

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 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

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 7

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

Question 8

The most common organ to be involved in the acute phase of Graft vs host disease is?

Accepted Answer

Skin

Answer

Bone marrow

Answer

Liver

Answer

Gut

Question 9

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 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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