Hematology Practice Questions

Q: A 9-year-old girl has a bleeding diathesis, with prolonged activated partial thromboplastin time (APTT) but normal prothrombin time (PT) and normal platelet count. The diagnosis is?

Von Willebrand's disease. ***Von Willebrand's disease*** - This condition is characterized by a **deficiency or dysfunction of von Willebrand factor (vWF)**, leading to impaired platelet adhesion and stabilization of factor VIII [2], [3]. - While it primarily affects **platelet function**, it can also cause a prolonged **APTT** due to the role of vWF in protecting factor VIII from degradation, thus impacting the intrinsic coagulation pathway [3]. *Factor V deficiency* - Factor V is a component of the **common pathway** of coagulation. - A deficiency would typically prolong both the **PT and APTT** [1]. *Haemophilia A* - This is a deficiency of **Factor VIII**, which is part of the **intrinsic pathway** [1]. - Haemophilia A causes a prolonged **APTT** but a normal PT, similar to the patient's presentation. However, vWD is more common and can also present with similar findings. *Haemophilia B* - This is a deficiency of **Factor IX**, also part of the **intrinsic pathway** [1]. - Haemophilia B also causes a prolonged **APTT** but a normal PT. However, vWD is a more common inheritable bleeding disorder.

Q: Which of the following statements is true regarding pernicious anemia?

Increased red cell MCV. ***Increased risk of gastric carcinoma is unlikely*** - **Pernicious anemia** significantly increases the risk of **gastric carcinoma** due to the chronic inflammation from atrophic gastritis. - This association is well-documented and highlights the **precancerous potential** of the condition. *Increased red cell MCV* - In pernicious anemia, there is often a **macrocytic anemia** characterized by an **increased mean corpuscular volume (MCV)**. - This occurs due to impaired DNA synthesis caused by vitamin B12 deficiency [2]. *Elevated serum levels of methyl malonic acid* - Patients with pernicious anemia will have **elevated levels of methylmalonic acid** due to decreased vitamin B12, impacting the metabolism of certain fatty acids. - This biochemical marker helps in differentiating deficiency of vitamin B12 from other forms of anemia. *Chronic atrophic gastritis* - Chronic atrophic gastritis is a **common feature** in pernicious anemia, leading to gastric mucosal atrophy and impaired intrinsic factor production [1]. - This condition is directly related to the **autoimmune nature** of pernicious anemia, further compromising vitamin B12 absorption [1].

Q: In chronic myeloid leukemia (CML), what is the serum vitamin B12 level?

Increased. ***Increased*** - In **CML**, there is an overproduction of **granulocytes**, which contain **transcobalamin I**, a protein that binds to and transports vitamin B12. - The elevated number of these cells leads to an increase in serum **transcobalamin I**, resulting in higher measured **serum vitamin B12 levels**, even though the biologically active free B12 might not be as high. *Slightly decreased* - This is an unlikely finding in **CML**; conditions like **pernicious anemia** or **malabsorption syndromes** are more typically associated with decreased B12. [1] - A slight decrease would not reflect the underlying proliferative process characteristic of **CML**. *Normal* - While vitamin B12 deficiency is common, a normal level would not align with the typical hematological changes seen in **CML**, where granulocyte proliferation affects vitamin B12 binding proteins. - A normal B12 level would not be a diagnostic indicator in the context of suspected **CML**. *Markedly decreased* - A markedly decreased vitamin B12 level is highly characteristic of conditions such as **pernicious anemia**, **vegan diet without supplementation**, or severe **malabsorption**, not **CML**. [1] - **CML** is associated with an overproduction of **white blood cells**, which usually leads to an *increase* in B12 binding proteins, thus elevating serum B12 levels.

Q: Etiology of disordered coagulation in antiphospholipid syndrome is

Hyper coagulation. ***Hyper coagulation*** - Antiphospholipid Syndrome (APS) is characterized by the presence of **antiphospholipid antibodies** that promote a **prothrombotic state**, leading to both arterial and venous clotting. [1] - These antibodies interfere with regulatory proteins of coagulation (e.g., **protein C, protein S**) and interact with phospholipids on cell surfaces, causing increased platelet activation and endothelial dysfunction. *Slow blood flow* - While **venous stasis** can contribute to thrombosis, it is not the primary etiological factor for disordered coagulation in APS itself. - Slow blood flow is a component of **Virchow's triad**, which also includes endothelial injury and hypercoagulability, with the latter being the core issue in APS. [2] *Thrombocytosis* - **Thrombocytosis** (an abnormally high platelet count) can increase the risk of thrombosis, but it is not the direct or primary cause of disordered coagulation in APS. - APS specificially involves **antibody-mediated hypercoagulability**, not just an increased number of platelets. *Vitamin K malabsorption* - **Vitamin K malabsorption** can lead to a *deficiency* in vitamin K-dependent clotting factors, typically resulting in a **bleeding tendency** rather than hypercoagulation. - This condition is associated with **hypocoagulability**, which is the opposite of the disordered coagulation seen in APS.

Q: Which of the following statements regarding G6PD deficiency are true? 1) It is an X-linked recessive disorder, 2) Bite cells are present in the blood smear, 3) It protects against kala-azar, 4) Enzyme levels are not directly proportional to the age of red blood cells, 5) There is a male predilection.

2 and 5. **2 and 5** - **Bite cells** (or degmacytes) are characteristic of G6PD deficiency, forming when macrophages remove membrane-bound denatured hemoglobin (Heinz bodies) from red blood cells. [2] - The disorder exhibits a **male predilection** because it is an **X-linked recessive genetic condition** [2], meaning males (XY) are affected with a single defective gene copy, whereas females (XX) usually need two defective copies to express the disease fully. *1 and 2* - While **bite cells** are indeed present in G6PD deficiency and it is an **X-linked recessive disorder** [2], this option is incomplete as it misses other true statements. - The statement that **enzyme levels are not directly proportional to the age of red blood cells** is incorrect; G6PD activity normally declines as red blood cells age. *1 and 3* - **G6PD deficiency is an X-linked recessive disorder** [2], but it provides protection against **malaria** [1], not kala-azar (leishmaniasis). - Protection against kala-azar is more commonly associated with other genetic conditions, not G6PD deficiency. *1, 2, 4, and 5* - While **G6PD deficiency is an X-linked recessive disorder** [2], **bite cells** are present, and there is a **male predilection**, statement 4 is incorrect. - **G6PD enzyme levels are directly proportional to the age of red blood cells**; older red blood cells have less enzyme activity, making them more susceptible to oxidative stress, which explains statement 4 is actually false.

Q: A 29-year-old woman was found to have a hemoglobin level of 7.8 mg/dl, with a reticulocyte count of 0.8%. The peripheral blood smear showed microcytic hypochromic anemia. On high-performance liquid chromatography (HPLC), hemoglobin A2 and hemoglobin F were 2.4% and 1.3%, respectively. The serum iron and total iron-binding capacity (TIBC) were 15 mg/dl and 420 micrograms per deciliter, respectively. What is the most likely cause of anemia in this patient?

Iron deficiency anemia. ### Iron deficiency anemia - The **low serum iron** level (15 mg/dl) and **high TIBC** (420 micrograms/dl) are classic indicators of iron deficiency. - Microcytic hypochromic anemia with a low reticulocyte count further supports this diagnosis, indicating insufficient **iron** for erythropoiesis [1]. [2] ### Beta thalassemia minor - Typically presents with **normal serum iron** levels, which is not consistent with the low iron levels seen here. - HPLC would show elevated hemoglobin A2, which is **not observed** in this patient's results. ### Sideroblastic anemia - Characterized by the presence of **ringed sideroblasts** in the bone marrow and often has normal or increased serum iron levels. - The iron levels reported here do not align with what is expected in sideroblastic anemia. ### Anemia due to chronic infection - Often associated with normocytic or mild microcytic anemia but does not typically lead to such **low serum iron** levels with high TIBC [1]. - This type typically presents with **inflammatory markers** and altered iron metabolism different from the findings in this case.

Q: A patient has just been admitted to the hospital for observation. Based on the patient's laboratory results, which indicate a hematocrit of 45%, hemoglobin of 16 g/dL, and platelets of 50 × 10^9/L, what is the most likely primary problem?

Immune Thrombocytopenic Purpura (ITP). ***Recurrent nose bleeds*** - The combination of a **hemoglobin level of 16 g/dL** and **platelet count of 50 × 10^9/L** suggests a thrombocytopenic disorder, which can lead to easy bruising and **epistaxis (nose bleeds)** [1]. - The decreased platelet count is significant, as it can cause bleeding tendencies [1], aligning with the presentation of recurrent nose bleeds [1]. *Hemochromatosis* - Typically associated with **elevated iron levels** and may lead to symptoms like fatigue and joint pain, not primarily affecting platelet count. - Hemoglobin and hematocrit levels are usually normal in hemochromatosis unless secondary complications arise. *Deep vein thrombosis* - Presents with localized swelling, pain, and tenderness in the affected limb, not associated with isolated thrombocytopenia. - Laboratory findings may show changes in coagulation but not specifically low platelet counts. *Hepatic vein thrombosis* - Known as Budd-Chiari syndrome, characterized by abdominal pain, ascites, and liver dysfunction, typically not focused on low platelet count alone. - Although it can cause complications in blood flow, it does not directly correlate with recurrent nose bleeds as a primary symptom.

Q: What is the most effective method to prevent transfusion-associated graft-versus-host disease?

Irradiation. Irradiation - **Irradiation** of blood products inactivates donor T-lymphocytes, which are responsible for initiating the immune attack in **transfusion-associated graft-versus-host disease (TA-GVHD)**. - This method is highly effective in preventing TA-GVHD, especially in immunocompromised patients or those receiving blood from biologically related donors. *Washing* - **Washing** removes plasma proteins, antibodies, and some leukocytes but is not effective in removing or inactivating all viable T-lymphocytes that can cause TA-GVHD. - It's primarily used to prevent allergic reactions or to remove excess potassium in certain patient populations. *Chemical treatment* - While experimental chemical treatments for pathogen inactivation exist, there is no widely accepted or clinically proven chemical treatment specifically for preventing **TA-GVHD** by inactivating lymphocytes. - Such methods are not part of routine blood banking practices for this purpose. *None of the options* - This option is incorrect because **irradiation** is a recognized and effective method for preventing **transfusion-associated graft-versus-host disease**.

Q: The drug of choice for chronic myeloid leukemia (CML) is:

Imatinib. ***Imatinib*** - **Imatinib** is a **tyrosine kinase inhibitor (TKI)** that specifically targets the **BCR-ABL fusion protein**, which is the hallmark of **chronic myeloid leukemia (CML)** [1], [2]. - Its high specificity and effectiveness in inhibiting the proliferation of CML cells make it the **first-line therapy** and drug of choice for this condition [2]. *Cisplatin* - **Cisplatin** is a **platinum-based chemotherapy agent** commonly used in the treatment of various solid tumors, such as lung, ovarian, and testicular cancers. - It works by forming **DNA adducts**, leading to cell death, but it is not a targeted therapy for CML. *Methotrexate* - **Methotrexate** is an **antimetabolite** that inhibits **folate reductase**, used in the treatment of certain cancers (e.g., leukemia, lymphoma), autoimmune diseases (e.g., rheumatoid arthritis), and ectopic pregnancy. - While used in some leukemias, it is **not the primary treatment for CML**, which requires targeted therapy against the BCR-ABL protein. *Tamoxifen* - **Tamoxifen** is a **selective estrogen receptor modulator (SERM)** used primarily in the treatment and prevention of **estrogen receptor-positive breast cancer**. - It works by blocking estrogen's effects on breast cancer cells and has no role in the treatment of chronic myeloid leukemia.

Q: Which of the following statements about fresh frozen plasma (FFP) is false?

To be used within 30 minutes of having trauma. ***To be used within 30 minutes of having trauma*** - This statement is false because while **fresh frozen plasma (FFP)** should be administered promptly in trauma or massive transfusion protocols, there is no strict "30-minute rule" from the moment of trauma. The promptness depends on the patient's **hemodynamic stability** and **coagulopathy** assessment. - FFP must be thawed before administration, which can take 20-30 minutes, making administration within 30 minutes of the traumatic event often impractical and not a general guideline. *Supplies major coagulation factors* - **Fresh frozen plasma (FFP)** is rich in all coagulation factors, including **Factor V**, **Factor VIII**, and **fibrinogen**, which are vital for hemostasis. - This makes it an essential blood product for patients with multiple coagulation factor deficiencies, such as those with **liver disease** or undergoing massive transfusions. *ABO match not required* - This statement is incorrect. While plasma contains less cellular material, it still contains **ABO antibodies** which can cause transfusion reactions if not matched. - **ABO compatibility** is crucial for FFP transfusions to prevent hemolytic reactions from transfused antibodies reacting with recipient red blood cells. *Should be used in replacement of factors in DIC / trauma* - **Fresh frozen plasma (FFP)** is indicated for the replacement of multiple clotting factors in conditions like **disseminated intravascular coagulation (DIC)**, especially when there is active bleeding or a high risk of bleeding [1]. - In massive trauma, FFP is a crucial component of **massive transfusion protocols** to correct acute coagulopathy caused by blood loss and dilution [1].

Question 1

A 9-year-old girl has a bleeding diathesis, with prolonged activated partial thromboplastin time (APTT) but normal prothrombin time (PT) and normal platelet count. The diagnosis is?

Accepted Answer

Von Willebrand's disease

Answer

Factor V deficiency

Answer

Haemophilia A

Answer

Haemophilia B

Question 2

Which of the following statements is true regarding pernicious anemia?

Accepted Answer

Increased red cell MCV

Answer

Chronic atrophic gastritis

Answer

Elevated serum levels of methyl malonic acid

Answer

Increased risk of gastric carcinoma

Question 3

In chronic myeloid leukemia (CML), what is the serum vitamin B12 level?

Accepted Answer

Increased

Answer

Slightly decreased

Answer

Normal

Answer

Markedly decreased

Question 4

Etiology of disordered coagulation in antiphospholipid syndrome is

Accepted Answer

Hyper coagulation

Answer

Slow blood flow

Answer

Thrombocytosis

Answer

Vitamin K malabsorption

Question 5

Which of the following statements regarding G6PD deficiency are true? 1) It is an X-linked recessive disorder, 2) Bite cells are present in the blood smear, 3) It protects against kala-azar, 4) Enzyme levels are not directly proportional to the age of red blood cells, 5) There is a male predilection.

Accepted Answer

2 and 5

Answer

1 and 2

Answer

1 and 3

Answer

1, 2, 4, and 5

Question 6

A 29-year-old woman was found to have a hemoglobin level of 7.8 mg/dl, with a reticulocyte count of 0.8%. The peripheral blood smear showed microcytic hypochromic anemia. On high-performance liquid chromatography (HPLC), hemoglobin A2 and hemoglobin F were 2.4% and 1.3%, respectively. The serum iron and total iron-binding capacity (TIBC) were 15 mg/dl and 420 micrograms per deciliter, respectively. What is the most likely cause of anemia in this patient?

Accepted Answer

Iron deficiency anemia

Answer

Beta thalassemia minor

Answer

Sideroblastic anemia

Answer

Anemia due to chronic infection

Question 7

A patient has just been admitted to the hospital for observation. Based on the patient's laboratory results, which indicate a hematocrit of 45%, hemoglobin of 16 g/dL, and platelets of 50 × 10^9/L, what is the most likely primary problem?

Accepted Answer

Immune Thrombocytopenic Purpura (ITP)

Answer

Hypersplenism

Answer

Aplastic anemia

Answer

Acute leukemia

Question 8

What is the most effective method to prevent transfusion-associated graft-versus-host disease?

Accepted Answer

Irradiation

Answer

Washing

Answer

Chemical treatment

Answer

None of the options

Question 9

The drug of choice for chronic myeloid leukemia (CML) is:

Accepted Answer

Imatinib

Answer

Methotrexate

Answer

Cisplatin

Answer

Tamoxifen

Question 10

Which of the following statements about fresh frozen plasma (FFP) is false?

Accepted Answer

To be used within 30 minutes of having trauma

Answer

ABO match not required

Answer

Supplies major coagulation factors

Answer

Should be used in replacement of factors in DIC / trauma

Hematology — MCQs

Hematology — MCQs

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