Hematology Practice Questions

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: Which of the following statements about Fanconi anemia is false?

Type I RTA. ***Type I RTA*** - **Fanconi's anemia** is mainly associated with **proximal renal tubular acidosis (RTA)**, not type I RTA, which specifically affects distal tubule function. - The renal manifestations include **glycosuria**, **aminoaciduria**, and **phosphate wasting**, distinct from the characteristics of type I RTA. *Pancytopenia* - **Pancytopenia** is a common feature of Fanconi's anemia due to **bone marrow failure** resulting in decreased blood cell production. - It manifests as a decrease in **red blood cells**, **white blood cells**, and **platelets** which are critical signs of this condition. *Autosomal recessive* - Fanconi's anemia is classified as an **autosomal recessive disorder**, requiring two copies of the mutated gene for the disease to manifest. - It involves multiple genetic defects that can affect the body's ability to repair DNA. *All are true* - This statement is incorrect as it includes the false assertion regarding **Type I RTA**, which does not apply to Fanconi's anemia. - At least one of the previous options is false, making this statement invalid.

Q: Sickle cell anemia leads to resistance towards?

Plasmodium falciparum. ***Plasmodium falciparum*** - Individuals with sickle cell trait or disease have **red blood cells** that are less hospitable to the growth and reproduction of **_Plasmodium falciparum_**, the most dangerous malaria parasite [1]. - The abnormal hemoglobin (HbS) reduces parasite growth, and infected sickle cells are prematurely destroyed, limiting parasite burden [1]. *Plasmodium malariae* - While all malaria parasites infect red blood cells, the selective pressure from **sickle cell anemia** is primarily against **_Plasmodium falciparum_**, not _P. malariae_ [2]. - There is no significant evidence of increased resistance to **_P. malariae_** in individuals with sickle cell disease. *Plasmodium vivax* - **_Plasmodium vivax_** preferentially invades **reticulocytes** (immature red blood cells), and its life cycle is not significantly altered by the presence of **hemoglobin S** in mature red blood cells [2]. - Resistance to _P. vivax_ is more commonly associated with the absence of the **Duffy antigen**, not sickle cell anemia. *Plasmodium ovale* - Like _P. vivax_, **_Plasmodium ovale_** also predominantly infects **reticulocytes** [2]. - There is no strong evidence to suggest that sickle cell anemia confers significant protection against **_P. ovale_** infections.

Q: Mentzer index more than 13 suggests a diagnosis of

Iron deficiency anemia. ***Iron deficiency anemia*** - A Mentzer index of **greater than 13** (calculated as MCV/RBC count) is highly suggestive of **iron deficiency anemia**. [1] - This index helps differentiate iron deficiency from thalassemia trait in microcytic anemias, as **MCV is disproportionately low** compared to RBC count in IDA. *Thalassemia* - Thalassemia typically presents with a **Mentzer index less than 13**, meaning the MCV reduction is proportional to or less severe than the RBC count reduction. - This condition is characterized by **reduced alpha or beta globin chain synthesis**, leading to microcytic hypochromic red blood cells but often with an elevated RBC count. *Hereditary Spherocytosis* - Hereditary spherocytosis is a **normocytic or macrocytic anemia**, not typically microcytic, making the Mentzer index less relevant for its diagnosis. - It is characterized by **spherocytes** on peripheral smear and increased red cell fragility due to membrane defects. *Autoimmune Hemolytic Anemia* - Autoimmune hemolytic anemia is typically a **normocytic to macrocytic anemia** [2] with features of hemolysis, such as elevated LDH and indirect bilirubin. - While it can be severe, it is not primarily characterized by **microcytosis** and therefore the Mentzer index is not a diagnostic tool for this condition.

Q: Which of the following is LEAST likely to increase the risk of developing deep vein thrombosis (DVT)?

Age less than 40. ### Age less than 40 - Younger age is considered a **protective factor** against DVT, as the risk generally increases with age due to accumulated comorbidities and decreased mobility. [1] - While DVT can occur at any age, the incidence is significantly lower in individuals under 40 years old, especially in the absence of other strong risk factors. [3] ### Prolonged immobilization - **Immobilization** significantly reduces blood flow velocity in the lower extremities, leading to **venous stasis**. [2] - This stasis promotes the accumulation of clotting factors and activation of the coagulation cascade, increasing the risk of thrombus formation. ### Obesity - Obesity is a significant risk factor for DVT due to its association with **chronic inflammation**, **endothelial dysfunction**, and a **prothrombotic state**. - Increased abdominal pressure from obesity can also impede venous return, further contributing to venous stasis. ### Extensive pelvic surgery of more than 30 minutes - Surgical procedures, particularly those involving the pelvis and lasting longer than 30 minutes, cause **endothelial injury**, activate the **coagulation system**, and result in **postoperative immobility**. [2] - These factors collectively contribute to Virchow's triad (venous stasis, endothelial injury, hypercoagulability), significantly increasing DVT risk.

Q: What is used for exchange blood transfusion?

Whole blood. ***Whole blood*** - Exchange blood transfusion aims to replace a significant volume of the patient's blood with donor blood, making **whole blood** the most appropriate component. - Using whole blood helps to concurrently address **anemia** (due to red blood cells), correct **coagulopathies** (due to plasma and platelets), and remove harmful substances like bilirubin or antibodies. *EPP* - EPP likely refers to **Erythrocyte Progenitor Cells** or another experimental component not routinely used for massive blood replacement. - It does not contain the plasma or platelets necessary for comprehensive exchange transfusion. *Serum* - **Serum** is plasma from which clotting factors have been removed and primarily contains antibodies and other proteins. - It lacks red blood cells and clotting factors, making it unsuitable for an exchange transfusion that requires oxygen-carrying capacity and coagulation support. *Packed cells* - **Packed red blood cells** are primarily used to increase oxygen-carrying capacity and treat anemia. - While they are a component of whole blood, they lack plasma and platelets, which are crucial for maintaining oncotic pressure, replacing clotting factors, and diluting harmful substances during an exchange transfusion.

Q: Which of the following statements about Hematochromatosis is true?

Hypogonadism may be seen. *Hypogonadism may be seen* - **Iron deposition** in the **pituitary gland** and **gonads** can lead to hormonal dysfunction, manifesting as **hypogonadism**. [1] - This symptom is common in **hemochromatosis** due to the systemic effects of iron overload. *Arthropathy is not a hallmark feature* - **Arthropathy** is a **hallmark feature** of hemochromatosis, often presenting as chronic pain in the **second and third metacarpophalangeal joints**. - It is caused by **iron deposition** in the joints, leading to inflammation and damage. *Desferrioxamine is treatment of choice* - While **desferrioxamine** is an iron chelating agent, the **treatment of choice** for hereditary hemochromatosis is generally **phlebotomy**, which is more effective at removing excess iron. [2] - Chelating agents like desferrioxamine are typically reserved for patients who cannot undergo phlebotomy, such as those with **anemia** or **cardiac disease**. *Diabetes Mellitus is rare in Hematochromatosis* - **Diabetes mellitus**, often referred to as "**bronze diabetes**" due to associated skin pigmentation, is a **common complication** of hemochromatosis. [1] - It results from **iron deposition** in the **pancreas**, leading to damage of **beta-islet cells** and impaired insulin production.

Q: A female patient presented with fatigue and a history of piles. Routine complete blood count analysis showed hemoglobin of 9 g/dL, MCV 60fL, and RBC count of 5.2 million. A peripheral smear is provided. Which of the following is the next best investigation after the smear for this patient?

Serum ferritin levels. ***Serum ferritin levels*** - The **low hemoglobin** and **low MCV (microcytic anemia)** indicate a likely iron deficiency, commonly assessed by serum ferritin levels [1]. - The patient's **history of piles** suggests possible gastrointestinal bleeding, further pointing to the need for iron studies. *Serum folate levels* - Typically evaluated in cases of **macrocytic anemia**, which is not indicated here due to a **low MCV**. - Folate deficiency leads to larger, immature red cells, contrasting the findings of microcytic anemia in this patient. *Serum homocysteine levels* - While elevated levels can indicate **vitamin B12 or folate deficiency**, they are not specific for iron deficiency anemia. - The current presentation does not suggest deficiencies of B12 or folate, making this test less relevant. *HbA2 levels* - Useful in diagnosing **beta-thalassemia trait**, which remains a differential diagnosis given the relatively **elevated RBC count (5.2 million)** and a **Mentzer index below 13** (MCV/RBC = 60/5.2 ≈ 11.5), a pattern more consistent with thalassemia trait than iron deficiency alone. - However, serum ferritin is the preferred **initial** test to assess iron stores first, as iron deficiency is the more common cause and must be excluded; HbA2 testing is indicated if ferritin is normal or after iron deficiency has been corrected [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Q: A young patient presents with a large retroperitoneal hemorrhage and a history of intermittent knee swelling after strenuous exercise. There is no history of mucosal bleeding. Which of the following clotting factors is primarily deficient in Hemophilia A?

Factor VIII. ***Factors VIII and IX*** - The symptoms indicate a **hemophilia**, where deficiencies in factors VIII or IX lead to a tendency for **retroperitoneal hemorrhage** and joint swelling, particularly after exercise [1]. - Intermittent swelling of the knees indicates **hemarthrosis**, a common manifestation in hemophilia, supporting the dysfunction of these factors [1]. *Factors XI and XII* - These factors are part of the **intrinsic pathway** but are not primarily associated with **joint bleeding** or sporadic retroperitoneal hemorrhage in young patients. - Deficiencies of these factors typically lead to a milder form of bleeding disorders and not the severe joint symptoms seen here. *Von Willebrand factor* - This factor is crucial for **platelet adhesion** and is associated with **mucosal bleeding**, which is not reported in this patient's history [2]. - The patient's **retroperitoneal hemorrhage** and knee swelling are not characteristic of von Willebrand disease, which typically involves more superficial bleeding [1,2]. *Lupus anticoagulant* - This is an **antiphospholipid antibody** associated with **thrombosis** rather than bleeding, and does not explain the joint swelling or hemorrhagic symptoms. - It leads to a false-positive on coagulation tests and can result in complications like recurrent **thromboembolic events**, rather than bleeding tendencies.

Q: Which of the following statements about deep venous thrombosis (DVT) is incorrect?

Mostly bilateral. ***Mostly bilateral*** - DVT is typically **unilateral**, affecting one limb [2]. Bilateral DVT is less common, making this statement incorrect [2]. - The symptoms of **pain, swelling, and tenderness** are usually localized to one leg [1]. *Most common clinically presents as pain and tenderness in calf* - **Calf pain and tenderness** are common clinical presentations of DVT, especially in the lower extremities [1]. - Other common signs include **swelling, warmth, and redness** in the affected leg [1]. *Some cases may directly present as pulmonary thromboembolism* - A significant concern with DVT is that a **clot can dislodge** and travel to the lungs, causing a **pulmonary embolism (PE)**, which can be the initial presentation [1]. - PE is a potentially life-threatening complication and may present with **dyspnea, chest pain, and hemoptysis**. *Clinical assessment highly reliable* - **Clinical assessment alone is not highly reliable** for diagnosing DVT due to its variable and often non-specific presentation [2]. - A definitive diagnosis usually requires objective diagnostic tests such as **compression ultrasonography** or **D-dimer assay** [3].

Question 1

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 2

Which of the following statements about Fanconi anemia is false?

Accepted Answer

Type I RTA

Answer

Associated with pancytopenia

Answer

All statements are true

Answer

Autosomal recessive

Question 3

Sickle cell anemia leads to resistance towards?

Accepted Answer

Plasmodium falciparum

Answer

Plasmodium malariae

Answer

Plasmodium vivax

Answer

Plasmodium ovale

Question 4

Mentzer index more than 13 suggests a diagnosis of

Accepted Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Hereditary Spherocytosis

Answer

Autoimmune Hemolytic Anemia

Question 5

Which of the following is LEAST likely to increase the risk of developing deep vein thrombosis (DVT)?

Accepted Answer

Age less than 40

Answer

Obesity

Answer

Prolonged immobilization

Answer

Extensive pelvic surgery of more than 30 minutes

Question 6

What is used for exchange blood transfusion?

Accepted Answer

Whole blood

Answer

EPP

Answer

Serum

Answer

Packed cells

Question 7

Which of the following statements about Hematochromatosis is true?

Accepted Answer

Hypogonadism may be seen

Answer

Desferrioxamine is treatment of choice

Answer

Arthropathy is not a hallmark feature

Answer

Diabetes Mellitus is rare in Hematochromatosis

Question 8

A female patient presented with fatigue and a history of piles. Routine complete blood count analysis showed hemoglobin of 9 g/dL, MCV 60fL, and RBC count of 5.2 million. A peripheral smear is provided. Which of the following is the next best investigation after the smear for this patient?

Accepted Answer

Serum ferritin levels

Answer

HbA2 levels

Answer

Serum folate levels

Answer

Serum homocysteine levels

Question 9

A young patient presents with a large retroperitoneal hemorrhage and a history of intermittent knee swelling after strenuous exercise. There is no history of mucosal bleeding. Which of the following clotting factors is primarily deficient in Hemophilia A?

Accepted Answer

Factor VIII

Answer

Von Willebrand factor

Answer

Lupus anticoagulant

Answer

Factor IX

Question 10

Which of the following statements about deep venous thrombosis (DVT) is incorrect?

Accepted Answer

Mostly bilateral

Answer

Most common clinically presents as pain and tenderness in calf

Answer

Some cases may directly present as pulmonary thromboembolism

Answer

Clinical assessment highly reliable

Hematology — MCQs

Hematology — MCQs

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