Hematology — MCQs

30 years old came with complaints of easy fatigability, exertional dyspnea, and weight loss. She also complains of frequent falls. physical examination revealed there was a bilateral decrease in vibration sense. Her hemoglobin levels were 8.2g%. She was treated with folate. Her anemia improved but neurological symptoms worsened. Which of the following is the most probable reason for her condition?

Hematology Indian Medical PG Practice Questions and MCQs

Question 1051: Which of the following statements about Hematochromatosis is true?

A. Hypogonadism may be seen (Correct Answer)
B. Desferrioxamine is treatment of choice
C. Arthropathy is not a hallmark feature
D. Diabetes Mellitus is rare in Hematochromatosis

Explanation: *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.

Question 1052: On medical check-up of a Punjabi student, the following findings were seen: Hb of 9.9 gm/dL, RBC count of 5.1 million, MCV of 62.5 fL, and RDW of 13.51%. What is the most probable diagnosis?

A. HbD
B. Thalassemia trait (Correct Answer)
C. Iron deficiency anemia
D. Anemia of chronic disease

Explanation: ***Thalassemia trait*** - The low **MCV (62.5 fl)** and normocytic **RBC count (5.1 million)** indicate microcytic anemia, typical of thalassemia trait. - The **RDW of 13.51%** is relatively normal, distinguishing it from iron deficiency anemia where RDW would be elevated. *Anemia of chronic disease* - This condition usually presents with **normocytic or microcytic anemia**, but typically involves a **low serum iron** and **high ferritin**, which are not mentioned here [1]. - It is often associated with chronic inflammatory conditions, not fitting the RBC parameters given [2]. *HbD* - HbD is a hemoglobin variant typically identified in **specific ethnic groups**, but it does not cause significant anemia like described here with low MCV. - Diagnosis is based on specific hemoglobin electrophoresis patterns, which are not indicated in this case. *Iron deficiency anemia* - Would typically show **lower ferritin levels** and higher RDW, indicating a variation in red cell size, which contrasts with the given RDW [1]. - The RBC count is normal, while iron deficiency often presents with *low RBC counts* and microcytic indices [2].

Question 1053: Which of the following is LEAST likely to increase the risk of developing deep vein thrombosis (DVT)?

A. Obesity
B. Age less than 40 (Correct Answer)
C. Prolonged immobilization
D. Extensive pelvic surgery of more than 30 minutes

Explanation: ### 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.

Question 1054: Sickle cell anemia leads to resistance towards?

A. Plasmodium falciparum (Correct Answer)
B. Plasmodium malariae
C. Plasmodium vivax
D. Plasmodium ovale

Explanation: ***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.

Question 1055: Which of the following statements about Fanconi anemia is false?

A. Type I RTA (Correct Answer)
B. Associated with pancytopenia
C. All statements are true
D. Autosomal recessive

Explanation: ***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.

Question 1056: Female on carbimazole therapy presents with sudden fever, rigors and sore throat. Which is the investigation of choice for this patient?

A. Check blood counts (Correct Answer)
B. Check C-reactive protein
C. Take a throat swab
D. Treat for malaria

Explanation: **Check blood counts** * **Carbimazole** is a known cause of **agranulocytosis**, a severe reduction in **white blood cell count**, making a complete blood count (CBC) crucial for diagnosis. * The symptoms of **fever, rigors, and sore throat** are classic signs of infection due to severe **neutropenia**, necessitating urgent investigation of blood counts [1]. Rigors specifically represent shivering that occurs with a rapid rise in body temperature [1]. * *Check C-reactive protein* * **C-reactive protein (CRP)** is a general marker of inflammation and infection but does not provide specific information about white blood cell counts. * While CRP would likely be elevated in an infection, it wouldn't distinguish between a common infection and **carbimazole-induced agranulocytosis**. * *Take a throat swab* * A **throat swab** could identify a causative pathogen for the **sore throat**, but it would not reveal the underlying cause of the patient's susceptibility to infection. * Prioritizing a throat swab over a CBC could delay the diagnosis of a life-threatening condition like **agranulocytosis**. * *Treat for malaria* * There is no information in the clinical scenario to suggest **malaria**, such as travel history to endemic areas. * Assuming malaria without proper diagnostic workup, especially in the context of carbimazole use, would be an inappropriate and potentially dangerous medical decision.

Question 1057: 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?

A. HbA2 levels
B. Serum ferritin levels (Correct Answer)
C. Serum folate levels
D. Serum homocysteine levels

Explanation: ***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**, but not indicated in the context of evident **microcytic anemia** and fatigue without hemolysis or family history [1]. - The patient's profile does not align with thalassemia, thus making this investigation unnecessary. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Question 1058: 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?

A. Von Willebrand factor
B. Lupus anticoagulant
C. Factor VIII (Correct Answer)
D. Factor IX

Explanation: ***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.

Question 1059: A child presents with intermittent jaundice and splenomegaly. There is a history of similar complaints in the elder brother. Peripheral smear shows the following finding. What is the most appropriate investigation for this condition?

A. Osmotic fragility test (Correct Answer)
B. Coombs test
C. G6PD deficiency testing
D. Flow cytometry for PNH

Explanation: ***Osmotic fragility test*** - The image shows **spherocytes** (dense, small red cells lacking central pallor), a hallmark of **hereditary spherocytosis**. The clinical picture of intermittent jaundice, splenomegaly, and a family history further supports this diagnosis. - The **osmotic fragility test** measures the red blood cell's susceptibility to hemolysis in hypotonic solutions and is the most appropriate test to confirm hereditary spherocytosis. *Coombs test* - The Coombs test (direct antiglobulin test) detects **antibodies on the surface of red blood cells** or in the serum. It is primarily used to diagnose **autoimmune hemolytic anemia**. - Hereditary spherocytosis is a membrane defect, not an immune-mediated condition, so the Coombs test would typically be negative. *G6PD deficiency testing* - **Glucose-6-phosphate dehydrogenase (G6PD) deficiency** leads to episodic hemolytic anemia triggered by oxidative stress (e.g., fava beans, certain drugs, infections). - While it causes episodic hemolysis and jaundice, the peripheral smear typically shows **Heinz bodies** and **bite cells** during hemolytic episodes, not spherocytes, and without specific triggers, it doesn't fit the clinical picture as well as hereditary spherocytosis. *Flow cytometry for PNH* - **Flow cytometry** is used to diagnose **paroxysmal nocturnal hemoglobinuria (PNH)** by detecting the absence of GPI-anchored proteins (e.g., CD55, CD59) on blood cells. - PNH is characterized by dark urine (hemoglobinuria), thrombosis, and bone marrow failure, and its peripheral smear does not typically show spherocytes.

Question 1060: 30 years old came with complaints of easy fatigability, exertional dyspnea, and weight loss. She also complains of frequent falls. physical examination revealed there was a bilateral decrease in vibration sense. Her hemoglobin levels were 8.2g%. She was treated with folate. Her anemia improved but neurological symptoms worsened. Which of the following is the most probable reason for her condition?

A. Folate therapy caused rapid use of Vit B12 stores aggravating symptoms (Correct Answer)
B. Vitamin B6 deficiency unmasked by folate treatment.
C. Impaired folate metabolism in the central nervous system.
D. Malabsorption of folate due to gastrointestinal issues.

Explanation: ***Folate therapy caused rapid use of Vit B12 stores aggravating symptoms*** - This patient likely has **Vitamin B12 deficiency**, which presents with **macrocytic anemia** and **neurological symptoms** like decreased vibration sense and frequent falls [1]. - While folate improves the anemia by allowing erythrocyte maturation, it can **deplete existing B12 stores** and worsen neurological symptoms in the presence of an underlying B12 deficiency [1]. *Vitamin B6 deficiency unmasked by folate treatment.* - **Vitamin B6 deficiency** can cause **microcytic anemia** and neuropathies, but it is not typically associated with the macrocytic anemia and specific neurological deterioration seen here after folate treatment. - Folate treatment itself does not directly unmask or worsen B6 deficiency in this manner. *Impaired folate metabolism in the central nervous system.* - Impaired folate metabolism in the CNS is rare and usually associated with specific genetic disorders or malformations, which does not explain the initial **macrocytic anemia** or the paradoxical worsening of neurological symptoms with folate. - The improvement in anemia with folate suggests that the central problem was not primarily impaired folate metabolism itself. *Malabsorption of folate due to gastrointestinal issues.* - If **folate malabsorption** was the primary issue, folate supplementation would not have improved the anemia, contrary to what is described in the case. - This option does not explain the worsening neurological symptoms after folate treatment.

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Anemia Evaluation and Management

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Hemoglobinopathies

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Thalassemias

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Platelet Disorders

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Coagulation Disorders

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Thrombotic Disorders

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Leukemias

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Lymphomas

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Multiple Myeloma and Plasma Cell Disorders

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Myeloproliferative Neoplasms

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Transfusion Medicine

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Hematopoietic Stem Cell Transplantation

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Hematology — MCQs

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