Hematology Practice Questions

Q: In which of the following conditions that cause polycythemia is the serum erythropoietin extremely low?

Polycythemia vera. The key to answering this question lies in distinguishing between **Primary** and **Secondary Polycythemia**. **1. Why Polycythemia Vera (PV) is correct:** Polycythemia Vera is a **Primary Polycythemia**, a myeloproliferative neoplasm characterized by an autonomous overproduction of red blood cells by the bone marrow, independent of Erythropoietin (EPO) stimulation. In >95% of cases, this is due to a **JAK2 V617F mutation** [1]. Because the red cell mass is high, the body’s normal physiological feedback loop suppresses the production of EPO in the kidneys [2]. Therefore, an **extremely low or subnormal serum EPO level** is a major diagnostic criterion for PV. **2. Why the other options are incorrect:** * **Dehydration (Option A):** This causes **Relative Polycythemia**. The red cell mass is normal, but the plasma volume is decreased. EPO levels remain within the normal range. * **Renal Cell Carcinoma (Option B):** This is a cause of **Secondary Polycythemia**. Certain tumors (RCC, Hepatoma, Cerebellar Hemangioblastoma) can ectopically produce EPO, leading to **elevated** EPO levels. * **Congenital Heart Disease (Option C):** Cyanotic heart diseases cause chronic tissue hypoxia. This triggers the kidneys to increase EPO production physiologically to improve oxygen-carrying capacity [2]. Thus, EPO levels are **elevated**. **High-Yield Clinical Pearls for NEET-PG:** * **Diagnostic Triad for PV:** Increased hemoglobin/hematocrit, JAK2 mutation, and low serum EPO [1]. * **Clinical Sign:** **Aquagenic pruritus** (itching after a warm bath) is highly specific for PV [1]. * **Complication:** Patients are at high risk for both arterial and venous thrombosis (e.g., Budd-Chiari Syndrome) [1]. * **Treatment of choice:** Therapeutic phlebotomy (target Hct <45%) and low-dose aspirin.

Q: A patient presents with splenomegaly, low RBC count, normal WBC and platelet count. Peripheral blood smear shows tear drop cells. Repeated bone marrow aspiration is unsuccessful. What is the probable diagnosis?

Myelofibrosis. ### Explanation The clinical presentation points towards **Primary Myelofibrosis (PMF)**, a myeloproliferative neoplasm characterized by the replacement of bone marrow with collagenous connective tissue [1]. **Why Myelofibrosis is the correct answer:** 1. **Tear Drop Cells (Dacrocytes):** These are the hallmark of myelofibrosis [1]. They occur because RBCs are physically squeezed and damaged as they attempt to pass through the fibrotic marrow and the distorted vasculature of the spleen [1]. 2. **"Dry Tap" on Bone Marrow Aspiration:** The extensive fibrosis (increased reticulin/collagen) prevents the aspiration of marrow contents [1]. This is a classic board-exam finding for PMF. 3. **Splenomegaly:** Due to marrow failure, the body resorts to **Extramedullary Hematopoiesis (EMH)**, primarily in the spleen and liver, leading to significant organomegaly [1]. **Why the other options are incorrect:** * **Thalassemia:** While it can show splenomegaly and target cells, it does not cause a "dry tap" on aspiration. * **Chronic Myeloid Leukemia (CML):** Typically presents with a massively elevated WBC count (leukocytosis) and a hypercellular marrow that is easily aspirated. * **Iron Deficiency Anemia:** Characterized by microcytic hypochromic cells and pencil cells, not tear drop cells. The bone marrow is easily aspirated. **NEET-PG High-Yield Pearls:** * **Leukoerythroblastic Picture:** PMF often shows immature WBCs and nucleated RBCs on the peripheral smear [1]. * **JAK2 Mutation:** Present in approximately 50-60% of PMF cases [1]. * **Silver Stain:** Used on bone marrow biopsy to visualize increased reticulin fibers [1]. * **Differential for Dry Tap:** Remember the mnemonic **"M-3"**: **M**yelofibrosis, **M**etastatic secondary deposits, and **M**yeloid leukemias (like Hairy Cell Leukemia).

Q: What is the most common presentation of sickle cell anemia?

Fever. **Explanation:** In the context of Sickle Cell Anemia (SCA), **Fever** is considered the most common clinical presentation, particularly in the pediatric population. While SCA is characterized by vaso-occlusive events, fever is the most frequent reason for hospital admission and medical evaluation. This is primarily due to the state of **functional hyposplenism** (caused by repeated splenic infarcts), which renders patients highly susceptible to infections by encapsulated organisms like *Streptococcus pneumoniae* and *Haemophilus influenzae* [1]. **Analysis of Options:** * **Bone Pain (Vaso-occlusive Crisis):** This is the most common *symptom* of a sickle cell crisis and the hallmark of the disease [1]. However, statistically, febrile episodes (often associated with underlying infection or acute chest syndrome) occur more frequently as an initial presentation in clinical settings. * **Priapism:** This is a well-known complication involving a painful, persistent erection due to sickling in the corpora cavernosa [1]. While high-yield for exams, it occurs in only about 30-40% of male patients and is not the "most common" presentation. * **Splenomegaly:** While common in early childhood, repeated infarctions lead to **autosplenectomy** (shrunken, fibrotic spleen) by adulthood [1]. Therefore, it is a transient finding rather than the most common presentation across the disease spectrum. **NEET-PG High-Yield Pearls:** * **Most common cause of death in children:** Sepsis (Streptococcus pneumoniae). * **Most common cause of death in adults:** Acute Chest Syndrome (ACS). * **Osteomyelitis in SCA:** While *Staphylococcus aureus* is the most common cause of osteomyelitis generally, **Salmonella** is uniquely associated with and highly characteristic of SCA patients. * **First clinical sign:** Dactylitis (Hand-foot syndrome) usually appearing between 6 months to 2 years of age.

Q: A 17-year-old female with von Willebrand disease presents for dental extraction. What is the appropriate prior line of management?

Desmopressin (DDAVP). **Explanation:** The correct answer is **Desmopressin (DDAVP)**. **1. Why Desmopressin (DDAVP) is the correct answer:** Desmopressin is a synthetic analogue of vasopressin (ADH) that acts on V2 receptors to stimulate the release of **endogenous von Willebrand Factor (vWF)** and **Factor VIII** from the Weibel-Palade bodies of endothelial cells. In patients with Type 1 vWD (the most common form), DDAVP can increase plasma levels of vWF 3 to 5-fold, making it the treatment of choice for minor surgical procedures like dental extractions. It avoids the risks associated with blood products. **2. Why other options are incorrect:** * **Cryoprecipitate:** While it contains vWF and Factor VIII, it is no longer the first-line treatment due to the risk of transfusion-transmitted infections. It is reserved only when specific concentrates are unavailable. * **Fresh Frozen Plasma (FFP):** FFP contains all coagulation factors but in low concentrations. To achieve therapeutic vWF levels, a large volume would be required, risking fluid overload. * **Recombinant Factor VIII:** Pure recombinant Factor VIII lacks vWF. While some vWF-containing concentrates are used in severe cases (Type 3), pure Factor VIII will not correct the primary platelet adhesion defect in vWD. **Clinical Pearls for NEET-PG:** * **Type 1 vWD:** Quantitative deficiency (Most common). Best response to DDAVP. * **Type 2B vWD:** DDAVP is **contraindicated** as it may cause transient thrombocytopenia due to increased platelet aggregation. * **Type 3 vWD:** Total deficiency; DDAVP is ineffective (requires vWF-containing concentrates). * **Side Effects of DDAVP:** Hyponatremia (due to water retention) and facial flushing. Patients should be advised on fluid restriction. * **Diagnostic screening:** Prolonged Bleeding Time (BT) and often a prolonged aPTT. Platelet count is usually normal.

Q: A patient presents with macroglossia and loss of tongue papillae. His Hb is 11.5 g/dL and MCV is 100 fL. What should be the next step in investigating this patient?

Vitamin B12 estimation. ### Explanation The clinical presentation of **macroglossia** (enlarged tongue) combined with the **loss of tongue papillae** (atrophic glossitis/Hunter’s glossitis) and a **borderline high MCV** (100 fL) strongly suggests a nutritional deficiency, specifically **Vitamin B12 deficiency**. **1. Why Vitamin B12 estimation is the correct step:** Atrophic glossitis is a classic physical finding in megaloblastic anemia. Vitamin B12 is essential for DNA synthesis; its deficiency leads to ineffective erythropoiesis (causing macrocytosis/high MCV) and rapid turnover cell depletion, such as the lingual papillae [1]. While the Hb (11.5 g/dL) is only mildly low, the MCV and oral findings are early indicators that necessitate checking serum B12 levels before invasive procedures [3]. **2. Why other options are incorrect:** * **Brush/Incision Biopsy (B & D):** These are indicated for suspected malignancy (e.g., Squamous Cell Carcinoma). However, the diffuse loss of papillae and macroglossia in a patient with macrocytosis point toward a systemic metabolic cause rather than a localized neoplastic process. * **Fluconazole treatment (C):** This is used for Oral Candidiasis (thrush). While thrush can cause tongue discomfort, it typically presents with white curd-like plaques that can be scraped off, not a smooth, beefy red tongue with macrocytosis. **Clinical Pearls for NEET-PG:** * **Hunter’s Glossitis:** Specifically refers to the smooth, "beefy red" tongue seen in B12 deficiency. * **Macroglossia Differential:** Common causes include Amyloidosis (most common chronic cause), Hypothyroidism (Myxedema), Acromegaly, and Down Syndrome [2]. * **MCV Trends:** Always correlate a high-normal or elevated MCV with oral signs; B12 and Folate deficiencies are the most common culprits in the Indian subcontinent [3].

Question 1

Heterozygous sickle cell anemia provides protection against which of the following?

Accepted Answer

Malaria

Answer

G6PD deficiency

Answer

Thalassemia

Answer

Dengue fever

Question 2

In which of the following conditions that cause polycythemia is the serum erythropoietin extremely low?

Accepted Answer

Polycythemia vera

Answer

Dehydration

Answer

Renal cell carcinoma

Answer

Congenital heart disease

Question 3

A patient presents with splenomegaly, low RBC count, normal WBC and platelet count. Peripheral blood smear shows tear drop cells. Repeated bone marrow aspiration is unsuccessful. What is the probable diagnosis?

Accepted Answer

Myelofibrosis

Answer

Thalassemia

Answer

Chronic myeloid leukemia

Answer

Iron deficiency anemia

Question 4

Which of the following is NOT related to the treatment of thalassemia?

Accepted Answer

Routine iron therapy

Answer

Fresh blood transfusion

Answer

Folic acid supplementation

Answer

Deferoxamine improves pregnancy outcome

Question 5

What is the most common presentation of sickle cell anemia?

Accepted Answer

Fever

Answer

Priapism

Answer

Bone pain

Answer

Splenomegaly

Question 6

A 17-year-old female with von Willebrand disease presents for dental extraction. What is the appropriate prior line of management?

Accepted Answer

Desmopressin (DDAVP)

Answer

Cryoprecipitate

Answer

Fresh Frozen Plasma (FFP)

Answer

Recombinant factor VIII

Question 7

Which of the following anemias is caused by H. pylori infection?

Accepted Answer

Iron deficiency anemia

Answer

Sideroblastic anemia

Answer

Hemolytic anemia

Answer

Aplastic anemia

Question 8

In a patient of hemophilia scheduled for dental extraction, which of the following statements is true, EXCEPT?

Accepted Answer

Extraction should be done under general anesthesia and skilled anesthetic care.

Answer

All patients should be screened for HIV.

Answer

Factor VIII or cryoprecipitate may be needed.

Answer

The dose of lignocaine required for anesthesia is the same as for normal individuals.

Question 9

A patient presents with macroglossia and loss of tongue papillae. His Hb is 11.5 g/dL and MCV is 100 fL. What should be the next step in investigating this patient?

Accepted Answer

Vitamin B12 estimation

Answer

Brush biopsy of the lesion

Answer

Fluconazole treatment

Answer

Incision biopsy

Question 10

Which of the following is FALSE regarding sickle cell anemia?

Accepted Answer

High reticulocyte count

Answer

Dactylitis

Answer

Osteomyelitis

Answer

Granulocytosis

Hematology — MCQs

Hematology — MCQs

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