Hematology Practice Questions

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].

Q: Stage of Hodgkin's lymphoma with right-sided neck nodes and a left inguinal node, without fever?

Stage IIIa. ### Explanation The staging of Hodgkin’s Lymphoma follows the **Ann Arbor Staging System** (modified by the Cotswolds criteria). The stage is determined by the location of lymph node involvement relative to the **diaphragm** [1]. **Why Stage IIIa is correct:** * **Stage III** is defined as involvement of lymph node regions on **both sides of the diaphragm**. In this case, the right-sided neck nodes (cervical) are above the diaphragm, and the left inguinal node is below the diaphragm [1]. * The suffix **'a'** denotes the **absence** of constitutional "B" symptoms (fever, night sweats, or weight loss >10% in 6 months) [1]. Since the patient does not have fever, it is classified as Stage IIIa. **Why other options are incorrect:** * **Stage Ia:** Involvement of a single lymph node region or a single extralymphatic site [1]. This patient has multiple regions involved on both sides of the diaphragm. * **Stage IIa:** Involvement of two or more lymph node regions on the **same side** of the diaphragm [1]. * **Stage IVa:** Diffuse or disseminated involvement of one or more **extralymphatic organs** (e.g., liver, bone marrow, or lungs), with or without associated lymph node involvement [1]. **High-Yield Clinical Pearls for NEET-PG:** 1. **B Symptoms:** Fever (>38°C), drenching night sweats, and unexplained weight loss (>10% in 6 months). Their presence adds the suffix 'b' (e.g., IIIb) [1]. 2. **Bulky Disease:** Defined as a nodal mass >10 cm or >1/3rd of the transthoracic diameter [1]. 3. **Pel-Ebstein Fever:** A classic but rare cyclic fever pattern associated with Hodgkin’s Lymphoma. 4. **Alcohol-induced pain:** Pain in the lymph nodes after drinking alcohol is a highly specific (though uncommon) symptom of Hodgkin’s. 5. **Most Common Subtype:** Nodular Sclerosis is the most common histological subtype overall.

Q: Which of the following is NOT a feature of hereditary spherocytosis?

Increased MCV. Hereditary Spherocytosis (HS) is an autosomal dominant disorder caused by defects in red cell membrane proteins (most commonly **Ankyrin**, followed by Spectrin) [2]. This leads to a loss of membrane surface area, forcing the RBC to adopt a spherical shape rather than a biconcave disc [1], [2]. **Why "Increased MCV" is the correct answer:** In HS, the **MCV (Mean Corpuscular Volume) is typically normal or slightly decreased**. Because the cell loses membrane fragments (vesiculation) while maintaining its internal contents, the cell becomes smaller and more compact. An increased MCV is characteristic of megaloblastic anemias or reticulocytosis, not the primary pathology of HS. **Analysis of incorrect options:** * **Increased MCHC:** This is a **hallmark finding** of HS [1]. As the cell loses surface area but retains hemoglobin, the concentration of hemoglobin per cell increases (often >36 g/dL) [1]. * **Increased Osmotic Fragility:** Spherocytes have a **decreased surface area-to-volume ratio**, making them unable to expand when placed in hypotonic solutions. They lyse much earlier than normal cells. * **Decreased surface area to volume ratio:** This is the fundamental morphological change in HS [1]. The spherical shape is the most geometrically efficient way to hold a volume, resulting in the lowest possible surface area for that volume. **NEET-PG High-Yield Pearls:** * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test (Flow cytometry). * **Screening Test:** Osmotic Fragility Test (using incubated blood). * **Peripheral Smear:** Spherocytes (small, dark cells lacking central pallor). * **Clinical Triad:** Anemia, Jaundice (unconjugated), and Splenomegaly [2]. * **Complication:** Pigmented gallstones (calcium bilirubinate) and Aplastic crisis (associated with Parvovirus B19).

Q: Cold agglutinin disease can be associated with all of the following, except:

Systemic Lupus Erythematosus. **Explanation:** The core concept in this question is differentiating between **Warm** and **Cold Autoimmune Hemolytic Anemia (AIHA)**. **Why Systemic Lupus Erythematosus (SLE) is the correct answer:** SLE is classically associated with **Warm AIHA**, which is mediated by **IgG** antibodies that react at body temperature ($37^\circ\text{C}$) [1]. In contrast, Cold Agglutinin Disease (CAD) is mediated by **IgM** antibodies that react at low temperatures ($0\text{--}4^\circ\text{C}$) [3]. While SLE involves multiple autoantibodies, it is not a typical cause of cold agglutinins [2]. **Analysis of Incorrect Options:** * **Mycoplasma pneumoniae:** This is a classic trigger for **transient/acute CAD** [3]. The bacteria share antigenic similarities with the **I antigen** on human RBCs, leading to cross-reactive IgM production. * **Waldenstrom Macroglobulinemia:** This is a lymphoproliferative disorder characterized by monoclonal IgM production. It is a leading cause of **chronic/primary CAD** [3]. * **Epstein-Barr Virus (EBV):** Similar to Mycoplasma, EBV (Infectious Mononucleosis) can trigger cold agglutinins, specifically targeting the **i antigen** (lowercase 'i') on fetal/neonatal RBCs. **High-Yield Clinical Pearls for NEET-PG:** 1. **Antibody Type:** Warm AIHA = **IgG** (Extravascular hemolysis in Spleen); Cold AIHA = **IgM** (Intravascular or Liver-based hemolysis) [1], [3]. 2. **Antigen Targets:** Mycoplasma = **I antigen**; EBV = **i antigen** [3]. 3. **Blood Smear:** Look for **RBC agglutination** (clumping) in CAD, which disappears upon warming the slide [3]. 4. **Coombs Test:** In CAD, the Direct Antiglobulin Test (DAT) is positive for **C3d** (complement) but negative for IgG [1].

Question 1

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 2

What is the most common presentation of sickle cell anemia?

Accepted Answer

Fever

Answer

Priapism

Answer

Bone pain

Answer

Splenomegaly

Question 3

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 4

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 5

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 6

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 7

Which of the following is FALSE regarding sickle cell anemia?

Accepted Answer

High reticulocyte count

Answer

Dactylitis

Answer

Osteomyelitis

Answer

Granulocytosis

Question 8

Stage of Hodgkin's lymphoma with right-sided neck nodes and a left inguinal node, without fever?

Accepted Answer

Stage IIIa

Answer

Stage Ia

Answer

Stage IIa

Answer

Stage IVa

Question 9

Which of the following is NOT a feature of hereditary spherocytosis?

Accepted Answer

Increased MCV

Answer

Increased osmotic fragility

Answer

Increased MCHC

Answer

Decreased surface area to volume ratio

Question 10

Cold agglutinin disease can be associated with all of the following, except:

Accepted Answer

Systemic Lupus Erythematosus

Answer

Mycoplasma pneumoniae infection

Answer

Waldenstrom macroglobulinemia

Answer

Epstein-Barr virus infection

Hematology — MCQs

Hematology — MCQs

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