Hematology Practice Questions

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].

Q: Central nervous system involvement in diffuse large B-cell lymphoma (DLBCL) is not typically seen with involvement of which of the following organs?

Ovary. In Diffuse Large B-cell Lymphoma (DLBCL), certain extranodal sites are associated with a significantly higher risk of secondary Central Nervous System (CNS) relapse. This is often due to the biological behavior of the tumor or the presence of "sanctuary sites" where systemic chemotherapy penetration is poor. [1] **Explanation of the Correct Option:** * **A. Ovary:** While DLBCL can involve the ovaries, it is **not** classically associated with an increased risk of CNS dissemination. In contrast to the male gonads, ovarian involvement does not mandate routine CNS prophylaxis in the same high-risk category as the other options listed. **Explanation of Incorrect Options:** * **B. Testis:** The testis is a classic "sanctuary site" due to the blood-testis barrier. Primary or secondary testicular DLBCL has a very high propensity (up to 15-30%) for CNS relapse. [1] * **C & D. Kidney and Adrenal Gland:** Involvement of the retroperitoneal organs, specifically the kidneys and adrenal glands, is a well-recognized independent risk factor for CNS involvement. [3] The **CNS-IPI (International Prognostic Index)** specifically includes "kidney or adrenal involvement" as a high-risk criterion. **High-Yield Clinical Pearls for NEET-PG:** * **CNS-IPI Score:** Used to predict CNS relapse. It includes the 5 standard IPI factors (Age >60, LDH >normal, Performance Status ≥2, Stage III/IV, >1 Extranodal site) **PLUS** kidney/adrenal involvement. [3] * **High-Risk Sites:** The "Rule of T's and R's" for CNS risk: **T**estis, **R**etroperitoneum (Kidney/Adrenal), and sometimes **B**one Marrow or **B**reast. * **Prophylaxis:** Patients with involvement of the testis, kidney, or adrenal glands often require CNS-directed therapy (e.g., Intrathecal Methotrexate or high-dose systemic Methotrexate) alongside R-CHOP. [2]

Q: In a patient with mild hemophilia, what would be the typical percentage of clotting factor (antihemophilic factor) activity?

Above 5%. Hemophilia (A or B) is an X-linked recessive bleeding disorder caused by a deficiency in Factor VIII or Factor IX [1]. The clinical severity of the disease is directly correlated with the plasma level of the deficient clotting factor. **1. Why Option A is Correct:** According to the standard classification of hemophilia severity: * **Severe:** 5% to <40%** of normal factor activity. (Bleeding usually occurs only after major trauma or surgery). Since mild hemophilia is defined as activity **above 5%**, Option A is the most accurate threshold for diagnosis. **2. Why Other Options are Incorrect:** * **Option B (Above 10%):** While a patient with 10% activity is indeed "mild," this option is too restrictive. A patient with 6% or 8% activity is also classified as mild, and this option would exclude them. * **Option C (Above 30%):** Normal factor levels range from 50% to 150%. The "mild" category ends at approximately 40%. Setting the threshold at 30% misses a significant portion of the mild hemophilia population (those between 5% and 30%). * **Option D:** Incorrect because the specific diagnostic cutoff for mild hemophilia starts at >5%. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause of death:** Intracranial hemorrhage. * **Most common site of bleeding:** Knee joint (Hemarthrosis) [1]. * **Lab Findings:** Prolonged **aPTT**, normal PT, normal Bleeding Time, and normal Platelet count. * **Treatment:** Factor replacement is the mainstay [1]. In **Mild Hemophilia A**, **Desmopressin (DDAVP)** can be used to transiently raise Factor VIII levels by releasing it from endothelial stores [2].

Q: A 67-year-old woman with a prosthetic aortic valve develops progressive anemia. Examination of a peripheral blood smear reveals reticulocytosis and schistocytes. What is the appropriate diagnosis?

Macroangiopathic hemolytic anemia. ### Explanation **Correct Option: D. Macroangiopathic hemolytic anemia (MAHA)** The clinical presentation of a **prosthetic heart valve** combined with **schistocytes** (fragmented RBCs) and **reticulocytosis** (indicating a compensatory bone marrow response to hemolysis) is a classic description of **Waring Blender Syndrome**. **Medical Concept:** This is a form of **Macroangiopathic Hemolytic Anemia**. It occurs due to mechanical trauma as RBCs pass through the turbulent flow or high-pressure gradients created by a prosthetic valve (especially mechanical valves or paravalvular leaks) [1]. The physical impact shears the RBC membranes, leading to intravascular hemolysis and the formation of schistocytes [1]. **Why Incorrect Options are Wrong:** * **A. Acanthocytosis:** Characterized by "spur cells" (irregularly spiked RBCs), typically seen in **Abetalipoproteinemia** or severe liver disease, not mechanical trauma. * **B. Henoch-Schönlein purpura (HSP):** A small-vessel vasculitis presenting with a triad of palpable purpura, arthralgia, and abdominal pain. It does not typically cause mechanical hemolysis. * **C. Idiopathic thrombocytopenic purpura (ITP):** An immune-mediated destruction of platelets. While it causes bleeding, it does not cause RBC fragmentation or schistocytes. **NEET-PG High-Yield Pearls:** * **Schistocytes** are the hallmark of **Micro**angiopathic Hemolytic Anemia (TTP, HUS, DIC) and **Macro**angiopathic Hemolytic Anemia (Prosthetic valves, Aortic stenosis). * In MAHA due to valves, **haptoglobin** will be decreased, and **LDH** will be elevated. * **Iron deficiency anemia** can eventually develop in these patients due to chronic **hemosiderinuria** (loss of iron in urine following intravascular hemolysis).

Q: Which of the following infections is most commonly related to aplastic anemia?

Hepatitis. **Explanation:** **Aplastic Anemia (AA)** is a clinical syndrome characterized by pancytopenia resulting from bone marrow hypoplasia. While most cases are idiopathic, viral infections are a well-recognized trigger. **Why Hepatitis is the Correct Answer:** **Post-hepatitic aplastic anemia** is a distinct clinical entity. It typically occurs 2–3 months after an episode of acute hepatitis. Interestingly, in the majority of these cases, the common hepatitis viruses (A, B, C, or E) are **not** the culprits; it is usually caused by unidentified non-A, non-B, non-C, non-E viruses. The pathophysiology involves a T-cell-mediated immune destruction of hematopoietic stem cells triggered by the viral infection. It is often severe and carries a high mortality rate if not treated with bone marrow transplantation or immunosuppressive therapy. **Why Other Options are Incorrect:** * **H. pylori:** This is primarily associated with peptic ulcer disease, gastric MALT lymphoma, and **Immune Thrombocytopenic Purpura (ITP)** or Iron Deficiency Anemia, but not aplastic anemia. * **Tetanus:** Caused by *Clostridium tetani* neurotoxins; it affects the neuromuscular system and has no association with bone marrow failure. * **Bacteremia:** While severe sepsis can cause transient cytopenias or DIC, it does not cause the chronic stem cell failure characteristic of aplastic anemia. **High-Yield Clinical Pearls for NEET-PG:** * **Most common virus** associated with AA: Non-A, non-B, non-C, non-G hepatitis viruses. * **Other associated viruses:** EBV (Infectious Mononucleosis), CMV, HIV, and Parvovirus B19 (though B19 typically causes **Pure Red Cell Aplasia**, especially in sickle cell patients). * **Drug of choice:** For patients not eligible for transplant, **Antithymocyte Globulin (ATG) + Cyclosporine** is the standard treatment. * **Gold Standard Diagnosis:** Bone marrow biopsy showing hypocellularity and increased fat content ("dry tap").

Question 1

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 2

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 3

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

Question 4

Central nervous system involvement in diffuse large B-cell lymphoma (DLBCL) is not typically seen with involvement of which of the following organs?

Accepted Answer

Ovary

Answer

Testis

Answer

Kidney

Answer

Adrenal gland

Question 5

A 40-year-old male has Hb-10 gm%, MCV-65 fL, RDW-16%, platelet count 4.5 lac/pt. What additional finding would be expected?

Accepted Answer

Total iron-binding capacity

Answer

Low serum transferrin

Answer

Total serum copper

Answer

Serum ferritin

Question 6

In a patient with mild hemophilia, what would be the typical percentage of clotting factor (antihemophilic factor) activity?

Accepted Answer

Above 5%

Answer

Above 10%

Answer

Above 30%

Answer

Any of the above

Question 7

A 67-year-old woman with a prosthetic aortic valve develops progressive anemia. Examination of a peripheral blood smear reveals reticulocytosis and schistocytes. What is the appropriate diagnosis?

Accepted Answer

Macroangiopathic hemolytic anemia

Answer

Acanthocytosis

Answer

Henoch-Schönlein purpura

Answer

Idiopathic thrombocytopenic purpura

Question 8

Which of the following infections is most commonly related to aplastic anemia?

Accepted Answer

Hepatitis

Answer

H. pylori infection

Answer

Tetanus

Answer

Bacteremia

Question 9

Which of the following statements is not true regarding hemophilia A?

Accepted Answer

Factor VIII levels less than 50% are associated with spontaneous hemorrhage.

Answer

Hemophilia A is due to deficiency of factor VIII.

Answer

In hemophilia A, females are typically carriers.

Answer

Desmopressin can be useful in the management of hemophilia A.

Question 10

A 19-year-old college student develops a severe sore throat, cervical lymphadenopathy, and atypical lymphocytes on blood film. A heterophil antibody test is positive. For the above patient with a hematologic abnormality, select the most likely diagnosis.

Accepted Answer

infectious mononucleosis

Answer

hyperthyroidism

Answer

rectal cancer

Answer

renal cell carcinoma

Hematology — MCQs

Hematology — MCQs

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