Hematology Practice Questions

Q: What is the standard regimen used in the treatment of non-Hodgkin's Lymphoma?

CHOP. **Explanation:** The standard first-line chemotherapy regimen for **Non-Hodgkin’s Lymphoma (NHL)**, particularly Diffuse Large B-Cell Lymphoma (DLBCL), is **CHOP**. In modern practice, this is often combined with Rituximab (R-CHOP). **Breakdown of CHOP:** * **C:** Cyclophosphamide (Alkylating agent) * **H:** Hydroxydaunorubicin/Doxorubicin (Antitumor antibiotic/Anthracycline) * **O:** Oncovin/Vincristine (Vinca alkaloid) * **P:** Prednisolone (Glucocorticoid) **Analysis of Incorrect Options:** * **MOPP (Mechlorethamine, Oncovin, Procarbazine, Prednisolone):** This was the historical gold standard for **Hodgkin’s Lymphoma (HL)** but has been largely replaced due to high toxicity (infertility and secondary leukemias). * **ABVD (Adriamycin, Bleomycin, Vinblastine, Dacarbazine):** This is the current **standard of care for Hodgkin’s Lymphoma**. It is preferred over MOPP because it is less gonadotoxic and less leukemogenic. * **COPP (Cyclophosphamide, Oncovin, Procarbazine, Prednisolone):** Used occasionally in HL as a substitute for MOPP, but not the standard for NHL. **High-Yield Clinical Pearls for NEET-PG:** 1. **Doxorubicin Toxicity:** Monitor for **dilated cardiomyopathy** (cumulative dose-dependent). Dexrazoxane can be used as a cardioprotectant. 2. **Vincristine Toxicity:** Look for **peripheral neuropathy** (foot drop/paresthesia) and paralytic ileus. It is "M-phase" specific and notably **bone marrow sparing**. 3. **Cyclophosphamide Toxicity:** Characterized by **hemorrhagic cystitis** due to the metabolite **Acrolein**. This is prevented by aggressive hydration and **MESNA**. 4. **Rituximab:** A monoclonal antibody against **CD20**, frequently added to CHOP for B-cell NHL.

Q: Decreased RBC production is seen in which of the following conditions?

Renal failure. The correct answer is **Renal failure**. The primary mechanism behind decreased RBC production in chronic kidney disease (CKD) is the deficiency of **Erythropoietin (EPO)** [1]. EPO is a glycoprotein hormone produced by the peritubular interstitial cells of the renal cortex in response to hypoxia [1]. In renal failure, the loss of functional renal parenchyma leads to inadequate EPO production, resulting in a normocytic, normochromic anemia [1]. **Analysis of Options:** * **A. Intramuscular folate administration:** This would actually **increase** RBC production. Folate is essential for DNA synthesis; administering it to a deficient patient corrects megaloblastic maturation and stimulates erythropoiesis. * **C. Post gastrectomy:** This typically leads to anemia due to **increased loss or malabsorption** (Vitamin B12 deficiency due to loss of intrinsic factor and Iron deficiency due to loss of gastric acid) [2]. While it affects production eventually, it is classified primarily as a nutritional deficiency/malabsorption state rather than a primary failure of the bone marrow's production signal. * **D. Prosthetic valve hemolysis:** This is a classic example of **increased RBC destruction** (extravascular/mechanical hemolysis). The bone marrow is actually hyperactive in this condition, trying to compensate by increasing RBC production (reticulocytosis). **NEET-PG High-Yield Pearls:** * **Anemia of CKD:** Usually develops when GFR falls below **30-45 mL/min**. * **Target Hemoglobin:** When treating CKD patients with recombinant EPO, the target Hb is generally **10–11.5 g/dL**. Exceeding 13 g/dL increases the risk of cardiovascular events and thrombosis [1]. * **Echistocytes:** Always look for fragmented RBCs (schistocytes) on a peripheral smear in cases of prosthetic valve hemolysis (Microangiopathic Hemolytic Anemia).

Q: What is the leading cause of death in Chronic Lymphocytic Leukemia (CLL)?

Infections. **Explanation:** **1. Why Infections are the Leading Cause of Death:** In Chronic Lymphocytic Leukemia (CLL), the primary cause of mortality (responsible for 50-60% of deaths) is **infection** [1]. This susceptibility arises from a profound state of immune dysregulation characterized by: * **Hypogammaglobulinemia:** Progressive decline in serum immunoglobulin levels (IgG, IgA, and IgM) due to the suppression of normal B-cell function [1]. * **T-cell Dysfunction:** Impaired cell-mediated immunity, increasing the risk of viral (e.g., Herpes Zoster) and opportunistic infections. * **Neutropenia:** Often a result of advanced bone marrow infiltration or chemotherapy (e.g., Fludarabine). Common sites include the respiratory tract (pneumonia) and skin. **2. Why Other Options are Incorrect:** * **B. Bleeding:** While thrombocytopenia can occur due to marrow replacement or ITP (Evans Syndrome), it is a less frequent cause of death compared to sepsis [1]. * **C. Meningeal extension:** Central Nervous System (CNS) involvement is extremely rare in CLL. This is more characteristic of Acute Lymphoblastic Leukemia (ALL) or high-grade lymphomas. * **D. DIC:** Disseminated Intravascular Coagulation is a classic complication of **Acute Promyelocytic Leukemia (APL - M3)**, not CLL. **3. High-Yield Clinical Pearls for NEET-PG:** * **Richter’s Transformation:** In 3-10% of cases, CLL transforms into an aggressive **Diffuse Large B-Cell Lymphoma (DLBCL)**, marked by sudden clinical deterioration and rising LDH. * **Autoimmune Complications:** CLL is associated with **Autoimmune Hemolytic Anemia (AIHA)** and ITP [1]. * **Smudge Cells:** A classic peripheral smear finding (crushed lymphocytes). * **Staging:** Remember the **Rai** (USA) and **Binet** (Europe) staging systems for exam questions.

Q: A 17-year-old girl with mild von Willebrand disease is scheduled for dental extraction. She has a previous history of bleeding during surgery. What medication can be given prophylactically to prevent bleeding?

Desmopressin. ### Explanation **Correct Answer: C. Desmopressin (DDAVP)** **Mechanism and Rationale:** Desmopressin (DDAVP) is the treatment of choice for patients with **Type 1 von Willebrand Disease (vWD)**, which accounts for approximately 80% of cases. It is a synthetic analogue of vasopressin that stimulates the release of endogenous **vWF and Factor VIII** from the Weibel-Palade bodies of endothelial cells. In minor surgical procedures like dental extractions, a single dose of DDAVP (intravenous or intranasal) can transiently increase vWF levels 3 to 5-fold, providing adequate hemostasis without the need for blood products. **Analysis of Incorrect Options:** * **A. Cryoprecipitate:** While it contains vWF and Factor VIII, it is no longer recommended due to the risk of transfusion-transmitted infections (TTIs). It is reserved only for life-threatening emergencies where specific concentrates are unavailable. * **B. Fresh Frozen Plasma (FFP):** FFP contains low concentrations of vWF and requires large volumes to achieve therapeutic levels, risking fluid overload. It is not a first-line treatment for vWD. * **D. High-purity Factor VIII concentrates:** These are used in Hemophilia A but are **ineffective** in vWD because the purification process removes vWF. If factor replacement is needed (e.g., in Type 3 vWD or major surgery), **Intermediate-purity Factor VIII concentrates** (which contain vWF) or recombinant vWF should be used. **NEET-PG High-Yield Pearls:** * **vWD Type 1:** Quantitative deficiency (Most common). * **vWD Type 2:** Qualitative defect. * **vWD Type 3:** Severe, total deficiency (Autosomal Recessive). * **DDAVP Side Effect:** Hyponatremia (due to water retention); patients should be advised to restrict fluid intake for 24 hours post-administration. * **Diagnostic Clue:** Prolonged Bleeding Time (BT) and potentially prolonged aPTT, with a normal Platelet Count.

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).

Question 1

Which of the following statements regarding hematological malignancies is true?

Accepted Answer

Acute lymphoid leukemia in infants less than 1 year of age has a poor prognosis.

Answer

Chronic myeloid leukemia characteristically occurs in individuals over 50 years of age.

Answer

Hairy cell leukemia in individuals less than 50 years of age generally has a good prognosis.

Answer

Chronic lymphocytic leukemia characteristically occurs in individuals less than 50 years of age.

Question 2

What is the standard regimen used in the treatment of non-Hodgkin's Lymphoma?

Accepted Answer

CHOP

Answer

COPP

Answer

MOPP

Answer

ABVD

Question 3

Decreased RBC production is seen in which of the following conditions?

Accepted Answer

Renal failure

Answer

Intramuscular folate administration

Answer

Post gastrectomy

Answer

Prosthetic valve hemolysis

Question 4

What is the leading cause of death in Chronic Lymphocytic Leukemia (CLL)?

Accepted Answer

Infections

Answer

Bleeding

Answer

Meningeal and ventricular extension

Answer

Disseminated Intravascular Coagulation (DIC)

Question 5

A seven-year-old girl presents with repeated episodes of bleeding into joints. On investigation, her APTT was found to be prolonged and PT was normal. Which of the following is the most likely diagnosis?

Accepted Answer

Von Willebrand Disease

Answer

Factor VII deficiency

Answer

Factor VIII deficiency

Answer

Factor XII deficiency

Question 6

What concentration of methemoglobin appears as cyanosis?

Accepted Answer

1.5 gm/dL

Answer

5 gm/dL

Answer

2 gm/dL

Answer

12 gm/dL

Question 7

A 17-year-old girl with mild von Willebrand disease is scheduled for dental extraction. She has a previous history of bleeding during surgery. What medication can be given prophylactically to prevent bleeding?

Accepted Answer

Desmopressin

Answer

Cryoprecipitate

Answer

Fresh Frozen Plasma (FFP)

Answer

High-purity Factor VIII concentrates

Question 8

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

Accepted Answer

Malaria

Answer

G6PD deficiency

Answer

Thalassemia

Answer

Dengue fever

Question 9

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 10

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

Hematology — MCQs

Hematology — MCQs

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