Hematology Practice Questions

Q: What is the most common cause for complications of blood transfusion?

Human error. Explanation: 1. Why Human Error is the Correct Answer: Despite advancements in screening and cross-matching technology, human error remains the leading cause of transfusion-related complications and fatalities. This most commonly manifests as ABO incompatibility [1] due to clerical mistakes, such as mislabeling a sample, improper patient identification at the bedside, or administering the wrong unit to the wrong patient. Studies consistently show that technical failures are rare compared to procedural lapses in the "chain of transfusion." [1] 2. Why the Other Options are Incorrect: * Anaphylaxis: While serious, IgA-deficient patients experiencing anaphylaxis represent a very small percentage of transfusion reactions. * Graft-versus-host disease (GVHD): This is a rare, albeit fatal, complication occurring primarily in immunocompromised patients or when receiving blood from HLA-homozygous donors (e.g., directed donations from relatives). It is prevented by irradiation, not a common occurrence. * Presensitization: This leads to delayed hemolytic transfusion reactions or febrile non-hemolytic reactions due to prior exposure (pregnancy/transfusion). While frequent, these are statistically less common as a primary cause of major morbidity compared to errors in the administration process. 3. High-Yield Clinical Pearls for NEET-PG: * Most common overall reaction: Febrile Non-Hemolytic Transfusion Reaction (FNHTR) [1]. * Most common cause of transfusion-related death: Historically ABO incompatibility (human error), but in modern well-monitored settings, TRALI (Transfusion-Related Acute Lung Injury) is often cited as the leading cause of mortality. * Bedside Check: The final "clerical check" at the patient's bedside is the most critical step in preventing fatal hemolytic reactions [2]. * Bacterial Contamination: Most common with Platelets (stored at room temperature). [1] Davidson's Principles and Practice of Medicine, 22E, pp. 1033-1037.

Q: A 25-year-old patient presents with fatigue and abdominal pain. Examination reveals jaundice and splenomegaly. Ultrasound shows gallstones. What is the most likely diagnosis?

Hereditary spherocytosis. ### Explanation **Correct Answer: C. Hereditary spherocytosis** The clinical triad of **anemia (fatigue), jaundice, and splenomegaly** in a young patient, coupled with the presence of **gallstones**, is a classic presentation of chronic extravascular hemolysis. In **Hereditary Spherocytosis (HS)**, a defect in red blood cell (RBC) membrane proteins (most commonly **Ankyrin**, followed by Spectrin) leads to the formation of spherical, fragile RBCs. These spherocytes are trapped and destroyed in the splenic sinusoids, leading to splenomegaly. The chronic breakdown of RBCs results in unconjugated hyperbilirubinemia, which predisposes the patient to **pigment (calcium bilirubinate) gallstones**. **Why other options are incorrect:** * **A. Sickle cell anemia:** While it causes hemolysis and gallstones, the spleen in an adult sickle cell patient is usually small and fibrotic due to repeated infarctions (**autosplenectomy**), making splenomegaly unlikely at age 25. * **B. Acute pancreatitis:** Presents with severe epigastric pain radiating to the back and elevated amylase/lipase; it does not typically cause chronic jaundice or splenomegaly. * **D. Cholangitis:** Presents with Charcot’s Triad (fever, jaundice, RUQ pain). It is an acute bacterial infection of the biliary tree, not a chronic hemolytic process. **High-Yield NEET-PG Pearls:** * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test via flow cytometry. * **Screening Test:** Osmotic Fragility Test (increased fragility). * **Peripheral Smear:** Spherocytes (small, dark RBCs lacking central pallor) and reticulocytosis. * **MCHC:** Characteristically **increased** (the only anemia with high MCHC). * **Treatment of Choice:** Splenectomy (indicated in moderate to severe cases, usually after age 5 to reduce sepsis risk).

Q: Which of the following is a poor prognostic factor in Acute Lymphoblastic Leukemia (ALL)?

t (9;22); t (4; 11). In Acute Lymphoblastic Leukemia (ALL), prognosis is determined by age, initial white blood cell (WBC) count, and specific cytogenetic abnormalities [1]. ### **Explanation of the Correct Answer** **Option B: t(9;22) and t(4;11)** are well-established **poor prognostic factors** [1]. * **t(9;22):** Known as the Philadelphia chromosome ($Ph+$), it creates the *BCR-ABL1* fusion gene [1]. It is more common in adults and is associated with high resistance to standard chemotherapy and a high relapse rate. * **t(4;11):** Involves the *KMT2A* (MLL) gene rearrangement. It is typically seen in infant ALL and is associated with very high WBC counts and CNS involvement, signifying an aggressive disease course [1]. ### **Analysis of Incorrect Options** * **A. Hyperdiploidy:** Defined as >50 chromosomes per cell, this is a **favorable** prognostic factor. These cells are highly sensitive to methotrexate and apoptosis. * **C. 2–8 years of age:** This is the "golden age" for ALL prognosis. Patients between **1 and 10 years** have the best outcomes. Age 10 years (adolescents/adults) indicates a poorer prognosis. * **D. WBC count 50,000 in B-ALL (or >100,000 in T-ALL) is considered a high-risk feature [1]. ### **High-Yield Clinical Pearls for NEET-PG** * **Best Prognosis Cytogenetics:** t(12;21) involving *ETV6-RUNX1* (most common in children) and Hyperdiploidy. * **Worst Prognosis Cytogenetics:** t(9;22), t(4;11), and Hypodiploidy (<44 chromosomes) [1]. * **Immunophenotype:** Early pre-B cell (CD10/CALLA positive) has a better prognosis than mature B-cell or T-cell ALL. * **Minimal Residual Disease (MRD):** The most important predictor of relapse after starting treatment.

Q: A 25-year-old pregnant lady presents with leukocytosis, a platelet count less than 50,000, and fragmented red blood cells on peripheral smear. Which of the following is the least likely differential diagnosis?

Evans' syndrome. The clinical presentation describes a **Microangiopathic Hemolytic Anemia (MAHA)**, characterized by thrombocytopenia and fragmented RBCs (schistocytes) on a peripheral smear [1]. **1. Why Evans' Syndrome is the least likely (Correct Answer):** Evans' syndrome is defined as the simultaneous or sequential occurrence of **Immune Thrombocytopenic Purpura (ITP)** and **Autoimmune Hemolytic Anemia (AIHA)**. Crucially, AIHA is a warm-antibody mediated process where RBCs are destroyed by splenic macrophages, leading to **spherocytes**, not schistocytes. The absence of mechanical fragmentation (schistocytes) makes Evans' syndrome inconsistent with the peripheral smear findings provided. **2. Analysis of Incorrect Options:** * **HELLP Syndrome:** A pregnancy-specific complication (Hemolysis, Elevated Liver enzymes, Low Platelets) that is a classic cause of MAHA and schistocytes in the third trimester [1]. * **TTP:** Characterized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, fever, and renal failure. It is a major differential for MAHA in pregnancy [1]. * **DIC:** Often triggered by obstetric complications (e.g., abruptio placentae) [2]. It involves systemic activation of coagulation, leading to consumption of platelets and clotting factors, resulting in schistocytes on the smear [2]. **Clinical Pearls for NEET-PG:** * **Schistocytes = MAHA:** Always think of TTP, HUS, DIC, or HELLP [1]. * **Spherocytes = AIHA or Hereditary Spherocytosis:** Think of Evans' syndrome if associated with low platelets. * **TTP vs. DIC:** TTP usually has normal coagulation profiles (PT/aPTT), whereas DIC has prolonged PT/aPTT and low fibrinogen [2]. * **HELLP vs. TTP:** HELLP is typically associated with hypertension and significant transaminitis.

Q: Which of the following is the most serious late side effect of treatment of Hodgkin's disease?

Cardiac injury. The treatment of Hodgkin’s Lymphoma (HL) has a high cure rate, making the management of long-term survivorship and **late toxicities** a critical clinical focus. [1] **Cardiac injury** is considered the most serious late side effect because it is a leading cause of non-malignant morbidity and mortality in HL survivors. It typically manifests 10–20 years after treatment. The injury is multifactorial: 1. **Radiation Therapy (Mediastinal):** Causes accelerated coronary artery disease (CAD), valvular dysfunction, and pericarditis. [1] 2. **Chemotherapy (Anthracyclines like Doxorubicin):** Leads to dose-dependent, irreversible dilated cardiomyopathy and congestive heart failure. **Analysis of Incorrect Options:** * **B. Infertility:** While common (especially with alkylating agents like Procarbazine in the older MOPP regimen), it is not considered as "serious" or life-threatening as cardiac failure or secondary malignancies. Modern ABVD regimens have a much lower risk of permanent infertility. * **C. Hair Loss (Alopecia):** This is an **acute** side effect that occurs during treatment and is reversible. It is not a "late" side effect. * **D. Osteoporosis:** Can occur due to prolonged steroid use (Prednisone), but it is rarely the most life-limiting complication compared to cardiotoxicity. **NEET-PG High-Yield Pearls:** * The **most common** late cause of death in HL survivors is **Secondary Malignancies** (most commonly Breast Cancer and Lung Cancer). * If "Secondary Malignancy" is not an option, **Cardiac Injury** is the next most significant life-threatening late complication. * **Lhermitte’s sign** (electric shock sensation down the spine) is a transient late effect of neck radiation in HL patients. * Survivors require lifelong screening, including annual mammograms (starting 8-10 years post-radiation) and aggressive lipid management.

Q: To prevent excessive bleeding during surgery, a patient with hemophilia A may be given which of the following?

Factor VIII concentrate. **Explanation:** **Hemophilia A** is an X-linked recessive bleeding disorder caused by a deficiency of **Clotting Factor VIII** [1]. To achieve surgical hemostasis, the goal is to raise Factor VIII levels to 80–100% of normal. **Why Factor VIII concentrate is correct:** Factor VIII concentrate (either plasma-derived or recombinant) is the **treatment of choice** [1]. It provides a highly concentrated dose of the specific missing factor without the risk of volume overload. Recombinant products are preferred in modern practice to eliminate the risk of blood-borne viral transmission (HIV, Hepatitis B/C) [2]. **Why the other options are incorrect:** * **Whole blood:** Contains very low concentrations of clotting factors relative to volume. It is used for massive hemorrhage with shock, not targeted factor replacement. * **Fresh frozen plasma (FFP):** While FFP contains all coagulation factors, the concentration of Factor VIII is low. Achieving therapeutic levels for surgery would require massive volumes, leading to **Transfusion Associated Circulatory Overload (TACO)**. * **Factor IX concentrate:** This is the specific treatment for **Hemophilia B** (Christmas Disease). It has no role in treating Hemophilia A. **High-Yield Clinical Pearls for NEET-PG:** * **Cryoprecipitate:** Contains Factor VIII, Fibrinogen, von Willebrand Factor (vWF), and Factor XIII. It was historically used for Hemophilia A but is now a second-line treatment if concentrates are unavailable. * **Desmopressin (DDAVP):** Useful in **mild** Hemophilia A; it releases endogenous Factor VIII and vWF from endothelial Weibel-Palade bodies [2]. * **Mixing Study:** In Hemophilia, the prolonged aPTT **corrects** upon mixing with normal plasma (distinguishing it from Factor VIII inhibitors/antibodies). * **Emicizumab:** A newer bispecific monoclonal antibody that mimics Factor VIII function by bridging Factors IXa and X.

Q: Aplastic anemia can progress to all of the following except?

Pure red cell aplasia. Aplastic Anemia (AA) is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular marrow. It is considered a "clonal" disorder of hematopoietic stem cells, which predisposes patients to late clonal evolution into other myeloid malignancies. **Why Pure Red Cell Aplasia (PRCA) is the correct answer:** PRCA is a condition characterized by a selective failure of erythropoiesis (only red cell precursors are absent), while white cells and platelets remain normal. It is **not** a complication or a progressive stage of Aplastic Anemia. In fact, the relationship is often the reverse: PRCA is a lineage-specific failure, whereas AA is a global stem cell failure. Progression from a global failure (AA) to a single-lineage failure (PRCA) does not occur. **Analysis of Incorrect Options:** * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** There is a strong clinical link between AA and PNH. Up to 40-50% of AA patients may have a small PNH clone (PIGA mutation) at diagnosis, and many go on to develop clinical PNH. * **Myelodysplastic Syndrome (MDS) & Acute Myeloid Leukemia (AML):** These represent the "clonal evolution" of AA [1]. Approximately 10-15% of patients with AA (especially those treated with immunosuppressive therapy) may eventually develop MDS or AML, often associated with chromosomal abnormalities like Monosomy 7. **Clinical Pearls for NEET-PG:** * **Most common chromosomal abnormality** in clonal evolution of AA: **Monosomy 7**. * **Gold Standard Treatment:** Bone Marrow Transplant (for young patients with a matched sibling donor) or Immunosuppressive Therapy (ATG + Cyclosporine + Eltrombopag). * **PNH Screening:** All patients with Aplastic Anemia must be screened for PNH clones using **Flow Cytometry** (CD55/CD59 deficiency).

Q: What is the standard treatment for Central Nervous System (CNS) leukemia?

Intrathecal methotrexate. **Explanation:** The Blood-Brain Barrier (BBB) prevents most systemic chemotherapeutic agents from reaching therapeutic concentrations within the cerebrospinal fluid (CSF) [3]. Therefore, standard intravenous therapy is often ineffective for treating or preventing CNS involvement in leukemia [1]. **1. Why Intrathecal Methotrexate is Correct:** **Intrathecal (IT) Methotrexate** is the gold standard because it bypasses the BBB, delivering high concentrations of the antimetabolite directly into the subarachnoid space. It is used both as **prophylaxis** (to prevent CNS relapse in ALL) and as **treatment** for active CNS leukemia [1]. It is often used alone or as part of "triple intrathecal therapy" (Methotrexate + Cytarabine + Hydrocortisone). **2. Why the other options are incorrect:** * **Vincristine and Prednisolone (Option B):** While these are backbone drugs for induction therapy in Acute Lymphoblastic Leukemia (ALL) [1], they are administered systemically and do not achieve sufficient CNS penetration to treat active CNS disease. * **Intrathecal Vincristine (Option C):** **CRITICAL CONTRAINDICATION.** Vincristine is highly neurotoxic if given intrathecally. Accidental IT administration leads to progressive ascending paralysis and is almost **universally fatal**. It must only be given intravenously. * **Intravenous Prednisolone (Option D):** While glucocorticoids (like Dexamethasone) have some CNS penetration [2], they are insufficient as monotherapy for CNS leukemia. **Clinical Pearls for NEET-PG:** * **Prophylaxis:** CNS prophylaxis is mandatory in all patients with ALL because the CNS acts as a "pharmacological sanctuary." * **Triple IT Therapy:** Consists of Methotrexate, Cytarabine (Ara-C), and Hydrocortisone. * **Fatal Error:** Always remember: *"Vincristine is for the Vein only."* * **Alternative:** Cranial irradiation is an alternative but is less preferred in children due to long-term neurocognitive sequelae.

Question 1

What is the most common cause for complications of blood transfusion?

Accepted Answer

Human error

Answer

Anaphylaxis

Answer

Graft-versus-host disease (GVHD)

Answer

Presensitization

Question 2

A 67-year-old elderly male presents with headache, recurrent infections, and multiple punched-out lytic lesions on X-ray skull and lumbago for the past 1 month. Which investigation will be most helpful in establishing a diagnosis?

Accepted Answer

Protein electrophoresis

Answer

Serum calcium

Answer

Alkaline phosphatase levels

Answer

PSA levels

Question 3

A 25-year-old patient presents with fatigue and abdominal pain. Examination reveals jaundice and splenomegaly. Ultrasound shows gallstones. What is the most likely diagnosis?

Accepted Answer

Hereditary spherocytosis

Answer

Sickle cell anemia

Answer

Acute pancreatitis

Answer

Cholangitis

Question 4

Which of the following is a poor prognostic factor in Acute Lymphoblastic Leukemia (ALL)?

Accepted Answer

t (9;22); t (4; 11)

Answer

Hyperdiploidy

Answer

2-8 years of age

Answer

WBC count < 50,000

Question 5

A 25-year-old female presented with mild pallor and moderate hepatosplenomegaly. Her hemoglobin was 92 g/L and fetal hemoglobin level was 65%. She has not received any blood transfusions to date. She is most likely to be suffering from:

Accepted Answer

Thalassemia intermedia

Answer

Thalassemia major

Answer

Hereditary persistent fetal hemoglobin, homozygous state

Answer

Hemoglobin D, homozygous state

Question 6

A 25-year-old pregnant lady presents with leukocytosis, a platelet count less than 50,000, and fragmented red blood cells on peripheral smear. Which of the following is the least likely differential diagnosis?

Accepted Answer

Evans' syndrome

Answer

Disseminated Intravascular Coagulation (DIC)

Answer

Thrombotic Thrombocytopenic Purpura (TTP)

Answer

HELLP syndrome

Question 7

Which of the following is the most serious late side effect of treatment of Hodgkin's disease?

Accepted Answer

Cardiac injury

Answer

Infertility

Answer

Hair loss

Answer

Osteoporosis

Question 8

To prevent excessive bleeding during surgery, a patient with hemophilia A may be given which of the following?

Accepted Answer

Factor VIII concentrate

Answer

Whole blood

Answer

Fresh frozen plasma

Answer

Factor IX concentrate

Question 9

Aplastic anemia can progress to all of the following except?

Accepted Answer

Pure red cell aplasia

Answer

Acute myeloid leukemia (AML)

Answer

Myelodysplastic syndrome

Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Question 10

What is the standard treatment for Central Nervous System (CNS) leukemia?

Accepted Answer

Intrathecal methotrexate

Answer

Vincristine and prednisolone

Answer

Intrathecal vincristine

Answer

Intravenous prednisolone

Hematology — MCQs

Hematology — MCQs

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