Hematology Practice Questions

Q: Which of the following is the best candidate for allogeneic bone marrow transplant?

Hereditary immunodeficiency. Explanation: **Hereditary Immunodeficiency (Correct Answer):** Allogeneic Bone Marrow Transplant (BMT) or Hematopoietic Stem Cell Transplant (HSCT) is the **definitive curative treatment** for many primary immunodeficiency disorders (e.g., SCID, Wiskott-Aldrich syndrome) [3]. Since the defect lies in the hematopoietic stem cell line, replacing the patient’s defective immune system with healthy donor stem cells restores normal immune function [1]. **Analysis of Incorrect Options:** * **Thalassemia Minor:** Patients with thalassemia minor are usually asymptomatic or have mild anemia that does not require treatment. Allogeneic BMT is reserved for **Thalassemia Major**, where the burden of iron overload and transfusion dependency justifies the risks of transplant. * **Autoimmune Disease:** While HSCT is being researched for refractory cases (like Scleroderma or MS), it is not the "best" or standard candidate compared to life-threatening immunodeficiencies. Furthermore, **Autologous** transplant is more commonly used in autoimmune protocols to "reset" the immune system. * **Multiple Myeloma:** The standard of care for eligible myeloma patients is **Autologous BMT** (using the patient's own cells) to allow for high-dose chemotherapy. Allogeneic BMT is rarely used due to high treatment-related mortality and is not the primary choice. **NEET-PG High-Yield Pearls:** * **Best Timing for Thalassemia Major BMT:** Ideally performed before the onset of iron overload and hepatomegaly (Lucarelli Class I). * **Graft vs. Host Disease (GVHD):** A major complication of Allogeneic BMT, but the "Graft vs. Leukemia" effect is beneficial in malignancies [2]. * **SCID:** Considered a pediatric emergency; BMT should be performed as early as possible (ideally before 3.5 months of age) for the best prognosis [3].

Q: Which of the following clinical findings favors Thrombotic thrombocytopenic purpura in pregnancy?

Severe hemolysis. **Explanation:** Thrombotic Thrombocytopenic Purpura (TTP) is a medical emergency characterized by a deficiency of the ADAMTS13 enzyme, leading to microangiopathic hemolytic anemia (MAHA) and consumptive thrombocytopenia. In the context of pregnancy, differentiating TTP from other conditions like HELLP syndrome or Acute Fatty Liver of Pregnancy (AFLP) is critical. **1. Why "Severe Hemolysis" is correct:** TTP is defined by **intense microangiopathic hemolysis**. Clinical markers include a dramatic rise in LDH (often >1000 U/L), significant schistocytes on peripheral smear, and very low haptoglobin. While HELLP also features hemolysis, the degree of hemolysis in TTP is typically much more profound and occurs independently of significant hepatic dysfunction. **2. Why other options are incorrect:** * **Mild DIC:** TTP is characteristically a **non-consumptive coagulopathy**. PT, aPTT, and Fibrinogen levels are usually **normal** [1]. The presence of DIC strongly favors Abruptio Placentae or AFLP over TTP [1]. * **Marked elevation of transaminases:** This is a hallmark of **HELLP syndrome or AFLP**. In TTP, liver enzymes are usually normal or only mildly elevated due to passive congestion or mild ischemia. * **Moderate thrombocytopenia:** TTP typically presents with **severe thrombocytopenia** (often <20,000/µL). Moderate counts (50,000–100,000/µL) are more common in Gestational Thrombocytopenia or Preeclampsia. **Clinical Pearls for NEET-PG:** * **The Pentad of TTP:** Microangiopathic hemolytic anemia (MAHA), Thrombocytopenia, Fever, Renal failure, and Neurological symptoms (FAT RN). * **Key Differentiator:** Normal coagulation profile (PT/aPTT) in a patient with severe hemolysis and schistocytes points directly to TTP/HUS. * **Treatment:** Immediate **Plasmapheresis (Plasma exchange)** is the gold standard. Unlike HELLP, TTP does not resolve with delivery.

Q: Fresh Frozen Plasma (FFP) is stored at what temperature?

Below -25 degrees Celsius. ### Explanation **Correct Option: C (Below -25 degrees Celsius)** Fresh Frozen Plasma (FFP) is the liquid portion of whole blood that is separated and frozen within 8 hours of collection [1]. The primary goal of FFP storage is to preserve **labile coagulation factors**, specifically **Factor V and Factor VIII**. According to standard guidelines (including WHO and DGHS India), FFP must be stored at temperatures **below -25°C** to maintain a shelf life of up to **1 year**. If stored between -18°C and -25°C, the shelf life is reduced to 3 months [1]. **Analysis of Incorrect Options:** * **A & D (2-4°C and 2-10°C):** These are the storage temperature ranges for **Packed Red Blood Cells (PRBCs)** and Whole Blood [1]. At these temperatures, labile clotting factors in plasma degrade rapidly, rendering it ineffective for treating coagulopathies. * **B (0 to -25°C):** While FFP can be stored at -18°C, the standard "ideal" storage for maximum factor preservation and the specific threshold tested in competitive exams is "below -25°C." **High-Yield Clinical Pearls for NEET-PG:** * **Thawing:** FFP must be thawed at **30-37°C** in a water bath before use. Once thawed, it must be transfused within **24 hours** (stored at 2-6°C) to ensure factor activity. * **Indications:** Multiple coagulation factor deficiencies (e.g., Liver disease, DIC), Warfarin reversal, and TTP (Plasmapheresis). * **Dosage:** The standard dose is **10-15 mL/kg**. * **Cryoprecipitate:** Formed by thawing FFP at 4°C; it is rich in **Factor VIII, Fibrinogen, von Willebrand Factor, and Factor XIII**.

Q: All of the following are used in the treatment of Thrombotic Thrombocytopenic Purpura, EXCEPT?

Heparin. Thrombotic Thrombocytopenic Purpura (TTP) is a life-threatening hematologic emergency caused by a deficiency of the enzyme ADAMTS13. This deficiency leads to the accumulation of ultra-large von Willebrand factor (vWF) multimers, causing microvascular thrombosis, microangiopathic hemolytic anemia (MAHA), and thrombocytopenia. **Why Heparin is the Correct Answer (EXCEPT):** Despite the presence of extensive microthrombi, **Heparin is not used** in the treatment of TTP. Clinical trials have shown no benefit, and it significantly increases the risk of severe bleeding in patients who already have profound thrombocytopenia. Furthermore, the underlying pathology is vWF-mediated platelet aggregation, not a primary activation of the coagulation cascade (unlike DIC) [1]. **Analysis of Other Options:** * **Plasmapheresis (Plasma Exchange/PEX):** This is the **gold standard** and first-line treatment. It works by removing the ADAMTS13 autoantibodies and ultra-large vWF multimers while replenishing the deficient ADAMTS13 enzyme. * **Corticosteroids:** Used as adjunctive therapy to suppress the production of autoantibodies against ADAMTS13. * **Immunotherapy:** **Rituximab** (anti-CD20 monoclonal antibody) is now standard for refractory or high-risk cases to reduce the B-cell production of ADAMTS13 inhibitors. **Caplacizumab** (anti-vWF nanobody) is also a newer immunotherapy used to prevent platelet adhesion. **NEET-PG High-Yield Pearls:** * **Classic Pentad:** Fever, Anemia (MAHA), Thrombocytopenia, Neurological symptoms, and Renal failure (Mnemonic: **FAT RN**). * **Diagnosis:** Schistocytes on peripheral smear and ADAMTS13 activity **<10%**. * **Contraindication:** **Platelet transfusion** is generally contraindicated as it may "fuel the fire" by worsening microthrombosis. * **PT/aPTT:** Usually **normal** in TTP (helps differentiate it from DIC) [1].

Q: A 32-year-old man has mild anemia diagnosed on routine testing. His clinical examination is normal, and a blood film reveals some target cells with some red cells having intraerythrocytic crystals. For this patient with a hemoglobin abnormality, what is the most likely diagnosis?

HbC disease. The correct answer is **HbC disease**. This diagnosis is classically associated with the presence of **intraerythrocytic crystals** (often described as "bar-of-gold" or rhomboid-shaped crystals) and **target cells** (codocytes) on a peripheral blood smear. **Why HbC disease is correct:** HbC results from a point mutation in the $\beta$-globin gene where **glutamic acid is replaced by lysine** at the 6th position. This substitution reduces the solubility of the hemoglobin, causing it to precipitate and form characteristic hexagonal or rod-shaped crystals within the red blood cells, especially in dehydrated cells or after splenectomy. Patients typically present with mild hemolytic anemia and splenomegaly, though many are asymptomatic. **Why other options are incorrect:** * **$\beta$-thalassemia major:** Presents with severe microcytic hypochromic anemia, nucleated RBCs, and significant target cells, but does not feature intraerythrocytic crystals. * **HbH disease:** A form of $\alpha$-thalassemia characterized by "golf ball" inclusions (precipitated $\beta$-globin tetramers) visible with supravital stains like Brilliant Cresyl Blue, not rhomboid crystals. * **Sickle cell disease (HbS):** Characterized by sickle-shaped cells (drepanocytes) due to the polymerization of hemoglobin under deoxygenated conditions [1]. While target cells can be seen, the pathognomonic finding is the sickle cell, not the HbC crystal [1]. **NEET-PG High-Yield Pearls:** * **Mnemonic for Mutations:** **S**ickle = **V**aline (Glutamic acid to Valine); Hb**C** = **L**ysine (Glutamic acid to **L**ysine). Remember: *"Ly-C-ne"* for HbC. * **Electrophoresis:** HbC migrates the slowest toward the anode (it is the most positively charged) among common variants (A, S, F, C) [1]. * **Target Cells:** Seen in "HALT" (HbC, Asplenia, Liver disease, Thalassemia).

Q: Bence Jones proteins are:

Light chains of IgG. **Explanation:** **Bence Jones proteins (BJP)** are monoclonal globulins found in the urine, representing free **monoclonal light chains** (either kappa or lambda) of immunoglobulins. In plasma cell dyscrasias like Multiple Myeloma, there is an overproduction of these light chains by malignant plasma cells [1]. Due to their low molecular weight (approx. 22-44 kDa), they are easily filtered by the glomerulus and appear in the urine. **Analysis of Options:** * **Option D (Correct):** BJP are specifically the **light chains** of immunoglobulins (IgG being the most common class involved in Multiple Myeloma) [1]. * **Option A:** Heavy chains are not excreted in the urine in this manner; disorders involving heavy chains are classified separately as "Heavy Chain Diseases." * **Option B:** While produced by plasma cells in the bone marrow, BJP are clinically defined and detected by their presence in the **urine** [1]. * **Option C:** While some B-cell lymphomas can produce paraproteins, BJP are the classic hallmark of **Multiple Myeloma** and Waldenström Macroglobulinemia, not generalized lymphoma [1]. **High-Yield Clinical Pearls for NEET-PG:** 1. **Heat Test:** BJP have unique thermal properties; they precipitate when heated to **40-60°C** and **redissolve** upon boiling (100°C). 2. **Detection:** They are **NOT** detected by routine urine dipsticks (which detect albumin). They require **Sulphosalicylic Acid (SSA) test** or Urine Protein Electrophoresis (UPEP) [1]. 3. **Renal Impact:** BJP are nephrotoxic and lead to "Myeloma Kidney" (Cast Nephropathy) by forming waxy, eosinophilic intratubular casts. 4. **Diagnosis:** The presence of BJP is one of the diagnostic criteria for symptomatic Multiple Myeloma (CRAB features) [1].

Q: A 6-year-old child presents with abdominal pain, chronic hemolysis, and abnormal red blood cell shape on peripheral smear. What is the most likely disorder responsible for this condition?

Point mutation. The clinical presentation of a 6-year-old child with chronic hemolysis, abdominal pain (likely due to vaso-occlusive crises or biliary stones), and abnormal RBC morphology is classic for Sickle Cell Anemia (SCA) [1]. The underlying molecular defect in SCA is a Point Mutation (specifically a missense mutation) in the β-globin gene on chromosome 11 [2]. This involves the substitution of Glutamic acid by Valine at the 6th position of the β-chain [2]. This single nucleotide change causes hemoglobin to polymerize under deoxygenated conditions, leading to the characteristic "sickle" shape, hemolysis, and microvascular occlusion [1]. **Analysis of Incorrect Options:** * **Option A (Trinucleotide repeat disorder):** These involve expansions of specific gene sequences (e.g., Huntington’s disease, Fragile X syndrome). They do not cause hemolytic anemias. * **Option C (Antibodies against RBC membrane):** This describes Autoimmune Hemolytic Anemia (AIHA). While it causes hemolysis, it is an acquired condition and typically presents with spherocytes, not the specific "abnormal shapes" (sickle cells) implied in pediatric genetic contexts. * **Option D (Genetic imprinting):** This refers to epigenetic marking (e.g., Prader-Willi or Angelman syndromes) and is not the mechanism for hemoglobinopathies. **High-Yield Clinical Pearls for NEET-PG:** * **HbS Mutation:** GAG → GTG (Glutamic acid to Valine) [2]. * **HbC Mutation:** Glutamic acid to **Lysine** at the same 6th position. * **Diagnosis:** Solubility test (screening) and **Hb Electrophoresis** or HPLC (confirmatory) [1]. * **Peripheral Smear:** Sickle cells and **Howell-Jolly bodies** (indicating functional asplenia) [1]. * **Commonest cause of death:** In children, it is *S. pneumoniae* sepsis; in adults, it is Acute Chest Syndrome.

Question 1

Which of the following is the best candidate for allogeneic bone marrow transplant?

Accepted Answer

Hereditary immunodeficiency

Answer

6-year-old with thalassemia minor

Answer

Autoimmune disease

Answer

Multiple myeloma

Question 2

Which of the following clinical findings favors Thrombotic thrombocytopenic purpura in pregnancy?

Accepted Answer

Severe hemolysis

Answer

Mild DIC

Answer

Marked elevation of transaminases

Answer

Moderate thrombocytopenia

Question 3

Fresh Frozen Plasma (FFP) is stored at what temperature?

Accepted Answer

Below -25 degrees Celsius

Answer

2-4 degrees Celsius

Answer

0 to -25 degrees Celsius

Answer

2-10 degrees Celsius

Question 4

Which laboratory determinations are most helpful in distinguishing iron deficiency anemia from anemia of chronic disease?

Accepted Answer

Serum ferritin

Answer

Erythrocyte: granulocyte ratio in bone marrow

Answer

Presence or absence of polychromatophilic target cells

Answer

Presence or absence of stippled erythrocytes

Question 5

All of the following are used in the treatment of Thrombotic Thrombocytopenic Purpura, EXCEPT?

Accepted Answer

Heparin

Answer

Plasmapheresis

Answer

Corticosteroids

Answer

Immunotherapy

Question 6

Constitutional pancytopenia can be seen in which of the following conditions, except?

Accepted Answer

Diamond–Blackfan syndrome

Answer

Fanconi's anemia

Answer

Dyskeratosis congenita

Answer

Shwachman Diamond syndrome

Question 7

Evans's syndrome refers to which of the following?

Accepted Answer

Autoimmune hemolytic anemia with autoimmune thrombocytopenia

Answer

Autoimmune hemolytic anemia with autoimmune neutropenia

Answer

Autoimmune hemolytic anemia with marked bone marrow suppression

Answer

Autoimmune hemolytic anemia with hypersplenism

Question 8

A 32-year-old man has mild anemia diagnosed on routine testing. His clinical examination is normal, and a blood film reveals some target cells with some red cells having intraerythrocytic crystals. For this patient with a hemoglobin abnormality, what is the most likely diagnosis?

Accepted Answer

HbC disease

Answer

beta-thalassemia major

Answer

HbH disease

Answer

sickle cell disease

Question 9

Bence Jones proteins are:

Accepted Answer

Light chains of IgG

Answer

Heavy chains of IgG

Answer

Present in bone marrow

Answer

Seen in lymphoma

Question 10

A 6-year-old child presents with abdominal pain, chronic hemolysis, and abnormal red blood cell shape on peripheral smear. What is the most likely disorder responsible for this condition?

Accepted Answer

Point mutation

Answer

Trinucleotide repeat disorder

Answer

Antibodies against red blood cell membrane

Answer

Genetic imprinting

Hematology — MCQs

Hematology — MCQs

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