Hematopathology — MCQs

Examination of a lymph node from the neck of a 26-year-old man with bilateral cervical lymphadenopathy reveals total effacement of nodal architecture, and at higher power, the characteristic cell shown below. Which of the following additional studies is best suited for confirmation of diagnosis in this case?

Q289Easy

Smudge cells are characteristic findings in which of the following hematological malignancies?

Q290Easy

Which blood index most accurately reflects iron deficiency?

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 281: During a routine blood test among members of a family, abnormally high potassium values were noted. Which hematological anomaly is known to cause such a finding?

A. Idiopathic Thrombocytopenic Purpura (ITP)
B. Polycythemia rubra vera
C. Bernard-Soulier syndrome
D. Hereditary elliptocytosis (Correct Answer)

Explanation: **Explanation:** The correct answer is **Hereditary Elliptocytosis (HE)**. The phenomenon described is known as **Pseudohyperkalemia**. **1. Why Hereditary Elliptocytosis is correct:** In certain variants of Hereditary Elliptocytosis (specifically **Hereditary Stomatocytosis** or "leaky erythrocyte" syndromes), there is a defect in the red cell membrane proteins (like Protein 4.1 or Spectrin) [1]. This leads to an abnormal increase in membrane permeability, causing potassium to leak out of the RBCs into the plasma *in vitro* (after the blood is drawn), especially if the sample is stored at room temperature or refrigerated. This results in a falsely elevated potassium level despite the patient being clinically asymptomatic (euvolemic and normokalemic *in vivo*) [1]. **2. Why other options are incorrect:** * **ITP:** Characterized by low platelet counts; it does not typically cause pseudohyperkalemia. * **Polycythemia Rubra Vera:** While extreme thrombocytosis (platelets >1 million) or leukocytosis can cause pseudohyperkalemia due to release of intracellular potassium during the clotting process in a serum tube, it is not the classic association for "familial" or membrane-leak-related high potassium. * **Bernard-Soulier Syndrome:** A platelet adhesion defect (GPIb-IX-V deficiency) characterized by giant platelets and thrombocytopenia, not electrolyte leakage. **Clinical Pearls for NEET-PG:** * **Pseudohyperkalemia** is most commonly caused by **hemolysis** during venipuncture or **prolonged tourniquet application**. * In hematological malignancies, it occurs due to cell lysis in the test tube (Tumor Lysis Syndrome is *true* hyperkalemia; this is different). * **High-Yield Fact:** If pseudohyperkalemia is suspected, measure potassium using **heparinized plasma** rather than serum and process the sample immediately. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598.

Question 282: Deficiency of all three components of coagulation factor VIII results in which of the following conditions?

A. Von Willebrand's disease (Correct Answer)
B. Hemophilia A
C. Parahemophilia
D. Hemophilia B

Explanation: **Explanation:** The correct answer is **Von Willebrand's disease (vWD)**. To understand this, one must recognize that the "Factor VIII complex" historically consists of three functional components: 1. **vWF (von Willebrand Factor):** Responsible for platelet adhesion to subendothelial collagen [1]. 2. **VIII:C (Coagulant portion):** The procoagulant protein deficient in Hemophilia A [3]. 3. **VIII-Ag (Related Antigen):** The antigenic expression of the vWF molecule. In **vWD**, there is a quantitative or qualitative deficiency of vWF. Since vWF acts as a stabilizer and carrier protein for Factor VIII:C (protecting it from rapid degradation), a deficiency in vWF leads to a secondary decrease in VIII:C levels [1]. Thus, all components of the complex are affected. **Analysis of Incorrect Options:** * **Hemophilia A:** This is an X-linked recessive disorder caused by a deficiency of **Factor VIII:C only** [3]. The vWF levels and platelet adhesion functions are normal. * **Hemophilia B (Christmas Disease):** This is caused by a deficiency of **Factor IX**. It has no direct relationship with the Factor VIII complex. * **Parahemophilia:** This is a rare autosomal recessive bleeding disorder caused by a deficiency of **Factor V**. **NEET-PG High-Yield Pearls:** * **vWD** is the most common inherited bleeding disorder. * **Clinical Presentation:** vWD presents with "mucocutaneous bleeding" (epistaxis, menorrhagia), whereas Hemophilia presents with "deep-seated bleeding" (hemarthrosis) [2]. * **Lab Findings in vWD:** Increased Bleeding Time (BT), increased/normal APTT, and **impaired Ristocetin-induced platelet aggregation (RIPA)**. * **Treatment:** Desmopressin (DDAVP) is used in Type 1 vWD as it releases stored vWF from Weibel-Palade bodies. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 669-670. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 623-624. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 670-671.

Question 283: A 32-year-old asymptomatic female, not requiring blood transfusion, presents with Hb 13.0 gm/dl. Her HbF levels are 95% and Hb A2 levels are 1.5%. Which of the following is the most likely diagnosis?

A. Hereditary persistence of fetal hemoglobin (Correct Answer)
B. Beta homozygous thalassemia
C. Thalassemia intermedia
D. Beta heterozygous thalassemia

Explanation: ### Explanation The key to solving this question lies in the **discordance between the high HbF levels and the normal Hemoglobin (Hb) concentration.** **1. Why Option A is Correct:** **Hereditary Persistence of Fetal Hemoglobin (HPFH)** is a benign condition characterized by the failure of the "gamma-to-beta globin switch" during infancy. * **Clinical Presentation:** Patients are typically **asymptomatic** with normal red cell indices and normal total Hb levels (as seen here: Hb 13.0 gm/dl). * **Electrophoresis:** In the pancellular form of HPFH, **HbF levels are significantly elevated (often >90%)**, while HbA2 and HbA are low or absent. Because HbF functions effectively as an oxygen carrier in these individuals, they do not suffer from anemia or hemolysis. **2. Why the Other Options are Incorrect:** * **B. Beta Homozygous Thalassemia (Thalassemia Major):** This presents with severe microcytic hypochromic anemia (Hb <7 gm/dl) and hepatosplenomegaly [1]. Patients are transfusion-dependent from early childhood. While HbF is high, the patient would not be asymptomatic with a normal Hb of 13.0 gm/dl. * **C. Thalassemia Intermedia:** These patients have moderate anemia (Hb 7–10 gm/dl) and clinical symptoms like bony changes or splenomegaly [1]. They do not maintain a normal Hb of 13.0 gm/dl with 95% HbF. * **D. Beta Heterozygous Thalassemia (Minor):** Characterized by a mild anemia or normal Hb, but the hallmark is **elevated HbA2 (>3.5%)**, not massive elevations of HbF (which is usually <5% in Trait). **Clinical Pearls for NEET-PG:** * **HPFH vs. Thalassemia:** The defining difference is that HPFH is a **clinically silent** condition with normal Hb, whereas Thalassemia involves **ineffective erythropoiesis** and anemia. * **Kleihauer-Betke Stain:** In pancellular HPFH, HbF is distributed **uniformly** across all RBCs. In Thalassemia or stress erythropoiesis, HbF distribution is **heterogeneous** (F-cells). * **HbA2 levels:** Normal range is 1.5–3.5%. Elevated HbA2 is the most reliable diagnostic marker for Beta-Thalassemia Trait. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 600-602.

Question 284: Bence Jones proteins are found in all of the following conditions except?

A. Multiple myeloma
B. Reactive plasmacytosis (Correct Answer)
C. Monoclonal gammopathy of undetermined significance
D. Waldenstroms macroglobulinemia

Explanation: **Explanation:** The presence of **Bence Jones proteins (BJP)** in urine signifies the production of monoclonal free light chains (kappa or lambda). This is a hallmark of **monoclonal (neoplastic) proliferation** of plasma cells or B-lymphocytes [1]. **Why Reactive Plasmacytosis is the correct answer:** Reactive plasmacytosis is a **polyclonal** increase in plasma cells, usually occurring in response to chronic infections, inflammation, or malignancy. Because the plasma cells are polyclonal, they produce a balanced variety of light chains that do not result in the excess "free light chain" spillover into the urine characteristic of BJP. **Analysis of Incorrect Options:** * **Multiple Myeloma:** This is the classic cause of BJP. Malignant plasma cells produce excessive monoclonal light chains that are small enough to be filtered by the glomerulus and detected in urine [2]. * **Monoclonal Gammopathy of Undetermined Significance (MGUS):** Although a premalignant state with lower levels of M-protein than myeloma, MGUS involves a monoclonal population and can frequently present with small amounts of BJP [3]. * **Waldenström Macroglobulinemia:** This is a lymphoplasmacytic lymphoma producing monoclonal IgM. Like other monoclonal gammopathies, it can result in the excretion of free light chains (BJP) in approximately 30-40% of cases [1], [3]. **High-Yield Clinical Pearls for NEET-PG:** * **Detection:** BJP are unique because they **precipitate at 40-60°C** and **redissolve at 100°C** (boiling). * **Dipstick Warning:** Standard urine dipsticks primarily detect albumin; they **cannot** detect Bence Jones proteins. Sulfosalicylic acid (SSA) testing or Urine Protein Electrophoresis (UPEP) is required. * **Complication:** BJP are nephrotoxic and lead to "Myeloma Kidney" (cast nephropathy) [4]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 608-609. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 606-607. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 942-943.

Question 285: All are examples of microangiopathic hemolytic anemia except?

A. Thrombotic thrombocytopenic purpura (TTP)
B. Hemolytic uremic syndrome (HUS)
C. Disseminated intravascular coagulation (DIC)
D. Immune thrombocytopenic purpura (ITP) (Correct Answer)

Explanation: **Explanation:** **Microangiopathic Hemolytic Anemia (MAHA)** is a subcategory of microangiopathic hemolytic anemias characterized by the mechanical destruction of red blood cells (fragmentation) as they pass through narrowed or fibrin-clotted small blood vessels [1]. The hallmark finding on a peripheral blood smear is the presence of **Schistocytes** (helmet cells) [4]. **Why ITP is the Correct Answer:** **Immune Thrombocytopenic Purpura (ITP)** is an isolated consumption of platelets caused by anti-platelet antibodies (Type II Hypersensitivity). It involves the destruction of platelets in the spleen, but it does **not** involve microvascular thrombi or fibrin deposition. Therefore, there is no mechanical trauma to RBCs, and schistocytes are absent. It is a primary platelet disorder, not a hemolytic anemia. **Analysis of Incorrect Options:** * **TTP:** Caused by a deficiency of **ADAMTS13** [2], leading to large vWF multimer-induced platelet thrombi that shear RBCs. * **HUS:** Typically follows Shiga-toxin-producing *E. coli* (O157:H7) infection; microthrombi in renal capillaries cause RBC fragmentation [3]. * **DIC:** Involves widespread activation of the coagulation cascade, leading to **fibrin strands** in the microvasculature that "slice" RBCs as they pass [1]. **High-Yield Clinical Pearls for NEET-PG:** * **The Pentad of TTP:** Fever, Anemia (MAHA), Thrombocytopenia, Neurological symptoms, and Renal failure (**FAT RN**) [2]. * **Diagnostic Hallmark:** Schistocytes on peripheral smear + Elevated LDH + Decreased Haptoglobin. * **Coagulation Profile:** PT and APTT are **normal** in TTP/HUS but **prolonged** in DIC [1]. * **Other MAHA causes:** Malignant hypertension, Pre-eclampsia/HELLP syndrome, and prosthetic heart valves (Macroangiopathic). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 667-668. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 947-948. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 946-947. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 540-541.

Question 286: Dohle bodies can be seen in which of the following conditions?

A. Plasma cell myeloma
B. Sepsis (Correct Answer)
C. Chronic granulomatous disease
D. Rheumatoid arthritis

Explanation: **Explanation:** **Dohle bodies** are small, light blue-grey, oval inclusions found in the periphery of the cytoplasm of **neutrophils**. They represent remnants of **rough endoplasmic reticulum (RER)** arranged in parallel rows. **Why Sepsis is Correct:** Dohle bodies are a hallmark of **toxic granulation** and "left shift" seen in states of intense inflammation or cytokine stress. In **sepsis** (or severe bacterial infections), there is accelerated granulopoiesis. The rapid maturation of neutrophils in the bone marrow leads to defects in cytoplasmic maturation, causing fragments of RER to persist in the mature cell. They are frequently seen alongside toxic granules and cytoplasmic vacuoles. **Analysis of Incorrect Options:** * **Plasma cell myeloma:** This is a malignancy of plasma cells characterized by Russell bodies (intracellular Ig) or Mott cells, not neutrophil inclusions. * **Chronic granulomatous disease (CGD):** This is a functional defect of the NADPH oxidase enzyme. Neutrophils appear morphologically normal under light microscopy but fail the Nitroblue Tetrazolium (NBT) test. * **Rheumatoid arthritis:** While a chronic inflammatory state, it does not typically manifest with Dohle bodies unless there is a concurrent acute systemic infection or Felty’s syndrome. **High-Yield Clinical Pearls for NEET-PG:** * **Composition:** Dohle bodies consist of **Ribosomal RNA/Rough ER**. * **Differential Diagnosis:** Apart from sepsis/infection, they are classically seen in **May-Hegglin Anomaly** (associated with giant platelets and thrombocytopenia), burns, and normal pregnancy. * **Stain:** They are best visualized using **Romanowsky stains** (Leishman or Giemsa). * **Toxic Triad:** In severe infection, look for the triad of **Dohle bodies, Toxic granulations, and Cytoplasmic vacuolation** in neutrophils.

Question 287: All of the following are present in cryoprecipitate, except?

A. Von Willebrand Factor
B. Factor I
C. Factor VIII
D. Factor V (Correct Answer)

Explanation: ### Explanation **Cryoprecipitate** is the cold-insoluble fraction of plasma obtained by thawing Fresh Frozen Plasma (FFP) at 1–6°C. It is a concentrated source of specific clotting factors, and understanding its composition is high-yield for NEET-PG. **Why Factor V is the correct answer:** Factor V is a **labile clotting factor** that remains in the supernatant (the liquid portion) during the cold-thawing process. It does not precipitate out. Therefore, Factor V is present in Fresh Frozen Plasma (FFP) but is **absent** in cryoprecipitate. **Analysis of Incorrect Options:** * **Factor I (Fibrinogen):** Cryoprecipitate is the primary source of concentrated fibrinogen (approx. 150–250 mg per unit). It is the treatment of choice for hypofibrinogenemia. * **Factor VIII:** It contains significant amounts of Factor VIII (anti-hemophilic factor), making it useful in Hemophilia A if specific concentrates are unavailable. * **Von Willebrand Factor (vWF):** Cryoprecipitate is rich in vWF, which is why it was historically used to treat Von Willebrand Disease. * *Note:* **Factor XIII** (Fibrin Stabilizing Factor) and **Fibronectin** are also present in cryoprecipitate. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for Cryoprecipitate contents:** "**1, 8, 13, vWF**" (Factors I, VIII, XIII, and vWF). * **Storage:** Cryoprecipitate is stored at **-18°C or colder** and has a shelf life of 1 year. Once thawed, it must be used within 6 hours. * **Indication:** The most common modern indication is **hypofibrinogenemia** (e.g., in DIC or massive transfusion protocols). * **Factor V deficiency:** Must be treated with **FFP**, as cryoprecipitate does not contain it.

Question 288: Examination of a lymph node from the neck of a 26-year-old man with bilateral cervical lymphadenopathy reveals total effacement of nodal architecture, and at higher power, the characteristic cell shown below. Which of the following additional studies is best suited for confirmation of diagnosis in this case?

A. Serum protein electrophoresis
B. Immunohistochemistry (Correct Answer)
C. Flow cytometry
D. Gene rearrangement studies

Explanation: ***Immunohistochemistry*** - **CD15** and **CD30** positivity is the **gold standard** for confirming **Hodgkin's lymphoma** with **Reed-Sternberg cells**, which show characteristic **owl's eye** appearance with large nucleoli. - **Immunohistochemistry** provides definitive identification of the **Reed-Sternberg cell phenotype** and distinguishes it from other large cell lymphomas through specific marker expression. *Serum protein electrophoresis* - Used primarily to detect **monoclonal gammopathy** and **multiple myeloma** through identification of **M-protein** spikes. - Not specific for **lymphoma diagnosis** and cannot identify the characteristic **Reed-Sternberg cell** markers needed for Hodgkin's lymphoma confirmation. *Flow cytometry* - **Reed-Sternberg cells** are typically **too large** and **fragile** to be effectively analyzed by flow cytometry, often getting lost during sample preparation. - More suitable for **B-cell** and **T-cell lymphomas** where smaller, intact lymphocytes can be analyzed for surface markers. *Gene rearrangement studies* - Primarily used to detect **clonal B-cell** or **T-cell populations** in **non-Hodgkin lymphomas** through **immunoglobulin** or **T-cell receptor** gene rearrangements. - **Hodgkin's lymphoma** diagnosis relies on **morphology** and **immunophenotype** rather than specific gene rearrangement patterns.

Question 289: Smudge cells are characteristic findings in which of the following hematological malignancies?

A. Nodular sclerosis Hodgkin's lymphoma
B. Chronic lymphocytic leukemia (Correct Answer)
C. Lymphocyte predominant Hodgkin's lymphoma
D. Sezary syndrome

Explanation: **Explanation:** **Chronic Lymphocytic Leukemia (CLL)** is the correct answer. Smudge cells (also known as **Basket cells**) are remnants of fragile, mature-appearing small lymphocytes that rupture during the preparation of a peripheral blood smear [1]. In CLL, the neoplastic B-cells have an altered cytoskeleton, making them mechanically delicate. While not pathognomonic, the presence of numerous smudge cells in an elderly patient with absolute lymphocytosis is a classic diagnostic hallmark of CLL/SLL [1]. **Analysis of Incorrect Options:** * **Nodular Sclerosis Hodgkin’s Lymphoma:** Characterized by **Lacunar cells** (RS cell variants) and collagen bands. Smudge cells are not a feature. * **Lymphocyte Predominant Hodgkin’s Lymphoma:** Characterized by **"Popcorn cells"** (L&H cells) which express B-cell markers like CD20. * **Sezary Syndrome:** A cutaneous T-cell lymphoma characterized by **Sezary cells**, which are atypical lymphocytes with **cerebriform nuclei** (grooved, brain-like appearance). **High-Yield Clinical Pearls for NEET-PG:** * **The "Smudge Cell" Hack:** Adding a drop of **bovine albumin** to the blood sample before spreading the film can prevent the formation of smudge cells, allowing for better visualization of nuclear morphology. * **Immunophenotype of CLL:** Characteristically positive for **CD5** (a T-cell marker), **CD19, CD20, and CD23** [1]. It shows weak expression of surface immunoglobulins (sIg). * **Prognostic Marker:** High levels of **ZAP-70** or **CD38** expression and **17p deletion** (TP53 mutation) indicate a poor prognosis in CLL. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, p. 602.

Question 290: Which blood index most accurately reflects iron deficiency?

A. MCV
B. MCH
C. MCHC (Correct Answer)
D. PCV

Explanation: **Explanation:** In Iron Deficiency Anemia (IDA), the primary defect is a decrease in hemoglobin synthesis. **MCHC (Mean Corpuscular Hemoglobin Concentration)** is the most accurate index for iron deficiency because it measures the concentration of hemoglobin in a given volume of packed red cells. As iron stores deplete, hemoglobin production falls significantly relative to the cell size, leading to **hypochromia**. A low MCHC is the hallmark of true hypochromic anemia. **Analysis of Options:** * **MCV (Mean Corpuscular Volume):** Reflects the average size of RBCs. While MCV decreases in IDA (microcytosis), it is also low in other conditions like Thalassemia trait, Anemia of Chronic Disease, and Sideroblastic anemia [1]. It is a sensitive but less specific indicator of iron status compared to MCHC. * **MCH (Mean Corpuscular Hemoglobin):** Measures the average weight of hemoglobin per RBC. Since MCH is mathematically dependent on the cell size (MCV), it often mirrors MCV and is less reliable as an isolated measure of hemoglobin concentration. * **PCV (Packed Cell Volume):** Also known as Hematocrit, it reflects the proportion of blood occupied by RBCs. It is used to screen for anemia in general but does not provide specific information about the morphological type or etiology. **NEET-PG High-Yield Pearls:** * **Earliest sign of IDA:** Decreased **Serum Ferritin** (most sensitive biochemical marker). * **Earliest hematological change:** Increase in **RDW** (Red Cell Distribution Width), reflecting anisocytosis [1]. * **Mentzer Index (MCV/RBC count):** Used to differentiate IDA (>13) from Thalassemia trait (<13). * **Gold Standard for Iron Stores:** Bone marrow aspiration with **Perl’s Prussian Blue stain** (showing absent hemosiderin). **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

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