Hematopathology — MCQs

A 23-year-old female presented with palpable lumps in the axilla and supra-clavicular region. Following investigations including X-ray, USG-guided biopsy, and HPE, a diagnosis was made. Flow cytometry revealed CD15 and CD30 positivity. She was started on chemotherapy. Later, the patient developed fatigue, dyspnea, and orthopnea, with ECG showing left ventricular hypertrophy. Which of the following chemotherapeutic agents is most likely responsible for these manifestations?

Q667Easy

Dohle-like inclusions in neutrophils along with giant platelets is characteristic of which of the following conditions?

Q668Easy

Which of the following is a common bleeding manifestation of hemophilia?

Q669Easy

Multiple myeloma is characterized by all of the following except?

Q670Easy

Ringed sideroblasts are seen in which of the following conditions?

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 661: Which of the following is NOT a feature of multiple myeloma?

A. Hypercalcemia
B. Anemia
C. Hyperviscosity
D. Elevated alkaline phosphatase (Correct Answer)

Explanation: **Explanation:** In **Multiple Myeloma (MM)**, the characteristic bone lesions are **purely lytic** (“punched-out” lesions) [1, 5]. These are caused by the activation of osteoclasts (via RANKL) and the inhibition of osteoblasts [1]. Because there is a lack of new bone formation or osteoblastic activity, the serum **Alkaline Phosphatase (ALP) remains normal**. An elevated ALP in a patient with lytic lesions should prompt a search for alternative diagnoses, such as metastatic carcinoma or healing fractures. **Analysis of Options:** * **A. Hypercalcemia:** This is a hallmark of MM (part of the **CRAB** criteria) [5]. It results from extensive bone resorption by osteoclasts, releasing calcium into the bloodstream [1]. * **B. Anemia:** Most patients present with normocytic normochromic anemia due to bone marrow infiltration by malignant plasma cells and decreased erythropoietin production (secondary to renal impairment) [1]. * **C. Hyperviscosity:** This occurs due to the excessive production of monoclonal immunoglobulins (M-protein) [2]. While more common in Waldenström Macroglobulinemia, it can occur in MM, especially with IgA or high-concentration IgG paraproteins [4]. **NEET-PG High-Yield Pearls:** 1. **CRAB Criteria:** **C**alcium (elevated), **R**enal failure, **A**nemia, **B**one lesions [5]. 2. **Diagnosis:** Bone marrow plasma cells **≥10%** or biopsy-proven plasmacytoma [1, 2]. 3. **Blood Film:** **Rouleaux formation** (due to decreased zeta potential between RBCs) and increased ESR [3]. 4. **Urine:** **Bence-Jones proteins** (free light chains) which precipitate at 40-60°C and redissolve at 100°C [2, 3]. 5. **Radiology:** Skeletal survey is preferred over Bone Scan (Bone scans are often negative because they detect osteoblastic activity, which is absent in MM) [5]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 617-618. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617. [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. 607-608. [4] 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. [5] 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. 608.

Question 662: What is the shelf life of blood stored in CPDA buffer in a blood bank?

A. 21 days
B. 30 days
C. 35 days (Correct Answer)
D. 42 days

Explanation: **Explanation:** The shelf life of stored blood is determined by the anticoagulant-preservative solution used, which maintains red cell viability and function by providing glucose for metabolism and preventing coagulation. **1. Why C is correct:** **CPDA-1 (Citrate Phosphate Dextrose Adenine)** is the most commonly used preservative in blood banks. The addition of **Adenine** is the key factor here; it acts as a substrate for ATP synthesis, which maintains the red cell membrane integrity and extends the shelf life to **35 days**. Without adenine (as in CPD), the shelf life is significantly shorter. **2. Why the other options are incorrect:** * **A (21 days):** This is the shelf life for older preservatives like **ACD (Acid Citrate Dextrose)** and **CPD (Citrate Phosphate Dextrose)**. These lack adenine, leading to faster ATP depletion. * **B (30 days):** This is not a standard shelf life for any conventional blood bag preservative. * **D (42 days):** This is the shelf life for blood stored with **Additive Solutions (SAGM - Saline, Adenine, Glucose, and Mannitol)**. In this system, the plasma is removed, and the additive solution is added to the packed red cells to extend viability. **3. High-Yield Clinical Pearls for NEET-PG:** * **Storage Temperature:** Whole blood and PRBCs must be stored at **2°C to 6°C**. * **The "Storage Lesion":** During storage, there is a decrease in 2,3-DPG (shifting the oxygen dissociation curve to the left), a decrease in pH, and an **increase in extracellular Potassium** (important in neonatal transfusions). * **Platelet Storage:** Unlike RBCs, platelets are stored at **20°C to 24°C** with constant agitation and have a shelf life of only **5 days**. * **FFP Storage:** Frozen at **-30°C or colder**, FFP has a shelf life of **1 year**.

Question 663: Cytogenetic studies in a 70-year-old woman with chronic myelogenous leukemia (CML) demonstrate a t(9;22) chromosomal translocation. Which of the following best explains the role of this translocation in the pathogenesis of leukemia in this patient?

A. Altered DNA methylation status
B. Enhanced expression of telomerase gene
C. Expansion of a trinucleotide repeat
D. Protooncogene activation (Correct Answer)

Explanation: **Explanation:** **1. Why Protooncogene Activation is Correct:** The hallmark of Chronic Myelogenous Leukemia (CML) is the **Philadelphia chromosome [t(9;22)(q34;q11)]** [1]. This translocation involves the fusion of the **ABL1** proto-oncogene (from chromosome 9) with the **BCR** gene (on chromosome 22) [2], [3]. The resulting **BCR-ABL1 fusion gene** encodes a chimeric protein with constitutive (permanently "on") **tyrosine kinase activity** [3], [4]. This unregulated signaling drives uncontrolled proliferation of myeloid stem cells and inhibits apoptosis, directly leading to leukemogenesis [2], [4]. Thus, the translocation activates a proto-oncogene (ABL) into a potent oncogene. **2. Why Other Options are Incorrect:** * **A. Altered DNA methylation status:** This refers to epigenetic modification (e.g., silencing of tumor suppressor genes), which is common in many cancers but is not the primary mechanism driven by the t(9;22) translocation. * **B. Enhanced expression of telomerase gene:** While telomerase activation allows cancer cells to achieve immortality, it is a downstream feature of many malignancies rather than the specific result of the Philadelphia translocation. * **C. Expansion of a trinucleotide repeat:** This is the mechanism for neurogenetic disorders like Huntington’s disease or Fragile X syndrome, not hematologic malignancies. **3. NEET-PG High-Yield Pearls:** * **The Fusion Protein:** The most common variant in CML is the **p210** protein. (Note: p190 is associated with ALL; p230 with Chronic Neutrophilic Leukemia). * **Diagnosis:** Gold standard is **FISH** (for the BCR-ABL gene) [1] or **RT-PCR**. * **Treatment:** Targeted therapy with Tyrosine Kinase Inhibitors (TKIs) like **Imatinib** has revolutionized CML management [2]. * **Cytogenetics:** The Philadelphia chromosome is found in >95% of CML cases [1], [3]. Its absence in a suspected case suggests a different myeloproliferative neoplasm. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 225-226. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 295-296. [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, p. 624. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 605-607.

Question 664: What is the most common mutation found in hereditary elliptocytosis?

A. Spectrin (Correct Answer)
B. Ankyrin
C. Glycophorin A
D. Band 4.2

Explanation: **Explanation:** Hereditary Elliptocytosis (HE) is an autosomal dominant red blood cell membrane disorder characterized by the presence of elliptical or cigar-shaped erythrocytes. The primary defect lies in the **horizontal interactions** of the cytoskeleton, which leads to mechanical instability of the RBC membrane. **Why Spectrin is correct:** The most common molecular defect in HE involves mutations in **α-spectrin** (specifically the SPTA1 gene), followed by β-spectrin. These mutations typically disrupt the formation of **spectrin dimers into tetramers**. When the spectrin scaffold cannot properly assemble or cross-link, the cell fails to recoil to its biconcave shape after passing through microcapillaries, resulting in permanent elliptical deformation. **Why other options are incorrect:** * **Ankyrin:** Mutations in Ankyrin are the most common cause of **Hereditary Spherocytosis (HS)**, affecting the *vertical* stability between the skeleton and the lipid bilayer [1]. * **Glycophorin A:** While Glycophorin C mutations can cause a rare subtype of HE (Leach phenotype), Glycophorin A is primarily a blood group antigen carrier and not the most common site for HE mutations. * **Band 4.2:** Deficiency of Protein 4.2 is a known but less common cause of Hereditary Spherocytosis [1], particularly prevalent in the Japanese population. **High-Yield Clinical Pearls for NEET-PG:** * **HE Hallmark:** Defect in **Horizontal interactions** (Spectrin tetramer assembly). * **HS Hallmark:** Defect in **Vertical interactions** (Ankyrin/Band 3/Spectrin binding to membrane) [1]. * **Blood Smear:** >25% elliptocytes is diagnostic for HE. * **Southeast Asian Ovalocytosis (SAO):** A specific subtype of HE caused by a mutation in **Band 3**, providing protection against malaria. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-641.

Question 665: Which cells are characteristic of Hodgkin's disease?

A. Lacunar cells
B. Reed-Sternberg cells (Correct Answer)
C. Giant cells
D. Eosinophils

Explanation: ### Explanation **Correct Answer: B. Reed-Sternberg cells** **Reasoning:** The hallmark of Hodgkin Lymphoma (HL) is the presence of **Reed-Sternberg (RS) cells** within a polymorphic inflammatory background [2]. These are large, multinucleated (or bilobed) B-cells characterized by prominent, eosinophilic, "owl-eye" nucleoli [1]. While they are the neoplastic component, they typically constitute only 1–5% of the total tumor mass, with the remainder being a reactive inflammatory infiltrate [2]. Their classic immunophenotype is **CD15+ and CD30+** (except in the Nodular Lymphocyte Predominant subtype). **Analysis of Incorrect Options:** * **A. Lacunar cells:** These are a *variant* of RS cells seen specifically in the **Nodular Sclerosis** subtype of HL, appearing as a cell within an open space created by cytoplasmic disruption [1]. While characteristic of that subtype, "Reed-Sternberg cell" is the broader, definitive term for the disease as a whole. * **C. Giant cells:** This is a non-specific term. While RS cells are technically giant cells [2], the term "Giant cells" usually refers to Langhans or foreign-body giant cells seen in granulomatous inflammation (e.g., Tuberculosis). * **D. Eosinophils:** These are part of the reactive inflammatory background recruited by cytokines (like IL-5) secreted by RS cells. While common in HL, they are not the diagnostic neoplastic cells [1]. **High-Yield NEET-PG Pearls:** * **Popcorn Cells (L&H cells):** Characteristic of Nodular Lymphocyte Predominant HL (CD20+, CD15-, CD30-). * **Bimodal Age Distribution:** HL shows peaks in the 20s and after age 50 [2]. * **EBV Association:** Most commonly associated with the **Mixed Cellularity** subtype, where RS cells are infected with EBV in about 70% of cases [3]. * **Staging:** The **Ann Arbor Staging System** is used to determine the extent of the disease. **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. 616. [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. 614-616. [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. 616-618.

Question 666: A 23-year-old female presented with palpable lumps in the axilla and supra-clavicular region. Following investigations including X-ray, USG-guided biopsy, and HPE, a diagnosis was made. Flow cytometry revealed CD15 and CD30 positivity. She was started on chemotherapy. Later, the patient developed fatigue, dyspnea, and orthopnea, with ECG showing left ventricular hypertrophy. Which of the following chemotherapeutic agents is most likely responsible for these manifestations?

A. Daunorubicin
B. Bleomycin (Correct Answer)
C. Capecitabine
D. Cyclophosphamide

Explanation: ### Explanation **Diagnosis and Clinical Context:** The patient is a young female with lymphadenopathy (axillary and supraclavicular) and flow cytometry showing **CD15+ and CD30+** markers [2]. This is the classic immunophenotype for **Hodgkin Lymphoma (HL)**. The standard treatment for HL is the **ABVD regimen** (Adriamycin/Doxorubicin, Bleomycin, Vinblastine, and Dacarbazine). **Why Bleomycin is the Correct Answer:** The patient developed fatigue, dyspnea, and orthopnea, which are signs of respiratory distress. While the question mentions ECG showing Left Ventricular Hypertrophy (LVH), this is often a "distractor" or a secondary finding in chronic pulmonary hypertension [1]. The primary toxicity associated with **Bleomycin** is **Pulmonary Fibrosis**. This leads to restrictive lung disease, impaired gas exchange, and eventual right-sided heart strain (cor pulmonale), which can mimic or exacerbate symptoms of heart failure. *Note: While Doxorubicin causes cardiotoxicity, Bleomycin is the "classic" high-yield association for pulmonary symptoms in HL patients.* **Analysis of Incorrect Options:** * **A. Daunorubicin:** An anthracycline similar to Doxorubicin. While it causes dilated cardiomyopathy (congestive heart failure), it is primarily used in acute leukemias (AML/ALL), not the standard ABVD regimen for Hodgkin Lymphoma. * **C. Capecitabine:** An oral prodrug of 5-Fluorouracil used mainly in colorectal and breast cancers. Its hallmark side effect is Hand-Foot Syndrome. * **D. Cyclophosphamide:** An alkylating agent used in the CHOP regimen (for Non-Hodgkin Lymphoma). Its most characteristic side effect is hemorrhagic cystitis. **High-Yield Clinical Pearls for NEET-PG:** 1. **Bleomycin Toxicity:** Dose-dependent pulmonary fibrosis; monitored via Pulmonary Function Tests (PFTs), specifically **DLCO** (Diffusion Capacity of Carbon Monoxide). 2. **Hodgkin Markers:** Reed-Sternberg cells are typically **CD15+, CD30+, and CD45–** [2]. 3. **Anthracycline Toxicity:** Doxorubicin/Daunorubicin cause cardiotoxicity via oxygen free radical formation; prevented by **Dexrazoxane**. 4. **Vincristine (from MOPP/OEPA):** Associated with peripheral neuropathy (foot drop/paralytic ileus). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, p. 707. [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. 614-618.

Question 667: Dohle-like inclusions in neutrophils along with giant platelets is characteristic of which of the following conditions?

A. Bernard-Soulier syndrome
B. Chédiak-Higashi syndrome
C. May-Hegglin anomaly (Correct Answer)
D. Pelger-Huët anomaly

Explanation: **Explanation:** **May-Hegglin Anomaly (MHA)** is an autosomal dominant disorder characterized by a triad of **thrombocytopenia, giant platelets, and large, basophilic leukocyte inclusions** (Döhle-like bodies). 1. **Why it is correct:** The condition is caused by a mutation in the **MYH9 gene**, which encodes the non-muscle myosin heavy chain IIA. This defect leads to disordered megakaryocyte maturation (resulting in giant platelets) and the precipitation of myosin heavy chains in the cytoplasm of neutrophils, forming **Döhle-like bodies**. Unlike true Döhle bodies (seen in infections/burns), these are larger, more prominent, and persist even in the absence of inflammation. 2. **Why other options are incorrect:** * **Bernard-Soulier Syndrome:** Features giant platelets and thrombocytopenia (due to GpIb-IX-V deficiency) [1], but **lacks** leukocyte inclusions. * **Chédiak-Higashi Syndrome:** Characterized by **massive lysosomal granules** in leukocytes and partial albinism, not Döhle-like bodies or giant platelets. * **Pelger-Huët Anomaly:** Involves a failure of nuclear segmentation (hyposegmented, "pince-nez" neutrophils) but does not affect platelets. **High-Yield Clinical Pearls for NEET-PG:** * **MYH9-Related Disorders:** MHA is part of a spectrum including Epstein and Fechtner syndromes (which add nephritis and sensorineural hearing loss to the hematologic findings). * **Döhle-like vs. Döhle bodies:** True Döhle bodies are composed of **rough endoplasmic reticulum**; Döhle-like bodies in MHA are composed of **myosin heavy chains**. * **Peripheral Smear:** Always look for the combination of "Big Platelets + Blue Inclusions" to diagnose May-Hegglin. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669.

Question 668: Which of the following is a common bleeding manifestation of hemophilia?

A. Hemoptysis
B. Hemarthrosis (Correct Answer)
C. Hematomas
D. Hematuria

Explanation: **Explanation:** Hemophilia (A and B) is a disorder of **secondary hemostasis** caused by deficiencies in clotting factors VIII and IX, respectively [1]. Unlike primary hemostatic defects (platelet disorders), which present with superficial mucosal bleeding, secondary hemostatic defects manifest as deep-seated bleeding. **Why Hemarthrosis is Correct:** **Hemarthrosis** (bleeding into joint spaces) is the hallmark clinical manifestation of hemophilia [1]. It occurs most commonly in the weight-bearing joints (knees, elbows, and ankles). Repeated episodes lead to synovial hypertrophy and chronic joint destruction, known as hemophilic arthropathy [1]. **Analysis of Incorrect Options:** * **Hemoptysis (A):** Bleeding from the respiratory tract is rare in hemophilia and usually suggests underlying pulmonary pathology (e.g., TB or malignancy) rather than a primary coagulation defect. * **Hematomas (C):** While large, deep muscle hematomas are common in hemophilia, they are generally considered less specific or "classic" than hemarthrosis [1]. In the context of a "most common/hallmark" question, hemarthrosis is the preferred answer. * **Hematuria (D):** Though it can occur in hemophiliacs, it is not the primary or most characteristic presenting feature [1]. **NEET-PG High-Yield Pearls:** * **Inheritance:** Both Hemophilia A and B are **X-linked recessive**. * **Lab Findings:** Characterized by a **prolonged aPTT** with a **normal PT and Bleeding Time (BT)**. * **Mixing Study:** The prolonged aPTT will **correct** upon mixing with normal plasma (differentiating it from factor inhibitors). * **Treatment:** Factor replacement is the mainstay. For mild Hemophilia A, **Desmopressin (DDAVP)** can be used as it releases stored Factor VIII and vWF from Weibel-Palade bodies. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 623-624.

Question 669: Multiple myeloma is characterized by all of the following except?

A. Presence of light chains
B. Monoclonal gammopathy
C. Polyclonal gammopathy (Correct Answer)
D. Hypergammaglobulinemia

Explanation: **Explanation:** Multiple myeloma (MM) is a **monoclonal** neoplastic proliferation of plasma cells, typically derived from a single clone [1]. This results in the overproduction of a specific, identical immunoglobulin (usually IgG or IgA), known as the **M-protein** or **Paraprotein** [1]. **1. Why "Polyclonal Gammopathy" is the correct answer (The Exception):** In MM, the malignant transformation occurs in a single plasma cell clone [3]. This leads to **Monoclonal Gammopathy**, characterized by a sharp "M-spike" on Serum Protein Electrophoresis (SPEP). Conversely, *Polyclonal* gammopathy involves an increase in many different types of antibodies and is seen in chronic infections, liver disease, or autoimmune conditions—not in plasma cell dyscrasias like Multiple Myeloma. **2. Analysis of Incorrect Options:** * **Monoclonal Gammopathy & Hypergammaglobulinemia:** These are hallmarks of MM [1]. The massive production of the M-protein leads to an overall increase in serum gamma globulins (hypergammaglobulinemia), but specifically of one type (monoclonal) [2]. * **Presence of Light Chains:** In many cases, plasma cells produce excess free light chains (Kappa or Lambda). These are small enough to be filtered by the kidney and appear in the urine as **Bence-Jones proteins** [2]. **Clinical Pearls for NEET-PG:** * **CRAB Criteria:** Remember the classic presentation: **C**alcium elevation, **R**enal insufficiency, **A**nemia, and **B**one lesions (punched-out lytic lesions) [5]. * **Diagnosis:** Bone marrow biopsy showing **>10% clonal plasma cells** is a major diagnostic criterion. * **Peripheral Smear:** Look for **Rouleaux formation** due to increased serum proteins decreasing the zeta potential between RBCs [4]. * **Flame Cells:** Associated with IgA myeloma; **Mott cells** (grape-like inclusions) are also a high-yield morphological finding. **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, pp. 606-607. [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] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 616-617. [4] 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. 607-608. [5] 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. 608.

Question 670: Ringed sideroblasts are seen in which of the following conditions?

A. Iron deficiency anemia
B. Myelodysplastic syndrome (Correct Answer)
C. Thalassemia
D. Anemia of chronic disease

Explanation: **Explanation:** **Ringed sideroblasts** are erythroblasts with iron-laden mitochondria encircling at least one-third of the nucleus. This occurs due to a defect in **heme synthesis**, where iron enters the mitochondria but cannot be incorporated into protoporphyrin, leading to mitochondrial iron overload. 1. **Why Myelodysplastic Syndrome (MDS) is correct:** MDS (specifically the subtype **MDS-RS**) is a classic cause of ringed sideroblasts [1]. It is often associated with a mutation in the **SF3B1 gene**. The ineffective erythropoiesis leads to the accumulation of non-ferritin iron in the mitochondria, visible on **Perls’ Prussian Blue stain**. 2. **Why the other options are incorrect:** * **Iron Deficiency Anemia:** There is a total lack of iron; therefore, sideroblasts (even normal ones) are absent or significantly decreased. * **Thalassemia:** While there is iron overload, it is usually systemic or cytoplasmic. Ringed sideroblasts are not a defining feature of Thalassemia. * **Anemia of Chronic Disease:** Iron is trapped within the reticuloendothelial system (macrophages) due to high **hepcidin** levels [2]. Iron is unavailable to the erythroid precursors, so ringed sideroblasts are not formed. **High-Yield Clinical Pearls for NEET-PG:** * **Stain used:** Perls’ Prussian Blue is essential to visualize ringed sideroblasts. * **Definition:** A ringed sideroblast must have $\geq$ 5 iron granules covering $\geq$ 1/3rd of the nuclear circumference. * **Other Causes:** Sideroblastic anemia can also be caused by **Lead poisoning**, **Alcoholism**, **Isoniazid (Vitamin B6 deficiency)**, and **Chloramphenicol**. * **Mnemonic:** "LEAD" for Sideroblastic causes: **L**ead, **E**thanol, **A**IDS drugs/Isoniazid, **D**eficiency of B6. **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, pp. 622-624. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 658-662.

Practice by Chapter

Anemias: Classification and Approach

Practice Questions

Hemolytic Anemias

Practice Questions

Myeloproliferative Neoplasms

Practice Questions

Myelodysplastic Syndromes

Practice Questions

Acute Leukemias

Practice Questions

Chronic Leukemias

Practice Questions

Lymphomas and Lymphoid Neoplasms

Practice Questions

Plasma Cell Disorders

Practice Questions

Bleeding Disorders

Practice Questions

Thrombotic Disorders

Practice Questions

Want unlimited practice?

Get full access to all questions, explanations, and performance tracking.

Start For Free