Hematological Disorders — MCQs
Which of the following is the most common myeloproliferative disorder?
Which of the following conditions is least likely to cause proptosis?
Which condition is commonly associated with Disseminated Intravascular Coagulation (D.I.C.)?
Hyperviscosity is seen in all, except:
Parachute lesions are associated with which of the following conditions?
Retinitis pigmentosa is a feature of all except which of the following?
Match List-I with List-II and select the correct answer using the code given below the Lists:
All of the following may be used to differentiate Central Retinal Venous Occlusion (CRVO) from Ocular Ischemic Syndrome due to Carotid Artery Stenosis, EXCEPT:
Not a feature of ligneous conjunctivitis:
In a patient with AIDS, what typically causes chorioretinitis?
Hematological Disorders Indian Medical PG Practice Questions and MCQs
Question 1: Which of the following is the most common myeloproliferative disorder?
- A. Essential Thrombocythemia (Correct Answer)
- B. Polycythemia rubra vera
- C. CML
- D. Myelofibrosis
Explanation: ***Essential Thrombocythemia*** - **Essential thrombocythemia (ET)** is the **most common myeloproliferative neoplasm**, with an incidence of approximately 1.5-2.4 per 100,000 per year. - It is characterized by **persistent thrombocytosis** (platelet count >450,000/μL) and megakaryocytic proliferation [1]. - Commonly associated with **JAK2 V617F mutation** (~55-60%), **CALR mutations** (~25-30%), and **MPL mutations** (~3-5%) [2]. *Polycythemia rubra vera* - **Polycythemia vera (PV)** is the **second most common** classic MPN, with an incidence of approximately 0.8-2.3 per 100,000 per year. - Characterized by increased red blood cell mass, often with leukocytosis and thrombocytosis [1]. - Strongly associated with **JAK2 V617F mutation** (present in >95% of cases) [2][3]. *CML* - **Chronic myeloid leukemia (CML)** has similar incidence to PV (approximately 1-2 per 100,000 per year). - Defined by the presence of the **Philadelphia chromosome (BCR-ABL1 fusion gene)** [2]. - Treated distinctly with tyrosine kinase inhibitors (TKIs). *Myelofibrosis* - **Primary myelofibrosis (PMF)** is the **least common** of the classic MPNs, with an incidence of approximately 0.3-1.5 per 100,000 per year. - Characterized by bone marrow fibrosis, extramedullary hematopoiesis, and splenomegaly [3]. - Associated with **JAK2, CALR, or MPL mutations** [2]. **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. 627-628. [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, p. 624. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615.
Question 2: Which of the following conditions is least likely to cause proptosis?
- A. Sarcoidosis
- B. Myxoedema (Correct Answer)
- C. Grave's disease
- D. Pituitary adenoma
Explanation: ***Myxoedema*** - **Myxoedema** is associated with severe **hypothyroidism** and is characterized by non-pitting edema due to the accumulation of **glycosaminoglycans** in tissues. - While it can cause facial puffiness, it is **least likely** to cause **proptosis** as there is no direct mechanism for an increase in orbital contents to push the eye forward. *Grave's disease* - **Grave's disease** is the most common cause of **proptosis** due to **immune-mediated inflammation** and accumulation of **glycosaminoglycans** and fat in the orbital tissues, leading to expansion. - This condition specifically affects the **extraocular muscles** and fat, causing bulging of the eyes. *Sarcoidosis* - **Orbital sarcoidosis** can cause **proptosis** due to the formation of **granulomas** within the orbit, leading to mass effect and inflammation. - It can affect any part of the eye and orbit, leading to varied clinical presentations including axial or non-axial globe displacement. *Pituitary adenoma* - A **pituitary adenoma** itself does not directly cause proptosis as it is located in the **sella turcica**, posterior to the orbits. - However, **large adenomas** can cause proptosis indirectly if they invade the **cavernous sinus** and obstruct venous return from the orbit, or if they are associated with **acromegaly**, which can lead to bony overgrowth of the orbital structures and surrounding tissues.
Question 3: Which condition is commonly associated with Disseminated Intravascular Coagulation (D.I.C.)?
- A. Acute myelomonocytic leukemia
- B. Chronic myeloid leukemia
- C. Autoimmune hemolytic anemia
- D. Acute promyelocytic leukemia (Correct Answer)
Explanation: ***Acute promyelocytic leukemia*** - **Disseminated Intravascular Coagulation (D.I.C.)** is commonly associated with acute promyelocytic leukemia due to the release of **tissue factor** from promyelocytes [1]. - Patients typically present with **severe bleeding** and coagulopathy [1], driven by the rapid proliferation of these abnormal cells. *Acute myelomonocytic leukemia* - While this type of leukemia presents with myelomonocytic features, it is less frequently associated with **D.I.C.** compared to acute promyelocytic leukemia. - This condition is often characterized by **monocytic infiltration** but does not typically cause the severe coagulopathy associated with D.I.C. *Autoimmune hemolytic anemia* - This condition causes **hemolysis** due to antibodies but is mainly associated with **anemia**, not a coagulation disorder like D.I.C. - **D.I.C.** involves widespread **consumption coagulopathy** [1], which is not a feature of autoimmune hemolytic anemia. *CMC* - CMC refers to **Chronic Myeloid Leukemia**, which does not commonly lead to **D.I.C.** and presents primarily with splenomegaly and **chronic symptoms**. - The coagulation profile in CMC tends to be stable, with no link to the acute coagulopathy seen in D.I.C.
Question 4: Hyperviscosity is seen in all, except:
- A. Cryoglobulinemia
- B. MGUS (Correct Answer)
- C. Lymphoma
- D. Multiple myeloma
Explanation: ***MGUS*** - **Monoclonal gammopathy of undetermined significance (MGUS)** is characterized by a low concentration of **M-protein** in the blood, which is generally not sufficient to cause significant hyperviscosity. - While it is a precursor to multiple myeloma, the lack of end-organ damage and lower M-protein levels mean hyperviscosity syndrome is **rarely seen** in MGUS. *Cryoglobulinemia* - **Cryoglobulins** are immunoglobulins that precipitate at cold temperatures, leading to increased blood viscosity, especially in cooler body parts. - This precipitation can cause symptoms like **Raynaud's phenomenon**, **cutaneous vasculitis**, and **neuropathy**. *Lymphoma* - Certain lymphomas, particularly those producing **large amounts of monoclonal immunoglobulins** (e.g., Waldenström macroglobulinemia, which is a low-grade B-cell lymphoma), can lead to hyperviscosity syndrome [1]. - The elevated protein levels directly increase the **viscosity of the blood**, impairing blood flow [1]. *Multiple myeloma* - Multiple myeloma involves the overproduction of **monoclonal immunoglobulins (M-proteins)** by plasma cells, leading to high protein concentrations in the blood [1]. - These elevated proteins significantly increase blood viscosity, contributing to the symptoms of **hyperviscosity syndrome**, such as visual disturbances, neurological symptoms, and bleeding diathesis [1].
Question 5: Parachute lesions are associated with which of the following conditions?
- A. Eale's disease (Correct Answer)
- B. Diabetes
- C. Sickle cell anemia
- D. None of the options
Explanation: ***Eale's disease*** - **Eale's disease** is an idiopathic retinal perivasculitis affecting young males, characterized by recurrent vitreous hemorrhages. - In the context of this question (NEET-2013), the term "parachute lesions" refers to the characteristic pattern of hemorrhages seen in Eale's disease. - The disease features retinal periphlebitis, capillary non-perfusion, and neovascularization leading to vitreous hemorrhage. - **Note:** The term "parachute hemorrhages" in broader ophthalmology typically describes preretinal/subhyaloid hemorrhages that settle inferiorly (boat-shaped), more commonly seen in proliferative diabetic retinopathy. *Diabetes* - **Diabetic retinopathy** presents with microaneurysms, dot-blot hemorrhages, hard exudates, cotton-wool spots, and neovascularization. - While proliferative diabetic retinopathy can cause preretinal "parachute-shaped" hemorrhages (boat-shaped hemorrhages that settle inferiorly), this is not the association being tested in this NEET-2013 question. - The specific context of this exam question associates the term with Eale's disease. *Sickle cell anemia* - **Sickle cell retinopathy** features characteristic sea-fan neovascularization in the peripheral retina. - Can cause salmon-patch hemorrhages, black sunburst lesions, and angioid streaks. - While vitreous hemorrhage can occur, "parachute lesions" is not standard terminology for sickle cell retinopathy manifestations. *None of the options* - This option is incorrect because **Eale's disease** is the correct answer according to the NEET-2013 exam key.
Question 6: Retinitis pigmentosa is a feature of all except which of the following?
- A. NARP
- B. Refsum's disease
- C. Hallervorden-Spatz disease (Correct Answer)
- D. Abetalipoproteinemia
Explanation: ***Hallervorden-Spatz disease*** - Also known as **Pantothenate kinase-associated neurodegeneration (PKAN)**, this disorder primarily causes **extrapyramidal symptoms** such as dystonia and parkinsonism due to iron accumulation in the basal ganglia. - While it is a neurodegenerative disorder affecting the brain, **retinitis pigmentosa** is not a characteristic feature of Hallervorden-Spatz disease. *Refsum's disease* - This is an **autosomal recessive peroxisomal disorder** characterized by the accumulation of **phytanic acid**, which is toxic to various tissues. - **Retinitis pigmentosa** is a classic symptom, often presenting with night blindness and progressive vision loss, along with **ataxia**, **polyneuropathy**, and **ichthyosis**. *NARP* - **NARP (Neuropathy, Ataxia, Retinitis Pigmentosa)** is a rare mitochondrial disorder caused by mutations in the **MT-ATP6 gene**, leading to energy production deficits. - **Retinitis pigmentosa** is a core feature, contributing to visual impairment, alongside **sensory neuropathy**, **ataxia**, and **developmental delay**. *Abetalipoproteinemia* - This is an **autosomal recessive disorder** characterized by the inability to synthesize **apolipoprotein B**, essential for the formation of chylomicrons and VLDL, leading to severe **malabsorption of fat-soluble vitamins** (A, D, E, K). - Prolonged deficiency of **vitamin E** can result in progressive neurological dysfunction, including **ataxia** and **retinitis pigmentosa**, due to oxidative damage to photoreceptors and nervous tissue.
Question 7: Match List-I with List-II and select the correct answer using the code given below the Lists:
- A. A→3 B→2 C→1 D→4
- B. A→3 B→1 C→4 D→2
- C. A→1 B→3 C→2 D→4 (Correct Answer)
- D. A→4 B→2 C→3 D→1
Explanation: ***A→1 B→3 C→2 D→4*** - This option correctly matches the occupational exposures with their characteristic health effects based on the lists provided. - **Lead poisoning** classically causes **wrist drop** due to radial nerve palsy, a hallmark neurological manifestation. - **Coal tar** exposure is associated with **skin cancer** (especially scrotal cancer in historical chimney sweeps) and **lung cancer** in occupational settings. - **Aniline dye** (particularly β-naphthylamine) is a well-established cause of **bladder cancer** in dye industry workers. - **Benzol (Benzene)** is notorious for causing **hematological disorders** including aplastic anemia, pancytopenia, and leukemia. *A→3 B→2 C→1 D→4* - This option incorrectly associates the exposures with their health effects. - The matching does not align with established occupational health associations. *A→3 B→1 C→4 D→2* - This option incorrectly associates the exposures with their health effects. - The matching does not align with established occupational health associations. *A→4 B→2 C→3 D→1* - This option incorrectly associates the exposures with their health effects. - Although lead can cause anemia, its most characteristic neurological effect is **wrist drop**, which is a diagnostic hallmark. Benzol (benzene) is primarily associated with hematological disorders, not neurological manifestations like wrist drop.
Question 8: All of the following may be used to differentiate Central Retinal Venous Occlusion (CRVO) from Ocular Ischemic Syndrome due to Carotid Artery Stenosis, EXCEPT:
- A. Dilated Retinal Vein (Correct Answer)
- B. Tortuous Retinal Vein
- C. Retinal Artery Pressure
- D. Ophthalmodynamometry
Explanation: ***Dilated Retinal Vein*** - Both **Central Retinal Vein Occlusion (CRVO)** and **Ocular Ischemic Syndrome (OIS)** can present with dilated retinal veins. - In OIS, venodilation occurs due to reduced perfusion pressure, while in CRVO, it's a direct consequence of venous outflow obstruction. *Tortuous Retinal Vein* - **Tortuous retinal veins** are a characteristic finding in **CRVO** due to the increased resistance to blood flow and the resulting venous congestion. - While mild venodilation can occur in **OIS**, marked tortuosity is less typical as the primary pathology is arterial insufficiency, not venous outflow obstruction. *Retinal Artery Pressure* - **Retinal artery pressure** is typically **reduced** in **Ocular Ischemic Syndrome (OIS)** due to severe carotid artery stenosis, reflecting decreased arterial inflow. - In **CRVO**, retinal artery pressure usually remains normal, as the primary issue is venous outflow obstruction, not arterial supply. *Ophthalmodynamometry* - **Ophthalmodynamometry** measures the pressure required to induce pulsations or collapse of the central retinal artery, indirectly assessing **retinal artery perfusion pressure**. - This test would show **reduced pressure** in **Ocular Ischemic Syndrome (OIS)** due to compromised carotid flow, whereas it would typically be normal in patients with **CRVO**.
Question 9: Not a feature of ligneous conjunctivitis:
- A. Recurrent nature
- B. Wood-like membranes
- C. Bacterial origin (Correct Answer)
- D. Plasminogen deficiency
Explanation: ***Bacterial origin*** - **Ligneous conjunctivitis** is primarily a genetic disorder due to **plasminogen deficiency**, not a bacterial infection. - While secondary bacterial infections can occur, they are not the underlying cause or hallmark feature of this condition. *Recurrent nature* - The disease is characterized by a **recurrent nature**, with pseudomembranes frequently reforming even after surgical removal. - This tendency for recurrence underscores the underlying systemic enzymatic deficiency. *Wood-like membranes* - The hallmark clinical feature of ligneous conjunctivitis is the formation of **dense, rigid, wood-like pseudomembranes** on the conjunctiva, particularly the palpebral conjunctiva. - These membranes are composed of fibrin and other extracellular matrix components, which accumulate due to impaired fibrinolysis. *Plasminogen deficiency* - Ligneous conjunctivitis is caused by a profound deficiency or dysfunction of **plasminogen**, an enzyme crucial for fibrinolysis. - This deficiency leads to impaired breakdown of fibrin, resulting in its abnormal accumulation in various tissues, most notably the eye.
Question 10: In a patient with AIDS, what typically causes chorioretinitis?
- A. Cytomegalovirus (Correct Answer)
- B. Toxoplasma gondii
- C. Cryptococcus neoformans
- D. Histoplasma capsulatum
Explanation: **Explanation:** **1. Why Cytomegalovirus (CMV) is Correct:** Cytomegalovirus (CMV) retinitis is the **most common opportunistic ocular infection** in patients with AIDS, typically occurring when the CD4+ T-cell count falls below **50 cells/mm³**. It is a full-thickness necrotizing retinitis. The classic clinical appearance is described as **"Pizza-pie" or "Cheese and Ketchup" retinopathy**, characterized by areas of white retinal necrosis mixed with prominent retinal hemorrhages and vasculitis. **2. Why the Other Options are Incorrect:** * **Toxoplasma gondii:** While it causes chorioretinitis, it is less common than CMV in AIDS patients. It typically presents as a "headlight in the fog" appearance (focal retinitis with overlying vitritis). In AIDS, it often presents as a reactivation of a previous infection. * **Cryptococcus neoformans:** This primarily causes fungal meningitis. Ocular involvement is usually secondary to increased intracranial pressure (papilledema) or direct optic nerve infiltration, rather than primary chorioretinitis. * **Histoplasma capsulatum:** Causes "Presumed Ocular Histoplasmosis Syndrome" (POHS), characterized by "punched-out" chorioretinal scars, peripapillary atrophy, and maculopathy. It is not specifically associated with the immunosuppression levels seen in AIDS. **3. High-Yield Clinical Pearls for NEET-PG:** * **CD4 Count Threshold:** CMV Retinitis = CD4 < 50 cells/mm³. * **Drug of Choice:** **Ganciclovir** (Intravenous or Intravitreal implants). Foscarnet and Cidofovir are alternatives. * **Immune Recovery Uveitis (IRU):** A paradoxical inflammatory response occurring in AIDS patients after starting HAART as their CD4 count rises. * **Cotton Wool Spots:** These are the most common *non-infectious* ocular finding in AIDS (part of HIV microangiopathy), but they do not represent true chorioretinitis.
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