Hematology Practice Questions

Q: All of the following are true regarding Anemia of Chronic Disease, except?

Decreased ferritin. **Explanation:** Anemia of Chronic Disease (ACD), also known as Anemia of Inflammation, is primarily mediated by **Hepcidin**, an acute-phase reactant produced by the liver in response to inflammatory cytokines (especially IL-6) [1]. **Why Option B is the Correct Answer (The "Except" statement):** In ACD, **Serum Ferritin is typically increased or normal**, not decreased. Ferritin acts as an acute-phase reactant; inflammation causes the body to sequester iron within storage sites (macrophages and hepatocytes) to withhold it from potential pathogens. A **decreased ferritin** is the hallmark of **Iron Deficiency Anemia (IDA)** [1], making this the false statement regarding ACD [2]. **Analysis of Incorrect Options:** * **Option A (Decreased serum iron):** True. Hepcidin causes the degradation of ferroportin channels, preventing iron release from macrophages and absorption from the gut, leading to low circulating serum iron (hypoferremia) [1]. * **Option C (Decreased TIBC):** True. In states of chronic inflammation, the body downregulates the synthesis of Transferrin (measured as TIBC) to limit iron availability [1]. This helps differentiate ACD from IDA (where TIBC is increased) [2]. * **Option D (Increased bone marrow iron):** True. Because iron is "trapped" inside the reticuloendothelial system (macrophages) and cannot be utilized for erythropoiesis, bone marrow aspirate stained with Prussian blue shows abundant iron stores. **High-Yield Clinical Pearls for NEET-PG:** * **Pathophysiology:** High Hepcidin → Low Ferroportin → Iron Sequestration [1]. * **Morphology:** Usually Normocytic Normochromic; can become Microcytic Hypochromic in long-standing cases [1]. * **Gold Standard Diagnosis:** Bone marrow iron staining (Prussian Blue), though rarely done clinically. * **Key Differentiator:** The **Soluble Transferrin Receptor (sTfR)** index is normal in ACD but elevated in IDA [2].

Q: A patient presents with splenomegaly, low RBC count, normal WBC and platelet count. Peripheral blood smear shows tear drop cells. Repeated bone marrow aspiration is unsuccessful. What is the probable diagnosis?

Myelofibrosis. ### Explanation The clinical presentation points towards **Primary Myelofibrosis (PMF)**, a myeloproliferative neoplasm characterized by the replacement of bone marrow with collagenous connective tissue [1]. **Why Myelofibrosis is the correct answer:** 1. **Tear Drop Cells (Dacrocytes):** These are the hallmark of myelofibrosis [1]. They occur because RBCs are physically squeezed and damaged as they attempt to pass through the fibrotic marrow and the distorted vasculature of the spleen [1]. 2. **"Dry Tap" on Bone Marrow Aspiration:** The extensive fibrosis (increased reticulin/collagen) prevents the aspiration of marrow contents [1]. This is a classic board-exam finding for PMF. 3. **Splenomegaly:** Due to marrow failure, the body resorts to **Extramedullary Hematopoiesis (EMH)**, primarily in the spleen and liver, leading to significant organomegaly [1]. **Why the other options are incorrect:** * **Thalassemia:** While it can show splenomegaly and target cells, it does not cause a "dry tap" on aspiration. * **Chronic Myeloid Leukemia (CML):** Typically presents with a massively elevated WBC count (leukocytosis) and a hypercellular marrow that is easily aspirated. * **Iron Deficiency Anemia:** Characterized by microcytic hypochromic cells and pencil cells, not tear drop cells. The bone marrow is easily aspirated. **NEET-PG High-Yield Pearls:** * **Leukoerythroblastic Picture:** PMF often shows immature WBCs and nucleated RBCs on the peripheral smear [1]. * **JAK2 Mutation:** Present in approximately 50-60% of PMF cases [1]. * **Silver Stain:** Used on bone marrow biopsy to visualize increased reticulin fibers [1]. * **Differential for Dry Tap:** Remember the mnemonic **"M-3"**: **M**yelofibrosis, **M**etastatic secondary deposits, and **M**yeloid leukemias (like Hairy Cell Leukemia).

Q: An elevated White Blood Cell (WBC) count is seen in all of the following conditions except?

Typhoid. The correct answer is **Typhoid (Option A)**. In clinical hematology, most bacterial infections cause leukocytosis (elevated WBC count) [3]. However, Typhoid fever (Enteric fever) is a classic exception. It typically presents with **leukopenia** (decreased WBC count) or a normal WBC count with a relative lymphocytosis. This occurs due to the sequestration of leukocytes in the spleen and bone marrow depression by the *Salmonella typhi* endotoxin [2]. **Analysis of other options:** * **Pertussis (Option B):** Unlike most bacterial infections that cause neutrophilia, *Bordetella pertussis* causes a marked **lymphocytic leukocytosis** [1]. This is due to "pertussis toxin," which prevents lymphocytes from leaving the blood and entering the lymph nodes. * **Lymphoreticular malignancy (Option C):** Conditions like Chronic Lymphocytic Leukemia (CLL) or lymphomas in the leukemic phase are characterized by the uncontrolled proliferation of white cells, leading to significantly elevated WBC counts [1]. * **Tuberculosis (Option D):** TB is a chronic granulomatous infection that typically presents with mild to moderate leukocytosis, often with an increase in monocytes or lymphocytes [1], [3]. In cases of miliary TB, a "leukemoid reaction" (WBC >50,000/µL) can sometimes occur. **High-Yield Clinical Pearls for NEET-PG:** * **Leukopenia in Infections:** Common causes include Typhoid [2], Brucellosis, Kala-azar, and viral infections (like Dengue or HIV). * **Eosinopenia:** A characteristic finding in the early stages of Typhoid fever (disappearance of eosinophils). * **Leukemoid Reaction:** Defined as a reactive increase in WBC count >50,000/µL. It is commonly seen in severe infections (Sepsis, TB) [3] or acute hemolysis, mimicking leukemia but with a high Leukocyte Alkaline Phosphatase (LAP) score.

Q: Leukopenia occurs in which of the following conditions, except?

Bacterial endocarditis. **Explanation:** The correct answer is **D. Bacterial endocarditis**. **1. Understanding the Concept:** Leukopenia (a decrease in the total white blood cell count, typically <4,000/mm³) is a common feature of many viral and specific bacterial infections. However, **Infective Endocarditis (IE)** is a systemic bacterial infection that typically triggers a **leukocytosis** (elevated WBC count) with a neutrophil predominance, as the body attempts to fight the persistent intravascular vegetation [2]. In chronic cases of IE, the WBC count may be normal, but it is almost never characterized by leukopenia. **2. Analysis of Incorrect Options:** * **A & B (Measles and Viral Hepatitis):** Most viral infections (except for a few like Rabies or HTLV-1) cause leukopenia [1]. This occurs due to the redistribution of lymphocytes, bone marrow suppression, or increased peripheral destruction of cells during the viremic phase. * **C (Typhoid/Enteric Fever):** This is a high-yield "classic" exception in bacteriology. While most acute bacterial infections cause leukocytosis, **Typhoid** typically presents with **leukopenia and relative bradycardia** (Faget’s sign). This is due to the invasion of the reticuloendothelial system and bone marrow suppression by *Salmonella typhi*. **3. High-Yield Clinical Pearls for NEET-PG:** * **Bacterial infections causing Leukopenia:** Typhoid, Brucellosis, Miliary Tuberculosis, and overwhelming Sepsis (due to marrow exhaustion). * **Viral infections causing Leukocytosis:** Infectious Mononucleosis (EBV) often presents with high lymphocyte counts (atypical lymphocytes/Downey cells). * **Infective Endocarditis Triad:** Fever, new-onset murmur, and anemia (usually Normocytic Normochromic). Leukocytosis is common but not universal [2].

Q: What characterizes HbH disease?

Deletion of three alpha chain genes. **Explanation:** **1. Why Option A is Correct:** Alpha-thalassemia is primarily caused by the **deletion** of alpha-globin genes located on chromosome 16. Normally, an individual has four alpha genes ($\alpha\alpha/\alpha\alpha$). **HbH disease** occurs when **three out of the four** alpha genes are deleted ($-\alpha/--$). The severe deficiency of alpha chains leads to an excess of beta ($\beta$) chains. These excess beta chains aggregate to form tetramers ($\beta_4$), known as **Hemoglobin H**. HbH is unstable, leads to Heinz body formation, and causes a moderate to severe microcytic hypochromic anemia with splenomegaly. **2. Why the Other Options are Incorrect:** * **Option B & C:** Alpha and Beta thalassemias are distinct genetic defects [1], [2]. While co-inheritance can occur, the classic definition of HbH disease specifically refers to the isolated deficiency of alpha chains. Deletion of beta genes leads to Beta-thalassemia, not HbH [3]. * **Option D:** The deletion of all **four alpha genes** ($--/--$) results in **Hb Barts** ($\gamma_4$). This condition is known as **Hydrops Fetalis**, which is usually fatal in utero or shortly after birth because Hb Barts has an extremely high affinity for oxygen and cannot deliver it to tissues. **3. NEET-PG High-Yield Pearls:** * **Peripheral Smear:** Shows "Golf ball" appearance of RBCs when stained with Supravital stains (Brilliant Cresyl Blue) due to precipitated HbH. * **Genetics:** Most common in Southeast Asian and Mediterranean populations. * **Classification:** * 1 gene deleted: Silent carrier. * 2 genes deleted: Alpha-thalassemia trait (mild anemia). * 3 genes deleted: HbH disease. * 4 genes deleted: Hb Barts/Hydrops Fetalis. * **Electrophoresis:** HbH migrates faster than HbA on alkaline electrophoresis (Fast-moving band) [1].

Q: What is the most common presentation of sickle cell anemia?

Fever. **Explanation:** In the context of Sickle Cell Anemia (SCA), **Fever** is considered the most common clinical presentation, particularly in the pediatric population. While SCA is characterized by vaso-occlusive events, fever is the most frequent reason for hospital admission and medical evaluation. This is primarily due to the state of **functional hyposplenism** (caused by repeated splenic infarcts), which renders patients highly susceptible to infections by encapsulated organisms like *Streptococcus pneumoniae* and *Haemophilus influenzae* [1]. **Analysis of Options:** * **Bone Pain (Vaso-occlusive Crisis):** This is the most common *symptom* of a sickle cell crisis and the hallmark of the disease [1]. However, statistically, febrile episodes (often associated with underlying infection or acute chest syndrome) occur more frequently as an initial presentation in clinical settings. * **Priapism:** This is a well-known complication involving a painful, persistent erection due to sickling in the corpora cavernosa [1]. While high-yield for exams, it occurs in only about 30-40% of male patients and is not the "most common" presentation. * **Splenomegaly:** While common in early childhood, repeated infarctions lead to **autosplenectomy** (shrunken, fibrotic spleen) by adulthood [1]. Therefore, it is a transient finding rather than the most common presentation across the disease spectrum. **NEET-PG High-Yield Pearls:** * **Most common cause of death in children:** Sepsis (Streptococcus pneumoniae). * **Most common cause of death in adults:** Acute Chest Syndrome (ACS). * **Osteomyelitis in SCA:** While *Staphylococcus aureus* is the most common cause of osteomyelitis generally, **Salmonella** is uniquely associated with and highly characteristic of SCA patients. * **First clinical sign:** Dactylitis (Hand-foot syndrome) usually appearing between 6 months to 2 years of age.

Q: Which of the following statements regarding POEMS syndrome are true or false? a) Organomegaly is described as a feature but rare in POEMS syndrome b) Patients usually have a severe, progressive sensorimotor polyneuropathy c) Type 2 diabetes mellitus occurs in about one-third of patients d) Amenorrhea in women and impotence and gynecomastia in men is seen e) The lymphadenopathy although rare, resembles Castleman's disease histologically

a-False; b, c, d, e-True. **POEMS Syndrome** is a rare multisystemic paraneoplastic disorder associated with an underlying plasma cell dyscrasia. The acronym stands for **P**olyneuropathy, **O**rganomegaly, **E**ndocrinopathy, **M**onoclonal protein, and **S**kin changes. ### **Analysis of Statements:** * **a) Organomegaly (False):** Contrary to the statement, organomegaly (hepatomegaly, splenomegaly, or lymphadenopathy) is a **common** feature, occurring in approximately 65-90% of cases, not rare. * **b) Polyneuropathy (True):** This is the hallmark of the syndrome. It is typically a symmetric, progressive, and severe sensorimotor polyneuropathy, often mimicking CIDP but with more axonal damage. * **c) Diabetes Mellitus (True):** Endocrinopathy is a major criterion. Type 2 DM occurs in about 1/3rd of patients. Other common issues include hypothyroidism and adrenal insufficiency. * **d) Gonadal Dysfunction (True):** Hypogonadism is the most common endocrinopathy. It manifests as amenorrhea in females and impotence/gynecomastia in males. * **e) Castleman’s Disease (True):** Lymph node biopsy in POEMS often shows the hyaline-vascular variant of Castleman’s disease. ### **High-Yield Clinical Pearls for NEET-PG:** * **Diagnostic Criteria:** Mandatory criteria include **Polyneuropathy** and **Monoclonal plasma cell proliferative disorder** (usually lambda light chain). * **VEGF:** Elevated serum Vascular Endothelial Growth Factor (VEGF) is a highly sensitive and specific marker for diagnosis and monitoring. * **Sclerotic Bone Lesions:** Unlike Multiple Myeloma (lytic lesions), POEMS is associated with **osteosclerotic** lesions. * **Skin Changes:** Look for hyperpigmentation, hypertrichosis, and glomeruloid hemangiomas. * **Treatment:** Targeted at the plasma cell clone (Radiation for localized lesions; Autologous Stem Cell Transplant for systemic disease).

Q: Which of the following is NOT a component of cryoprecipitate?

Factor V. 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 remembering its components is high-yield for NEET-PG. [2, 4] **Why Factor V is the correct answer:** Factor V is a **labile clotting factor** found in Fresh Frozen Plasma (FFP) but is **not** concentrated in cryoprecipitate. Cryoprecipitate specifically contains five main components: Factor VIII, Von Willebrand Factor (vWF), Fibrinogen (Factor I), Factor XIII, and Fibronectin. **Analysis of Incorrect Options:** * **Factor I (Fibrinogen):** This is the most abundant component of cryoprecipitate (approx. 150–250 mg per unit). It is the primary reason cryoprecipitate is used in clinical practice (e.g., in DIC or massive hemorrhage). [2] * **Factor VIII:** Cryoprecipitate contains significant amounts of Factor VIII (anti-hemophilic factor), making it a historical treatment for Hemophilia A. * **Von Willebrand Factor (vWF):** Cryoprecipitate is rich in vWF, which is why it is used to treat von Willebrand disease when specific concentrates are unavailable. **High-Yield Clinical Pearls for NEET-PG:** 1. **Indications:** The most common indication for cryoprecipitate today is **hypofibrinogenemia** (target fibrinogen level >100 mg/dL). [2] 2. **Storage:** It is stored at **-18°C or colder** and has a shelf life of 1 year. Once thawed, it must be used within 4–6 hours. 3. **Dosage:** One unit of cryoprecipitate typically raises the fibrinogen level by 5–10 mg/dL in an average adult. 4. **Mnemonic:** To remember the contents, use **"1, 8, 13, vWF, and Fibronectin."** (Note: Factor V is notably absent).

Question 1

All of the following are true regarding Anemia of Chronic Disease, except?

Accepted Answer

Decreased ferritin

Answer

Decreased serum iron

Answer

Decreased total iron-binding capacity

Answer

Increased bone marrow iron

Question 2

Which of the following conditions is characterized by an incoagulable state?

Accepted Answer

Snake envenomation

Answer

Acute-promyelocytic leukemia

Answer

Abruptio placenta

Answer

Heparin overdose

Question 3

A patient presents with splenomegaly, low RBC count, normal WBC and platelet count. Peripheral blood smear shows tear drop cells. Repeated bone marrow aspiration is unsuccessful. What is the probable diagnosis?

Accepted Answer

Myelofibrosis

Answer

Thalassemia

Answer

Chronic myeloid leukemia

Answer

Iron deficiency anemia

Question 4

An elevated White Blood Cell (WBC) count is seen in all of the following conditions except?

Accepted Answer

Typhoid

Answer

Pertussis

Answer

Lymphoreticular malignancy

Answer

Tuberculosis

Question 5

Which of the following is NOT related to the treatment of thalassemia?

Accepted Answer

Routine iron therapy

Answer

Fresh blood transfusion

Answer

Folic acid supplementation

Answer

Deferoxamine improves pregnancy outcome

Question 6

Leukopenia occurs in which of the following conditions, except?

Accepted Answer

Bacterial endocarditis

Answer

Measles

Answer

Viral hepatitis

Answer

Typhoid

Question 7

What characterizes HbH disease?

Accepted Answer

Deletion of three alpha chain genes

Answer

Deletion of three alpha chains and one beta chain genes

Answer

Deletion of two alpha and two beta chain genes

Answer

Deletion of four alpha chain genes

Question 8

What is the most common presentation of sickle cell anemia?

Accepted Answer

Fever

Answer

Priapism

Answer

Bone pain

Answer

Splenomegaly

Question 9

Which of the following statements regarding POEMS syndrome are true or false? a) Organomegaly is described as a feature but rare in POEMS syndrome b) Patients usually have a severe, progressive sensorimotor polyneuropathy c) Type 2 diabetes mellitus occurs in about one-third of patients d) Amenorrhea in women and impotence and gynecomastia in men is seen e) The lymphadenopathy although rare, resembles Castleman's disease histologically

Accepted Answer

a-False; b, c, d, e-True

Answer

a, b-False; c, d, e-True

Answer

a, e-True; b, c, d-False

Answer

a, b, e-True; c, d-False

Question 10

Which of the following is NOT a component of cryoprecipitate?

Accepted Answer

Factor V

Answer

Von Willebrand factor

Answer

Factor I

Answer

Factor VIII

Hematology — MCQs

Hematology — MCQs

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