Hematology Practice Questions

Q: Coomb's positive hemolytic anemia is seen in all except?

Alcoholic cirrhosis. The Coombs test (Antiglobulin test) detects antibodies or complement proteins attached to the surface of red blood cells. A positive result indicates **Autoimmune Hemolytic Anemia (AIHA)** [1]. **1. Why Alcoholic Cirrhosis is the correct answer:** Hemolytic anemia in alcoholic cirrhosis is typically **Coombs-negative**. It is primarily caused by **Zieve’s Syndrome** (triad of alcohol consumption, hemolytic anemia, and hyperlipidemia) or **Spur Cell Anemia**. In these cases, hemolysis occurs due to metabolic disturbances and changes in the RBC membrane lipid composition (acanthocytosis), leading to splenic sequestration, rather than an antibody-mediated process. **2. Why the other options are incorrect:** * **Chronic Active Hepatitis (Autoimmune Hepatitis):** This is a classic autoimmune condition frequently associated with other systemic autoimmune phenomena, including Warm-type AIHA (Coombs positive) [1]. * **Primary Biliary Cholangitis (PBC) & Primary Sclerosing Cholangitis (PSC):** Both are chronic cholestatic liver diseases with an underlying autoimmune pathophysiology. They are known to be associated with various extrahepatic autoimmune disorders, including AIHA, making them Coombs positive. **Clinical Pearls for NEET-PG:** * **Warm AIHA (IgG):** Associated with SLE, CLL, and drugs like α-methyldopa [1]. * **Cold AIHA (IgM):** Associated with *Mycoplasma pneumoniae* and Infectious Mononucleosis [1] [2]. * **Spur Cell Anemia:** A poor prognostic sign in end-stage liver disease; it is non-immune (Coombs negative). * **Zieve’s Syndrome:** Characterized by rapid improvement in hemolysis upon alcohol cessation.

Q: Which antibody class is primarily involved in Idiopathic Thrombocytopenic Purpura (ITP)?

IgG. **Explanation:** **Idiopathic Thrombocytopenic Purpura (ITP)**, now more commonly referred to as Immune Thrombocytopenic Purpura, is an acquired autoimmune disorder characterized by isolated thrombocytopenia [2]. **Why IgG is Correct:** The pathogenesis of ITP involves the production of **autoantibodies (predominantly of the IgG class)** directed against platelet surface antigens, most commonly the **GPIIb/IIIa** or **GPIb/IX** complexes. These IgG-coated platelets are recognized by the Fc receptors on splenic macrophages, leading to their premature sequestration and destruction in the **spleen**. **Why other options are incorrect:** * **IgM:** While IgM is the first antibody produced in a primary immune response and is involved in Cold Agglutinin Disease [1], it is not the primary mediator of platelet destruction in ITP. * **IgE:** This antibody class is associated with Type I hypersensitivity reactions (allergies, asthma) and parasitic infections, not autoimmune platelet destruction [2]. * **IgD:** Found on the surface of B-cells, its precise physiological function is less defined, but it plays no role in the pathogenesis of ITP. **High-Yield Clinical Pearls for NEET-PG:** * **Site of Destruction:** The **Spleen** is the primary site of both antibody production and platelet destruction in ITP. * **Bone Marrow Findings:** Characterized by **increased or normal megakaryocytes** (a compensatory response to peripheral destruction). * **First-line Treatment:** Corticosteroids (e.g., Prednisolone). In emergencies or prior to surgery, IVIG (Intravenous Immunoglobulin) is used to "clog" splenic Fc receptors [3]. * **Splenectomy:** Indicated in chronic/refractory cases as it removes the primary site of destruction.

Q: A 60-year-old man presented with fatigue, weight loss, and heaviness in the left hypochondrium for 6 months. The hemogram showed Hb 10 g/dL, TLC 5 lakhs/mm³, platelet count 4 lakhs/mm³, DLC: neutrophils 55%, lymphocytes 4%, monocytes 2%, basophils 6%, metamyelocytes 10%, myelocytes 18%, promyelocytes 2%, and blasts 3%. What is the most likely cytogenetic abnormality in this case?

t(9;22). ### Explanation **Diagnosis: Chronic Myeloid Leukemia (CML)** The clinical presentation of a 60-year-old male with massive splenomegaly (heaviness in the left hypochondrium) and a massive leukocytosis (TLC 5 lakhs/mm³) is classic for CML [2]. The differential leukocyte count (DLC) shows a **"myelocyte bulge"** (predominance of myelocytes and metamyelocytes) and **basophilia (6%)**, which are hallmark features of CML in the chronic phase. **1. Why t(9;22) is correct:** The cytogenetic hallmark of CML is the **Philadelphia (Ph) chromosome**, resulting from a reciprocal translocation between chromosomes 9 and 22, **t(9;22)(q34;q11)** [2]. This creates the *BCR-ABL1* fusion gene, which encodes a constitutive tyrosine kinase responsible for uncontrolled myeloid proliferation [2]. **2. Why the other options are incorrect:** * **t(1;21):** This is not a standard translocation associated with common leukemias. * **t(15;17):** This is the characteristic translocation for **Acute Promyelocytic Leukemia (APL - AML M3)**, involving the *PML-RARA* fusion [1]. Patients typically present with DIC and a high percentage of promyelocytes with Auer rods. * **Trisomy 21:** Associated with Down Syndrome, which carries an increased risk of **Acute Megakaryoblastic Leukemia (AML M7)** and Transient Myeloproliferative Disorder (TMD) in neonates. **Clinical Pearls for NEET-PG:** * **LAP Score:** Leukocyte Alkaline Phosphatase (LAP) score is characteristically **decreased** in CML (helps differentiate it from a Leukemoid reaction where it is increased). * **Basophilia:** The presence of increased basophils in a peripheral smear is a strong clue for a Myeloproliferative Neoplasm (MPN), specifically CML. * **Treatment of Choice:** Tyrosine Kinase Inhibitors (TKIs) like **Imatinib** [2]. * **Blast Crisis:** Defined as ≥20% blasts in blood or bone marrow.

Q: A 19-year-old man presents with a swollen and painful knee after an injury while playing football. He has no history of bleeding disorders. Arthrocentesis of the knee revealed hemarthrosis. Investigations show: Platelets 170,000/mL, PT normal, PTT elevated, bleeding time normal, factor VIII normal, factor IX reduced, and ristocetin cofactor assay normal. What is the most likely diagnosis for this patient's bleeding disorder?

hemophilia B. The clinical presentation of a young male with **hemarthrosis** (bleeding into joints) following minor trauma is a classic hallmark of a coagulation factor deficiency [1]. **1. Why Hemophilia B is correct:** The laboratory profile provided is the key to the diagnosis: * **Elevated PTT (aPTT):** Indicates a defect in the intrinsic pathway (Factors VIII, IX, XI, or XII). * **Normal PT and Bleeding Time:** Rules out extrinsic pathway defects and platelet/vessel wall disorders [1]. * **Factor IX reduced:** This is the definitive finding. Hemophilia B (Christmas Disease) is an X-linked recessive disorder caused by a deficiency of Factor IX [1]. **2. Why other options are incorrect:** * **von Willebrand Disease (vWD):** While it can cause an elevated aPTT (due to low Factor VIII), it typically presents with mucosal bleeding (epistaxis, menorrhagia) and an **abnormal Ristocetin Cofactor Assay**. Hemarthrosis is rare except in Type 3 vWD [2]. * **Hemophilia A:** This also presents with hemarthrosis and elevated aPTT, but it is caused by a deficiency of **Factor VIII**. The question explicitly states Factor VIII levels are normal [1]. * **Thrombotic Thrombocytopenic Purpura (TTP):** This is a microangiopathic hemolytic anemia characterized by the pentad of fever, anemia, thrombocytopenia, renal failure, and neurological symptoms. It would show a **low platelet count**, which is normal in this patient. **Clinical Pearls for NEET-PG:** * **Inheritance:** Both Hemophilia A and B are **X-linked recessive** (affecting males) [1]. * **Mixing Study:** If aPTT is prolonged, a mixing study is done. If it corrects, it indicates a factor deficiency; if not, it suggests an inhibitor. * **Treatment:** Hemophilia B is treated with recombinant Factor IX concentrate. Fresh Frozen Plasma (FFP) is used if specific concentrates are unavailable.

Q: Evans syndrome refers to the presence of which of the following hematologic changes?

Autoimmune thrombocytopenia in autoimmune hemolytic anemia. **Explanation:** **Evans Syndrome** is a rare autoimmune disorder characterized by the simultaneous or sequential development of **Autoimmune Hemolytic Anemia (AIHA)** and **Immune Thrombocytopenic Purpura (ITP)** [1]. In some cases, autoimmune neutropenia may also be present. 1. **Why Option A is Correct:** The pathophysiology involves the production of autoantibodies (usually IgG) directed against antigens on both red blood cells and platelets [1]. This leads to their premature destruction in the spleen. It is often associated with underlying conditions like SLE, CLL, or Primary Immunodeficiency (e.g., CVID) [1]. 2. **Why the Other Options are Incorrect:** * **Option B:** DIC involves a consumptive coagulopathy with low fibrinogen and elevated D-dimer, triggered by systemic inflammation or sepsis, not a primary autoimmune attack on RBCs and platelets [1]. * **Option C:** Thrombocytopenia in Hairy Cell Leukemia is typically due to bone marrow infiltration and hypersplenism, not an isolated autoimmune mechanism. * **Option D:** Evans syndrome is defined by a *deficiency* (thrombocytopenia), not an excess (thrombocytosis). **High-Yield Clinical Pearls for NEET-PG:** * **Coombs Test:** The Direct Antiglobulin Test (DAT) is typically **positive** (usually for IgG) [1]. * **Treatment:** First-line therapy is **Corticosteroids**. Refractory cases may require Rituximab, IVIG, or Splenectomy. * **Key Association:** Always screen patients with Evans Syndrome for **Systemic Lupus Erythematosus (SLE)**, as it is a common secondary cause [1]. * **Peripheral Smear:** Look for **Spherocytes** (due to AIHA) and a lack of platelets [1]. Unlike TTP, schistocytes are absent.

Q: The Donath-Landsteiner phenomenon is characteristic of which condition?

Paroxysmal Cold Hemoglobinuria. **Explanation:** The **Donath-Landsteiner phenomenon** is the hallmark of **Paroxysmal Cold Hemoglobinuria (PCH)**. It involves a unique **biphasic IgG antibody** (an anti-P autoantibody) that binds to red blood cells (RBCs) at low temperatures (cold phase) and fixes complement. When the blood subsequently warms to 37°C (warm phase), the complement cascade is activated, leading to intravascular hemolysis [1]. **Analysis of Options:** * **Paroxysmal Cold Hemoglobinuria (PCH):** Correct. Historically associated with late-stage syphilis, it is now more commonly seen as a self-limiting post-viral syndrome in children (e.g., after URI, measles, or mumps). * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** Incorrect. PNH is caused by an acquired mutation in the *PIGA* gene leading to a deficiency of GPI-anchored proteins (CD55/CD59) [2]. The screening test is Flow Cytometry (formerly Ham’s test or Sucrose Lysis test). * **Waldenstrom’s Macroglobulinemia:** Incorrect. This is a B-cell lymphoproliferative disorder characterized by monoclonal IgM. While it can be associated with Cold Agglutinin Disease (IgM-mediated), it does not involve the biphasic Donath-Landsteiner antibody [1]. * **Malaria:** Incorrect. While malaria causes episodic hemolysis and "Blackwater fever," the mechanism is direct parasitic rupture of RBCs [2], not a biphasic autoantibody. **NEET-PG High-Yield Pearls:** * **Antibody Type:** PCH involves **IgG** (unusual for "cold" disorders, which are typically IgM). * **Specificity:** The antibody is specific for the **P-antigen** on RBCs. * **Clinical Presentation:** Sudden onset of hemoglobinuria (dark urine) following cold exposure, often accompanied by fever and chills. * **Diagnosis:** The **Donath-Landsteiner Test** is the gold standard.

Q: A 26-year-old woman presents with severe menorrhagia. She reports that both her father and sister have a bleeding disorder. What is the most likely diagnosis, given an autosomal-dominant mode of inheritance for the hemostatic disorder?

von Willebrand's disease. The patient presents with **menorrhagia** (mucocutaneous bleeding) and a strong family history involving both genders (father and sister), which points toward an **Autosomal Dominant (AD)** inheritance pattern. [1] **1. Why von Willebrand’s Disease (vWD) is correct:** vWD is the most common inherited bleeding disorder. It typically presents with mucocutaneous bleeding (epistaxis, menorrhagia, gingival bleeding). [1] Most types (specifically Type 1 and Type 2A, 2B, and 2M) follow an **Autosomal Dominant** inheritance. Since it affects both males and females equally, it perfectly matches the clinical scenario. **2. Why the other options are incorrect:** * **Factor VIII (Hemophilia A) and Factor IX (Hemophilia B) deficiency:** These are **X-linked recessive** disorders. They primarily affect males, while females are typically asymptomatic carriers. They usually present with deep-seated bleeds (hemarthrosis/muscle hematomas) rather than mucosal bleeding. [1] * **Factor X deficiency:** This is a rare coagulation disorder that follows an **Autosomal Recessive** inheritance pattern, making it less likely in a multi-generational family history unless consanguinity is present. **Clinical Pearls for NEET-PG:** * **Screening Tests in vWD:** Prolonged Bleeding Time (BT) and often a prolonged Activated Partial Thromboplastin Time (aPTT) (due to low Factor VIII levels, as vWF stabilizes Factor VIII). [2] Platelet count is usually normal (except in Type 2B). * **Confirmatory Test:** Ristocetin Cofactor Assay (measures vWF activity). [2] * **Treatment of Choice:** Desmopressin (DDAVP) for Type 1; Factor VIII concentrates containing vWF for severe cases. [2] * **Inheritance Rule:** If a bleeding disorder affects both father and daughter, think AD (vWD); if it skips females and affects maternal uncles/nephews, think X-linked (Hemophilia).

Q: Which of the following is NOT a feature seen in hemolytic anemia?

Tear drop and Burr cells. In hemolytic anemia, the hallmark is the premature destruction of red blood cells (RBCs), leading to compensatory bone marrow activity and the release of breakdown products [1]. **Explanation of the Correct Option:** * **Option A (Tear drop and Burr cells):** These are not characteristic of hemolysis. **Tear drop cells (Dacrocytes)** are typically seen in **Myelofibrosis** (extramedullary hematopoiesis) or marrow infiltrative disorders. **Burr cells (Echinocytes)** are associated with **Uremia**, liver disease, or pyruvate kinase deficiency [2] (though the latter is hemolytic, Burr cells are not a general feature of hemolysis). The characteristic cell seen in hemolysis is the **Schistocyte** (fragmented cell) or **Spherocyte** [2]. **Explanation of Incorrect Options:** * **Option B (Reduced haptoglobin):** In intravascular hemolysis, free hemoglobin binds to haptoglobin. This complex is cleared by the liver, leading to a marked **decrease** in serum haptoglobin levels. * **Option C (Reticulocytosis):** To compensate for the loss of RBCs, the bone marrow increases production, releasing immature RBCs (reticulocytes) into the peripheral blood. * **Option D (Hemoglobinuria):** When the haptoglobin-binding capacity is saturated, free hemoglobin is filtered by the glomerulus, appearing in the urine (common in intravascular hemolysis) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Intravascular Hemolysis:** Look for Hemoglobinuria, Hemosiderinuria, and very low Haptoglobin [2]. * **Extravascular Hemolysis:** Look for Splenomegaly and Jaundice (unconjugated hyperbilirubinemia) without hemoglobinuria [1]. * **Most sensitive marker for hemolysis:** Increased **LDH** and decreased **Haptoglobin**. * **Direct Coombs Test:** Used to differentiate autoimmune hemolytic anemia from other causes.

Question 1

Coomb's positive hemolytic anemia is seen in all except?

Accepted Answer

Alcoholic cirrhosis

Answer

Chronic active hepatitis

Answer

Primary biliary cirrhosis

Answer

Primary sclerosing cholangitis

Question 2

Which of the following causes neutrophilia?

Accepted Answer

Glucocorticoids

Answer

Epinephrine

Answer

NSAIDs

Answer

Clozapine

Question 3

Which antibody class is primarily involved in Idiopathic Thrombocytopenic Purpura (ITP)?

Accepted Answer

IgG

Answer

IgM

Answer

IgE

Answer

IgD

Question 4

A 60-year-old man presented with fatigue, weight loss, and heaviness in the left hypochondrium for 6 months. The hemogram showed Hb 10 g/dL, TLC 5 lakhs/mm³, platelet count 4 lakhs/mm³, DLC: neutrophils 55%, lymphocytes 4%, monocytes 2%, basophils 6%, metamyelocytes 10%, myelocytes 18%, promyelocytes 2%, and blasts 3%. What is the most likely cytogenetic abnormality in this case?

Accepted Answer

t(9;22)

Answer

t(1;21)

Answer

t(15;17)

Answer

Trisomy 21

Question 5

A 19-year-old man presents with a swollen and painful knee after an injury while playing football. He has no history of bleeding disorders. Arthrocentesis of the knee revealed hemarthrosis. Investigations show: Platelets 170,000/mL, PT normal, PTT elevated, bleeding time normal, factor VIII normal, factor IX reduced, and ristocetin cofactor assay normal. What is the most likely diagnosis for this patient's bleeding disorder?

Accepted Answer

hemophilia B

Answer

von Willebrand disease

Answer

hemophilia A

Answer

thrombotic thrombocytopenic purpura (TTP)

Question 6

Evans syndrome refers to the presence of which of the following hematologic changes?

Accepted Answer

Autoimmune thrombocytopenia in autoimmune hemolytic anemia

Answer

Disseminated intravascular coagulation in autoimmune hemolytic anemia

Answer

Thrombocytopenia in hairy cell leukemia

Answer

Thrombocytosis in autoimmune hemolytic anemia

Question 7

The Donath-Landsteiner phenomenon is characteristic of which condition?

Accepted Answer

Paroxysmal Cold Hemoglobinuria

Answer

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Answer

Waldenstrom's Macroglobulinemia

Answer

Malaria

Question 8

A 26-year-old woman presents with severe menorrhagia. She reports that both her father and sister have a bleeding disorder. What is the most likely diagnosis, given an autosomal-dominant mode of inheritance for the hemostatic disorder?

Accepted Answer

von Willebrand's disease

Answer

Factor X deficiency

Answer

Factor VIII deficiency (hemophilia A)

Answer

Factor IX deficiency (hemophilia B)

Question 9

Which of the following is NOT a feature seen in hemolytic anemia?

Accepted Answer

Tear drop and Burr cells

Answer

Reduced haptoglobin

Answer

Reticulocytosis

Answer

Hemoglobinuria

Question 10

Which of the following is the least common feature of Multiple Myeloma?

Accepted Answer

Hyperviscosity syndrome

Answer

Bone pain

Answer

Normocytic normochromic anemia

Answer

Susceptibility to bacterial infection

Hematology — MCQs

Hematology — MCQs

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