Hematology Practice Questions

Q: A serious coagulopathy characterized by severe thrombocytopenia and hemorrhage due to platelet trapping within the tumor is called?

Kasabach-Merrit Phenomenon. ### Explanation **Kasabach-Merritt Phenomenon (KMP)** is the correct answer. It is a life-threatening hematologic condition characterized by the combination of a rapidly enlarging vascular tumor, **profound thrombocytopenia**, and consumptive coagulopathy. **1. Why it is correct:** The underlying mechanism involves **platelet trapping** and activation within the abnormal architecture of specific vascular tumors. This leads to the consumption of platelets and clotting factors (DIC-like picture), resulting in severe hemorrhage. It is most commonly associated with **Tufted Angioma** or **Kaposiform Hemangioendothelioma**, rather than common infantile hemangiomas. **2. Why other options are incorrect:** * **PHACE(S) Syndrome:** This is a neurocutaneous syndrome involving **P**osterior fossa malformations, **H**emangiomas (large, segmental), **A**rterial anomalies, **C**ardiac defects, and **E**ye anomalies. It does not inherently involve platelet sequestration. * **Kaposi’s Sarcoma:** A spindle-cell tumor caused by HHV-8, typically seen in immunocompromised patients (AIDS). While it is a vascular tumor, it does not cause the systemic consumptive coagulopathy seen in KMP. * **Hemangioma:** While common infantile hemangiomas are benign vascular tumors, they **do not** cause Kasabach-Merritt Phenomenon. KMP is specific to more aggressive vascular neoplasms like Kaposiform Hemangioendothelioma. **3. NEET-PG High-Yield Pearls:** * **Classic Triad:** Large vascular tumor + Thrombocytopenia + Microangiopathic hemolytic anemia. * **First-line Treatment:** Systemic corticosteroids and **Sirolimus** (mTOR inhibitor) are currently preferred. * **Lab Findings:** Low platelets, low fibrinogen, and elevated D-dimer (consumptive coagulopathy). * **Avoid:** Platelet transfusions should be avoided unless there is active bleeding, as they may "fuel the fire" by being trapped in the tumor.

Q: Fanconi's anemia is a type of:

Constitutional anemia. **Explanation:** **Fanconi’s Anemia (FA)** is the most common cause of **inherited (constitutional) aplastic anemia**. It is an autosomal recessive (rarely X-linked) DNA repair disorder characterized by hypersensitivity to DNA cross-linking agents. The term "constitutional" refers to the fact that the condition is congenital and arises from an underlying genetic defect present from birth, leading to progressive bone marrow failure. **Analysis of Options:** * **Option A (Correct):** It is a constitutional anemia because it is a hereditary syndrome involving pancytopenia. It typically presents between 5–10 years of age with macrocytic anemia progressing to aplastic anemia. * **Option B (Incorrect):** Hemolytic anemias involve the premature destruction of RBCs (e.g., Spherocytosis, G6PD deficiency). FA is a failure of production (hypoplasia), not destruction. * **Option C (Incorrect):** Iron deficiency is a nutritional microcytic anemia. FA is a normocytic to macrocytic anemia caused by stem cell depletion. * **Option D (Incorrect):** Autoimmune anemias (like AIHA) involve antibody-mediated destruction. While some aplastic anemias are T-cell mediated (acquired), FA is strictly genetic. **High-Yield Clinical Pearls for NEET-PG:** * **Physical Findings:** Short stature, **absent/hypoplastic thumbs**, radius abnormalities, microcephaly, and **Café-au-lait spots**. * **Gold Standard Diagnosis:** **Chromosomal Breakage Analysis** (using Mitomycin C or Diepoxybutane). * **Malignancy Risk:** High predisposition to **AML** and squamous cell carcinomas (head and neck). * **Treatment:** Hematopoietic stem cell transplant (HSCT) is the definitive cure for hematologic manifestations.

Q: A 4-year-old boy has appeared listless during the past week. He exhibits irritability when his arms or legs are touched. In the past 2 days, large ecchymoses have appeared on the right thigh and left shoulder. CBC shows hemoglobin, 9.3 g/dL; hematocrit, 28.7%; MCV, 96 mm3; platelet count, 45,000/mm3; and WBC count, 13,990/mm3. Examination of the peripheral blood smear shows blasts that lack peroxidase positive granules, but contain PAS-positive aggregates and stain positively for deoxynucleotidyl transferase negative (TdT-). Flow cytometry shows the phenotype of blasts to be CD19+, CD3-, and sIg-. Which of the following is the most likely diagnosis?

Acute lymphoblastic leukemia. **Explanation:** The clinical presentation and laboratory findings are classic for **Acute Lymphoblastic Leukemia (ALL)**, the most common malignancy in children. **1. Why the Correct Answer is Right:** * **Clinical Presentation:** The child presents with signs of bone marrow failure: anemia (listlessness), thrombocytopenia (ecchymoses), and bone pain (irritability when touched), which is caused by leukemic infiltration of the periosteum. * **Morphology & Cytochemistry:** The blasts are **PAS-positive** (characteristic of lymphoblasts) and **Peroxidase-negative** (ruling out AML). * **Immunophenotyping:** The presence of **CD19** (a B-cell marker) and the absence of surface immunoglobulin (**sIg-**) confirms a **Pre-B cell lineage**. While most ALL cases are TdT positive, the combination of CD19+, CD3-, and PAS positivity strongly points to ALL over any other option. **2. Why Incorrect Options are Wrong:** * **B. Acute Myelogenous Leukemia (AML):** AML blasts typically show **Myeloperoxidase (MPO)** positivity and may contain Auer rods. They would express myeloid markers (CD13, CD33) rather than CD19. * **C. Chronic Lymphocytic Leukemia (CLL):** CLL is a disease of the elderly (median age >60) and involves mature-appearing lymphocytes, not blasts. * **D. Chronic Myelogenous Leukemia (CML):** CML typically presents with massive splenomegaly and a spectrum of maturing myeloid cells (myelocytes, metamyelocytes) rather than a predominance of PAS-positive blasts. **3. NEET-PG High-Yield Pearls:** * **Most common childhood cancer:** ALL (Peak age: 2–5 years). * **Best Prognostic Factor:** Age 1–10 years and WBC count <50,000/mm³. * **Cytogenetics:** t(12;21) has a **good** prognosis; t(9;22) [Philadelphia chromosome] has a **poor** prognosis in ALL. * **Common Sites of Relapse:** Testes and CNS (due to the blood-brain/blood-testis barrier).

Q: What is the treatment of choice for severe ITP?

Intravenous immunoglobulin (IV gammaglobulins). **Explanation:** Immune Thrombocytopenic Purpura (ITP) in children is typically a self-limiting condition. However, in cases of **severe ITP** (defined by significant mucosal bleeding or a platelet count <10,000/mm³), rapid elevation of the platelet count is necessary to prevent life-threatening complications like intracranial hemorrhage. **Why IVIG is the Treatment of Choice:** Intravenous Immunoglobulin (IVIG) is the preferred treatment for severe ITP because it provides the **fastest rise in platelet count** (usually within 24–48 hours). It works by saturating the Fc receptors on splenic macrophages, thereby preventing the destruction of antibody-coated platelets. **Analysis of Other Options:** * **Steroids (Option D):** While steroids are the first-line treatment for *mild to moderate* symptomatic ITP, they take longer (3–7 days) to increase platelet counts compared to IVIG. * **Plasmapheresis (Option C):** This is used to remove circulating toxins or autoantibodies in conditions like TTP or Goodpasture syndrome, but it has no established role in the routine management of ITP. * **Bone Marrow Transplant (Option A):** BMT is used for bone marrow failure syndromes (e.g., Aplastic Anemia) or malignancies, not for peripheral destructive processes like ITP. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause** of isolated thrombocytopenia in an otherwise healthy child is ITP. * **Indication for treatment:** Treatment is based on **bleeding severity**, not just the absolute platelet count. * **Anti-D therapy:** Can be used as an alternative to IVIG in Rh-positive, non-splenectomized patients. * **Chronic ITP:** Defined as thrombocytopenia persisting for >12 months. Second-line agents include Rituximab or TPO-receptor agonists (Eltrombopag).

Q: A child presents with hypochromic microcytic anemia, with normal levels of free Erythrocyte Protoporphyrin. What is the most likely diagnosis?

Thalassemia. **Explanation:** The key to solving this question lies in understanding the heme synthesis pathway. **Free Erythrocyte Protoporphyrin (FEP)** is the precursor that combines with iron to form heme. If iron is unavailable or its utilization is blocked, FEP accumulates in the red blood cell. **1. Why Thalassemia is correct:** Thalassemia is a quantitative defect in **globin chain synthesis**, not heme synthesis. Since the machinery for producing protoporphyrin and the availability of iron are both normal, FEP levels remain **normal**. This is a classic biochemical marker used to differentiate Thalassemia from Iron Deficiency Anemia (IDA). **2. Why the other options are incorrect:** * **Iron Deficiency Anemia (IDA):** There is insufficient iron to bind with protoporphyrin. Consequently, "unbound" or Free Erythrocyte Protoporphyrin **increases**. * **Lead Toxicity:** Lead inhibits the enzyme *Ferrochelatase*, which prevents the insertion of iron into the protoporphyrin ring. This leads to a significant **increase** in FEP (specifically Zinc Protoporphyrin). * **Anemia of Chronic Disease (ACD):** Iron is trapped within macrophages (high ferritin) and is unavailable for erythropoiesis. Similar to IDA, the lack of available iron leads to **increased** FEP. **High-Yield Clinical Pearls for NEET-PG:** * **Mentzer Index:** (MCV/RBC count) 13 suggests IDA. * **RDW:** Usually normal in Thalassemia trait but increased in IDA. * **Confirmatory Test:** Hb Electrophoresis (showing increased HbA2 > 3.5%) is the gold standard for Beta-Thalassemia trait. * **FEP** is also increased in Sideroblastic anemia (except the X-linked ALAS2 deficiency subtype).

Q: A newborn girl presents with severe Purpura Fulminans. Her elder sibling died of severe purpura fulminans at 1 year of age. Two other siblings are normal. What is the diagnosis?

Protein C deficiency. **Explanation:** The clinical presentation of a newborn with **Purpura Fulminans** and a family history of a sibling death due to the same condition strongly points toward **Homozygous Protein C Deficiency**. **1. Why Protein C Deficiency is correct:** Protein C is a natural anticoagulant that inactivates Factors Va and VIIIa. In the rare autosomal recessive homozygous state, there is a complete absence of Protein C. This leads to unchecked thrombin generation, resulting in microvascular thrombosis, skin necrosis (Purpura Fulminans), and disseminated intravascular coagulation (DIC) shortly after birth. The family history suggests an autosomal recessive inheritance pattern (affected siblings, normal parents). **2. Why other options are incorrect:** * **Hemophilia A:** An X-linked recessive disorder causing Factor VIII deficiency. It presents with deep tissue bleeds or hemarthrosis, not purpura fulminans or neonatal skin necrosis. * **Immune Thrombocytopenic Purpura (ITP):** Usually occurs in older children following a viral infection. While it causes petechiae/purpura, it does not cause the life-threatening thrombotic necrosis seen in Purpura Fulminans. * **Von Willebrand Disease (vWD):** The most common inherited bleeding disorder, typically presenting with mucosal bleeds (epistaxis, menorrhagia) rather than neonatal thrombosis. **Clinical Pearls for NEET-PG:** * **Management:** Acute treatment involves **Fresh Frozen Plasma (FFP)** or Protein C concentrate. Long-term management requires oral anticoagulation (Warfarin). * **Warfarin-Induced Skin Necrosis:** Patients with heterozygous Protein C deficiency are at risk for skin necrosis when starting Warfarin without heparin bridging. * **Differential Diagnosis:** Consider **Protein S deficiency** or **Antithrombin III deficiency** in neonatal thrombosis, though Protein C deficiency is the classic association with Purpura Fulminans.

Question 1

What is the normal reticulocyte count in a newborn?

Accepted Answer

2.5-6%

Answer

0.2-1.5%

Answer

1-1.6%

Answer

6-10.2%

Question 2

A serious coagulopathy characterized by severe thrombocytopenia and hemorrhage due to platelet trapping within the tumor is called?

Accepted Answer

Kasabach-Merrit Phenomenon

Answer

Phace (S) Syndrome

Answer

Kaposi's sarcoma

Answer

Hemangioma

Question 3

Fanconi's anemia is a type of:

Accepted Answer

Constitutional anemia

Answer

Hemolytic anemia

Answer

Iron deficiency anemia

Answer

Autoimmune anemia

Question 4

A 4-year-old boy has appeared listless during the past week. He exhibits irritability when his arms or legs are touched. In the past 2 days, large ecchymoses have appeared on the right thigh and left shoulder. CBC shows hemoglobin, 9.3 g/dL; hematocrit, 28.7%; MCV, 96 mm3; platelet count, 45,000/mm3; and WBC count, 13,990/mm3. Examination of the peripheral blood smear shows blasts that lack peroxidase positive granules, but contain PAS-positive aggregates and stain positively for deoxynucleotidyl transferase negative (TdT-). Flow cytometry shows the phenotype of blasts to be CD19+, CD3-, and sIg-. Which of the following is the most likely diagnosis?

Accepted Answer

Acute lymphoblastic leukemia

Answer

Acute myelogenous leukemia

Answer

Chronic lymphocytic leukemia

Answer

Chronic myelogenous leukemia

Question 5

What is the treatment of choice for severe ITP?

Accepted Answer

Intravenous immunoglobulin (IV gammaglobulins)

Answer

Bone Marrow Transplant (BMT)

Answer

Plasmapheresis

Answer

Steroids

Question 6

All of the following are true regarding G-6PD deficiency except?

Accepted Answer

Females are commonly affected

Answer

A recessive X-linked trait

Answer

Oxidative stress causes hemolysis

Answer

Protective against Plasmodium falciparum malariae

Question 7

A child presents with hypochromic microcytic anemia, with normal levels of free Erythrocyte Protoporphyrin. What is the most likely diagnosis?

Accepted Answer

Thalassemia

Answer

Iron deficiency anemia

Answer

Lead toxicity

Answer

Anemia of chronic disease

Question 8

A 12-year-old male presented with a history of recurrent nose bleeds, easy bruising, frequent muscle hematoma formation, and prolonged bleeding following trivial trauma. On examination, certain skin lesions were seen along with jaundice, splenomegaly, lymphadenopathy, and joint and skin laxity. Lab findings revealed normal PT, prolonged aPTT, reduced ristocetin cofactor activity, and a normal factor VIII assay, normal platelet count and size, and normal platelet granules. What is the most probable diagnosis of the above condition?

Accepted Answer

von Willebrand disease

Answer

Bernard-Soulier syndrome

Answer

Hemophilia A

Answer

Platelet function defect

Question 9

A 14-year-old boy presents with acute onset of right flank pain, which developed after he helped his father paint the ceiling of his bedroom. Physical examination demonstrates an area of ecchymosis in the right flank that is tender to palpation. The patient has a lifelong history of easy bruising. His brother shows the same tendency. The serum level of clotting factor VIII is less than 2% of normal. Which of the following is the most likely underlying mechanism for bleeding tendency in this patient?

Accepted Answer

Genetic defect involving the factor VIII gene

Answer

Circulating antibodies directed against factor VIII

Answer

Decreased hepatic synthesis of multiple coagulation factors

Answer

Deficiency of vitamin K

Question 10

A newborn girl presents with severe Purpura Fulminans. Her elder sibling died of severe purpura fulminans at 1 year of age. Two other siblings are normal. What is the diagnosis?

Accepted Answer

Protein C deficiency

Answer

Hemophilia A

Answer

Immune Thrombocytopenic Purpura (ITP)

Answer

Von Willebrand disease (vWD)

Hematology — MCQs

Hematology — MCQs

On this page

Practice by Chapter

Want unlimited practice?