Hematopathology Practice Questions

Q: A 7-year-old boy presents with a week-long history of severe headache. Physical examination reveals tenderness on palpation of long bones, hepatosplenomegaly, and generalized lymphadenopathy, along with petechial hemorrhages on the skin. Laboratory findings include hemoglobin 8.8 g/dL, hematocrit 26.5%, platelet count 34,700/mm3, and WBC count 14,800/mm3. A bone marrow biopsy shows 100% cellularity, with near-complete replacement by large cells characterized by scant, agranular cytoplasm, delicate nuclear chromatin, and rare nucleoli. Chemotherapy is anticipated to induce a complete remission. Which of the following combinations of phenotypic and karyotypic markers is most likely to be present in the marrow cells of this boy?

Early pre-B CD19+ Hyperdiploidy. ### Explanation The clinical presentation of a 7-year-old boy with bone pain, hepatosplenomegaly, lymphadenopathy, and pancytopenia (anemia, thrombocytopenia) is classic for **Acute Lymphoblastic Leukemia (ALL)**, the most common pediatric malignancy. The bone marrow biopsy showing "scant, agranular cytoplasm and delicate chromatin" confirms the presence of lymphoblasts (L1/L2 morphology). **1. Why Option A is Correct:** * **Phenotype:** Approximately 75-80% of childhood ALL cases are of the **Early pre-B cell** lineage, which characteristically expresses **CD19**, CD10 (CALLA), and TdT. * **Karyotype:** **Hyperdiploidy** (>50 chromosomes) is the most common cytogenetic abnormality in pediatric B-ALL and is a **favorable prognostic factor**, correlating with the "complete remission" mentioned in the stem. * **Age:** The peak incidence is between 2–5 years, fitting this pediatric profile. **2. Why Other Options are Incorrect:** * **Option B:** While CD20 can be expressed in later stages of B-cell maturation, **t(9;22)** (Philadelphia chromosome) is associated with a **poor prognosis** and is more common in adults or a small subset of high-risk pediatric cases. * **Option C:** **CD5** is a T-cell marker (also found in B-CLL). ALL with T-cell markers usually presents with a mediastinal mass and higher WBC counts. * **Option D:** **CD23 and 11q deletion** are characteristic markers for **Chronic Lymphocytic Leukemia (CLL)**, which is a disease of the elderly and virtually never seen in a 7-year-old. **Clinical Pearls for NEET-PG:** * **Most common childhood cancer:** ALL. * **Good Prognosis Factors:** Age 1–9 years, WBC 10 years, WBC >50,000/µL, Hypodiploidy, t(9;22), t(4;11). * **Common sites of relapse:** Testes and CNS (due to the blood-brain/blood-testis barrier).

Q: A 2-year-old child in shock presents with multiple non-blanching purple lesions of various sizes scattered on the trunk and extremities. Petechiae are noted, and oozing from the venipuncture site has been observed. The child's peripheral blood smear is shown below. Clotting studies are likely to show which of the following?

The presence of fibrin split products. ***The presence of fibrin split products*** - The clinical presentation of **shock**, **non-blanching purple lesions**, **petechiae**, and **oozing from venipuncture sites** strongly suggests **disseminated intravascular coagulation (DIC)**. - In DIC, **fibrinogen** is converted to **fibrin** and subsequently broken down by **plasmin**, leading to elevated **fibrin degradation products (FDPs)** and **D-dimers** as hallmark findings. *Increased levels of factors V and VIII* - In DIC, **coagulation factors** including **factors V and VIII** are **consumed** during the coagulation cascade, resulting in **decreased** rather than increased levels. - The **consumption coagulopathy** depletes clotting factors as they are utilized in widespread **microthrombi formation** throughout the circulation. *A decreased prothrombin level* - While **prothrombin (factor II)** levels may be decreased due to consumption in DIC, this is not the **most characteristic** or **reliable** finding. - **Fibrin split products** are more **specific** and **sensitive** markers for diagnosing DIC compared to individual factor levels. *An increased fibrinogen level* - **Fibrinogen** levels are typically **decreased** in DIC due to **consumption** during widespread **fibrin formation** and subsequent **fibrinolysis**. - The **schistocytes** visible on blood smear indicate **microangiopathic hemolytic anemia**, supporting the diagnosis of DIC where fibrinogen is consumed.

Q: Acid Citrate Dextrose (ACD) is used to store blood and preserves Red Blood Cells (RBCs) for 21 days. What are the respective storage periods when phosphate alone is added to ACD, and when adenine and phosphate are added together to ACD?

21 days and 35 days. ### Explanation The storage life of Red Blood Cells (RBCs) in a blood bank depends on the composition of the anticoagulant-preservative solution, which maintains cell viability by providing nutrients and preventing metabolic decay. **1. Understanding the Correct Answer (Option B):** * **ACD (Acid Citrate Dextrose):** The baseline storage period is **21 days**. Citrate acts as an anticoagulant, while Dextrose provides a substrate for ATP production via glycolysis. * **CPD (Citrate Phosphate Dextrose):** When **Phosphate** is added to ACD, the storage period remains **21 days**. However, Phosphate acts as a buffer to maintain pH and maintains higher levels of 2,3-DPG, ensuring better oxygen delivery post-transfusion. * **CPDA-1 (Citrate Phosphate Dextrose Adenine):** When **Adenine** is added along with Phosphate, the storage period increases to **35 days**. Adenine provides a substrate for the synthesis of ATP, which is essential for maintaining the RBC membrane integrity and the sodium-potassium pump. **2. Why Other Options are Incorrect:** * **Option A & D:** 42 days is the storage life for **Additive Solutions** (like SAGM: Saline-Adenine-Glucose-Mannitol), not CPDA-1. 35 days refers to CPDA-1, not CPD alone. * **Option C:** 28 days and 45 days are not standard storage durations for the primary anticoagulant solutions used in clinical practice. **3. Clinical Pearls for NEET-PG:** * **Storage Temperature:** Blood is stored at **2°C to 6°C**. * **The "Storage Lesion":** During storage, there is a decrease in pH, 2,3-DPG, and Sodium, while there is an **increase in Potassium** and Lactate. * **SAGM (Additive Solution):** Extends shelf life to **42 days** by providing optimal nutrients after the plasma is removed. * **Frozen RBCs:** Can be stored for up to **10 years** using Glycerol as a cryoprotectant.

Q: Which cells are characteristic of Hodgkin's disease?

Reed-Sternberg cells. **Explanation:** **Reed-Sternberg (RS) cells** are the diagnostic hallmark of Hodgkin Lymphoma (HL) [1]. These are large, multinucleated or polylobed B-cells, typically featuring a "mirror-image" nuclei with prominent, eosinophilic, owl-eye nucleoli. While they are the neoplastic component, they usually constitute only 1–5% of the total tumor mass, with the remainder being a reactive inflammatory background. **Analysis of Options:** * **Lacunar cells (Option A):** These are a specific *variant* of RS cells seen primarily in the **Nodular Sclerosis** subtype of HL. While characteristic of that subtype, the classic RS cell is the defining feature for Hodgkin’s disease as a whole. * **Giant cells (Option C):** This is a non-specific term. While RS cells are large, "giant cells" typically refer to Langhans giant cells (Tuberculosis) or Foreign Body giant cells, which are not diagnostic of HL. * **Eosinophils (Option D):** These are commonly found in the reactive background of HL (recruited by IL-5 secreted by RS cells), especially in the Mixed Cellularity subtype, but they are not the neoplastic or diagnostic cells. **High-Yield Pearls for NEET-PG:** * **Immunophenotype:** Classic RS cells are typically **CD15+ and CD30+**, but **CD20- and CD45-**. * **Popcorn Cells (L&H cells):** These are variants seen in **Nodular Lymphocyte Predominant HL**; they are CD20+ and CD45+, but CD15- and CD30-. * **Bimodal Age Distribution:** HL shows peaks in the 20s and again after age 50. * **EBV Association:** Most strongly linked with the Mixed Cellularity subtype.

Q: Non-immune hemolytic anemia occurs in which of the following conditions?

Plasmodium vivax infection. **Explanation:** Hemolytic anemia is broadly classified into **Immune** (mediated by antibodies) and **Non-immune** (mediated by mechanical, infectious, or metabolic factors). **Why Option B is Correct:** *Plasmodium vivax* (Malaria) causes **non-immune hemolytic anemia** through direct mechanical destruction of red blood cells (RBCs) [1]. The parasite invades the RBC, consumes hemoglobin, and eventually causes the cell to rupture (erythrocytic schizogony). Additionally, the spleen sequesters and destroys both parasitized and non-parasitized RBCs due to increased fragility and oxidative stress. Since this process is driven by the pathogen and not by autoantibodies, it is non-immune. **Why the Other Options are Incorrect:** * **A. SLE:** This is a classic cause of **Autoimmune Hemolytic Anemia (AIHA)**, typically mediated by IgG antibodies (Warm AIHA). It is a "Type II Hypersensitivity" reaction. * **C. CLL:** This is the most common malignancy associated with **Warm AIHA** [2]. The neoplastic B-cells produce autoantibodies against RBC antigens. * **D. CML:** While CML causes anemia, it is typically due to bone marrow infiltration (myelophthisis) or anemia of chronic disease, not primary hemolysis. It is not a classic cause of immune or non-immune hemolytic anemia. **NEET-PG High-Yield Pearls:** * **Direct Antiglobulin Test (Coombs Test):** Positive in immune hemolysis (SLE, CLL) and **negative** in non-immune hemolysis (Malaria, G6PD deficiency, HUS/TTP). * **Malaria & RBCs:** *P. vivax* and *P. ovale* selectively infect **reticulocytes** (young RBCs), whereas *P. falciparum* infects RBCs of all ages, leading to higher parasitemia. * **Microangiopathic Hemolytic Anemia (MAHA):** Another high-yield non-immune cause characterized by **schistocytes** on peripheral smear.

Q: A 7-year-old child presents with fever, bleeding gums, and fatigue for 4 days. On examination, she has splenomegaly. CBC shows Hb of 6 gm%, TLC 75,000/µL, and platelet count of 35,000/µL. Bone marrow aspiration shows these cells comprising 50% of marrow cells. What is the diagnosis?

Acute leukemia. ***Acute leukemia*** - **Bone marrow blasts comprising 50%** of marrow cells confirms acute leukemia (≥20% blasts required for diagnosis). - Classic pediatric presentation with **fever**, **bleeding gums**, **fatigue**, **splenomegaly**, and **pancytopenia** (anemia, thrombocytopenia with elevated WBC count). *Infectious mononucleosis* - Typically caused by **Epstein-Barr virus** with **atypical lymphocytes** on blood smear, not blast cells. - **Bone marrow blasts** would not comprise 50% of cells; lymphadenopathy is more common than splenomegaly. *Myelofibrosis* - **Bone marrow fibrosis** leads to **dry tap** on aspiration, not cellular marrow with 50% blasts. - Characterized by **teardrop-shaped RBCs** and **leukoerythroblastic picture** on peripheral smear. *Juvenile CML* - **Chronic phase** typically shows **massive leukocytosis** (>100,000/µL) with **left shift** and **basophilia**. - **Bone marrow** shows **hypercellularity** with **<5% blasts**, not 50% as seen in this case.

Question 1

All of the following are causes of fragmented RBCs in peripheral blood EXCEPT:

Accepted Answer

Hemophilia A

Answer

Microangiopathic hemolytic anemia

Answer

Disseminated intravascular coagulation

Answer

Malignant hypertension

Question 2

A deficiency of iron can result in anemia. Which type of anemia would occur if iron were deficient?

Accepted Answer

Hypochromic, microcytic anemia

Answer

Megaloblastic anemia

Answer

Hemolytic anemia

Answer

Sickle cell anemia

Question 3

A 7-year-old boy presents with a week-long history of severe headache. Physical examination reveals tenderness on palpation of long bones, hepatosplenomegaly, and generalized lymphadenopathy, along with petechial hemorrhages on the skin. Laboratory findings include hemoglobin 8.8 g/dL, hematocrit 26.5%, platelet count 34,700/mm3, and WBC count 14,800/mm3. A bone marrow biopsy shows 100% cellularity, with near-complete replacement by large cells characterized by scant, agranular cytoplasm, delicate nuclear chromatin, and rare nucleoli. Chemotherapy is anticipated to induce a complete remission. Which of the following combinations of phenotypic and karyotypic markers is most likely to be present in the marrow cells of this boy?

Accepted Answer

Early pre-B CD19+ Hyperdiploidy

Answer

Early pre-B CD20+ t(9;22)

Answer

Pre-B CD5+ normal karyotype

Answer

Pre-B CD23+ 11q deletion

Question 4

Which of the following is NOT true about BCR-ABL variants?

Accepted Answer

P190 is associated with an indolent clinical course.

Answer

P190 is considered a poor prognostic factor.

Answer

P230 is found in chronic neutrophilic leukemia.

Answer

P230 is associated with an indolent clinical course.

Question 5

What is the best prognostic type of Hodgkin's disease?

Accepted Answer

Lymphocyte predominant

Answer

Lymphocyte depletion

Answer

Mixed cellularity

Answer

Nodular sclerosis

Question 6

A 2-year-old child in shock presents with multiple non-blanching purple lesions of various sizes scattered on the trunk and extremities. Petechiae are noted, and oozing from the venipuncture site has been observed. The child's peripheral blood smear is shown below. Clotting studies are likely to show which of the following?

Accepted Answer

The presence of fibrin split products

Answer

Increased levels of factors V and VIII

Answer

A decreased prothrombin level

Answer

An increased fibrinogen level

Question 7

Acid Citrate Dextrose (ACD) is used to store blood and preserves Red Blood Cells (RBCs) for 21 days. What are the respective storage periods when phosphate alone is added to ACD, and when adenine and phosphate are added together to ACD?

Accepted Answer

21 days and 35 days

Answer

35 days and 42 days

Answer

28 days and 45 days

Answer

35 days and 28 days

Question 8

Which cells are characteristic of Hodgkin's disease?

Accepted Answer

Reed-Sternberg cells

Answer

Lacunar cells

Answer

Giant cells

Answer

Eosinophils

Question 9

Non-immune hemolytic anemia occurs in which of the following conditions?

Accepted Answer

Plasmodium vivax infection

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

Chronic Myeloid Leukemia (CML)

Question 10

A 7-year-old child presents with fever, bleeding gums, and fatigue for 4 days. On examination, she has splenomegaly. CBC shows Hb of 6 gm%, TLC 75,000/µL, and platelet count of 35,000/µL. Bone marrow aspiration shows these cells comprising 50% of marrow cells. What is the diagnosis?

Accepted Answer

Acute leukemia

Answer

Infectious mononucleosis

Answer

Myelofibrosis

Answer

Juvenile CML

Hematopathology — MCQs

Hematopathology — MCQs

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