Hematopathology Practice Questions

Q: A 46-year-old lady presents with pallor. You suspect iron deficiency anemia. Which of the following blood investigation results is NOT consistent with this provisional diagnosis?

Decreased level of red cell protoporphyrin. In Iron Deficiency Anemia (IDA), the body lacks sufficient iron to complete the final step of heme synthesis. Under normal conditions, the enzyme **ferrochelatase** inserts ferrous iron into a precursor molecule called **protoporphyrin IX** to form heme. **Why Option D is the Correct Answer:** When iron is deficient, protoporphyrin has no iron to bind with. Consequently, it accumulates within the red blood cells. Therefore, in IDA, we see an **increased** level of **Free Erythrocyte Protoporphyrin (FEP)**, not a decreased level. This makes Option D inconsistent with the diagnosis. **Analysis of Incorrect Options:** * **A. Low plasma ferritin:** Ferritin is the storage form of iron [1]. A low serum ferritin is the **most specific** initial laboratory finding in IDA, reflecting depleted body stores [2]. * **B. Decreased saturation of transferrin:** As serum iron drops and Total Iron Binding Capacity (TIBC) increases, the percentage of transferrin saturated with iron falls (typically <16% in IDA) [2]. * **C. Decreased hemoglobin:** This is the hallmark of anemia [1]. In IDA, hemoglobin synthesis is impaired, leading to microcytic hypochromic anemia [2]. **NEET-PG High-Yield Pearls:** * **Earliest sign of IDA:** Decreased serum ferritin (depletion of stores) [2]. * **Earliest functional sign:** Increase in Red Cell Distribution Width (RDW) [2]. * **Gold Standard Investigation:** Bone marrow aspiration (Prussian blue staining) showing absent haemosiderin (though rarely done clinically) [2]. * **Mentzer Index:** (MCV/RBC count) 13 suggests IDA.

Q: In lymphoplasmacytoid lymphoma, which of the following monoclonal immunoglobulins is most commonly seen?

IgM. **Explanation:** **Lymphoplasmacytoid Lymphoma (LPL)** is a mature B-cell neoplasm characterized by a proliferation of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells. 1. **Why IgM is Correct:** The defining clinical feature of LPL is the secretion of a monoclonal protein. In the vast majority of cases (approximately 90-95%), this protein is **IgM** [1]. When LPL involves the bone marrow and is associated with a monoclonal IgM gammopathy of any size, it is clinically termed **Waldenström Macroglobulinemia (WM)** [1]. The large size of the IgM pentamer leads to the classic clinical presentation of hyperviscosity syndrome [1]. 2. **Why Other Options are Incorrect:** * **IgG and IgA:** While rare variants of LPL can secrete IgG or IgA, these are much more commonly associated with **Multiple Myeloma** or Monoclonal Gammopathy of Undetermined Significance (MGUS) [1]. * **IgD:** This is extremely rare and is typically associated with a specific, aggressive subtype of Multiple Myeloma, not LPL. **High-Yield Clinical Pearls for NEET-PG:** * **Genetic Marker:** Over 90% of LPL/WM cases harbor the **MYD88 L265P mutation**, which is a key diagnostic marker. * **Clinical Triad:** Look for anemia, hepatosplenomegaly/lymphadenopathy, and hyperviscosity (visual disturbances, mucosal bleeding, neurological symptoms) [1]. * **Morphology:** Bone marrow biopsy shows a "lymphoplasmacytoid" infiltrate and increased mast cells [1]. * **Differentiator:** Unlike Multiple Myeloma, LPL/WM typically does **not** cause lytic bone lesions or hypercalcemia (No "CRAB" features).

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).

Question 1

CD59 defect is a marker of which disease?

Accepted Answer

Paroxysmal Nocturnal Hemoglobinuria

Answer

PTEN

Answer

Hereditary spherocytosis

Answer

Cowden syndrome

Question 2

Burkitt's lymphoma is associated with which of the following gene anomalies?

Accepted Answer

t(8:14)

Answer

t(9:22)

Answer

del. 5q

Answer

t(17:15)

Question 3

A 46-year-old lady presents with pallor. You suspect iron deficiency anemia. Which of the following blood investigation results is NOT consistent with this provisional diagnosis?

Accepted Answer

Decreased level of red cell protoporphyrin

Answer

Low levels of plasma ferritin

Answer

Decreased saturation of transferrin

Answer

Decreased level of hemoglobin

Question 4

A triad of leukoerythroblastosis, tear drop erythrocytes, and large platelets is seen in which of the following conditions?

Accepted Answer

Primary myelofibrosis

Answer

Essential thrombocytosis

Answer

Myelodysplastic syndrome

Answer

Langerhans cell histiocytosis

Question 5

In lymphoplasmacytoid lymphoma, which of the following monoclonal immunoglobulins is most commonly seen?

Accepted Answer

IgM

Answer

IgA

Answer

IgD

Answer

IgG

Question 6

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 7

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 8

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 9

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 10

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

Accepted Answer

Lymphocyte predominant

Answer

Lymphocyte depletion

Answer

Mixed cellularity

Answer

Nodular sclerosis

Hematopathology — MCQs

Hematopathology — MCQs

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