Hematopathology Practice Questions

Q: Which of the following hematological malignancies is responsible for the finding shown in the image?

Acute myeloid leukemia (AML). ***Acute myeloid leukemia (AML)*** - The image shows **Auer rods**, which are **pathognomonic** (diagnostic) for AML and represent abnormal **azurophilic granules** in myeloid blast cells. - These pink-red, needle-like cytoplasmic inclusions are **highly specific** for AML and help distinguish it from other leukemias on **peripheral blood smear**. *Myelodysplastic syndrome (MDS)* - MDS shows **dysplastic changes** in blood cells but typically lacks **blast cells with Auer rods**. - Characterized by **cytopenia** with **hypercellular bone marrow** and morphological abnormalities, not the acute blast proliferation seen here. *Childhood chronic myeloid leukemia (CML)* - CML presents with **mature granulocytes** at various stages of development, not primitive blast cells. - The **Philadelphia chromosome** (BCR-ABL) is characteristic, and **Auer rods are not found** in CML cells. *Acute lymphoblastic leukemia (ALL)* - ALL involves **lymphoid blasts** that are typically **smaller and more uniform** than myeloid blasts. - **Auer rods are absent** in ALL as they are specific to **myeloid lineage** malignancies, not lymphoid.

Q: A 9-year-old child presents with continuous bleeding after tonsillectomy. Bleeding time and PTT are prolonged, while platelet count and PT are normal. What is the most likely diagnosis?

Von Willebrand disease. ### Explanation The clinical presentation of post-surgical bleeding combined with a specific laboratory profile points toward **Von Willebrand Disease (vWD)**, the most common inherited bleeding disorder. **Why Von Willebrand Disease is Correct:** Von Willebrand Factor (vWF) has two primary roles: 1. **Platelet Adhesion:** It acts as a bridge between platelets and the subendothelium. A deficiency leads to a defect in the platelet plug formation, resulting in a **prolonged Bleeding Time (BT)**. 2. **Carrier for Factor VIII:** vWF stabilizes Factor VIII in circulation. Low levels of vWF lead to a secondary decrease in Factor VIII, which causes a **prolonged Partial Thromboplastin Time (PTT)**. Since vWD does not affect the extrinsic pathway or platelet production, the **PT and Platelet count remain normal**. **Why Other Options are Incorrect:** * **Vitamin K Deficiency:** Affects Factors II, VII, IX, and X. This would primarily prolong the **PT** (due to Factor VII's short half-life) and PTT, but BT would be normal. * **Immune Thrombocytopenic Purpura (ITP):** Characterized by isolated **thrombocytopenia** (low platelet count). While BT would be prolonged, the PTT would be normal. * **Hemophilia A:** An isolated deficiency of Factor VIII. It causes a prolonged PTT, but the **BT is normal** because platelet-vWF interaction is unaffected. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Most common type (Type 1) is Autosomal Dominant. * **Screening Tests:** Prolonged BT + Prolonged PTT + Normal Platelet Count. * **Confirmatory Test:** Ristocetin Cofactor Assay (measures vWF activity). * **Treatment of Choice:** Desmopressin (DDAVP), which releases stored vWF from Weibel-Palade bodies. For severe cases, use vWF-containing concentrates.

Q: These types of RBCs are seen in which condition?

Chronic renal failure (CRF). ***Chronic renal failure (CRF)*** - **Echinocytes** (burr cells) are the characteristic RBC morphology seen in **chronic renal failure** due to uremic toxins affecting the RBC membrane. - **Uremia** causes metabolic changes that alter RBC shape, creating the spiky appearance of echinocytes on peripheral blood smear. *Hereditary spherocytosis* - Characterized by **spherocytes** (round, dense RBCs without central pallor) due to **membrane protein defects**. - Results from deficiencies in **spectrin**, **ankyrin**, or other membrane proteins, not the spiky echinocytes seen in the question. *Myelofibrosis* -典型表现为 **teardrop cells** (dacrocytes) due to **bone marrow fibrosis** causing mechanical distortion of RBCs. - Associated with **leukoerythroblastic** blood picture and **extramedullary hematopoiesis**, not echinocytes. *Myelodysplastic syndrome* - Features **dysplastic RBCs** with various morphological abnormalities including **macro-ovalocytes** and **dimorphic populations**. - Characterized by **ineffective erythropoiesis** and clonal stem cell disorders, not the uniform echinocyte morphology.

Q: A 60-year-old male patient complains of bleeding gums and loss of appetite. Histopathologic examination reveals basophilic leukocytosis. What condition might this patient have?

Chronic myeloid leukemia. The presence of **basophilic leukocytosis** (basophilia) is a hallmark diagnostic clue for **Chronic Myeloid Leukemia (CML)**. In clinical practice and for the NEET-PG exam, an absolute basophil count >2% is highly suggestive of a myeloproliferative neoplasm, and CML is the most common cause. **Why CML is correct:** CML is a myeloproliferative disorder characterized by the uncontrolled proliferation of the myeloid lineage. It typically presents with a massive shift to the left in the white cell line. Basophilia is a classic feature of CML and is often used as a marker for disease progression; an increasing basophil count often signals a transition from the Chronic Phase to the Accelerated Phase or Blast Crisis [1]. **Why other options are incorrect:** * **Hodgkin Lymphoma:** While it can cause eosinophilia, basophilia is not a characteristic feature. It typically presents with painless lymphadenopathy and Reed-Sternberg cells. * **Systemic Lupus Erythematosus (SLE):** SLE is an autoimmune condition usually associated with cytopenias (leukopenia, anemia, thrombocytopenia) rather than leukocytosis or basophilia. * **Bordetella pertussis:** This infection is famous for causing a marked **lymphocytosis** (absolute increase in lymphocytes), not basophilia. **High-Yield Clinical Pearls for NEET-PG:** * **Philadelphia Chromosome:** t(9;22) resulting in the *BCR-ABL1* fusion gene is the cytogenetic hallmark of CML [1]. * **LAP Score:** Leukocyte Alkaline Phosphatase (LAP) score is **decreased** in CML (helps differentiate it from a Leukemoid reaction where the score is increased). * **Drug of Choice:** Imatinib (Tyrosine Kinase Inhibitor) [1]. * **Splenomegaly:** CML is a common cause of massive splenomegaly [2].

Q: What is the CD marker for histiocytosis?

CD 1a. **Langerhans Cell Histiocytosis (LCH)** is a proliferative disorder of Langerhans cells, which are specialized dendritic cells [1]. In pathology, identifying these cells requires specific immunohistochemical (IHC) markers. **Why CD1a is the correct answer:** CD1a is the most specific and widely used diagnostic marker for Langerhans cells. It is a protein involved in presenting lipid antigens to T-cells. In the context of LCH, the presence of **CD1a** and **S100** on IHC, along with the identification of **Birbeck granules** (tennis-racket shaped organelles) on electron microscopy, confirms the diagnosis [1]. **Analysis of incorrect options:** * **CD1b, CD1c, and CD1d:** While these are also members of the CD1 family involved in lipid antigen presentation, they are not used as diagnostic markers for histiocytosis. CD1b and CD1c are expressed on various subsets of dendritic cells and B-cells, while CD1d is primarily involved in presenting lipids to NKT cells. They lack the diagnostic specificity required for LCH. **High-Yield Clinical Pearls for NEET-PG:** * **Langerin (CD207):** This is the most specific marker for LCH as it is directly associated with the formation of Birbeck granules [1]. * **BRAF V600E Mutation:** Found in approximately 50% of LCH cases; it is a common "hot topic" in molecular pathology [1]. * **Classic Triad (Hand-Schüller-Christian disease):** Calvarial bone defects, diabetes insipidus, and exophthalmos. * **Letterer-Siwe Disease:** The aggressive, multisystem form of LCH seen in infants (<2 years). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 629-630.

Q: Diagnostic criteria of pure red cell aplasia (PRCA) includes all of the following, except:

Thrombocytopenia. **Explanation:** **Pure Red Cell Aplasia (PRCA)** is a rare hematological syndrome characterized by a selective and severe deficiency in erythroid precursors in the bone marrow, while the production of leukocytes and platelets remains preserved [1]. **Why Thrombocytopenia is the Correct Answer:** The hallmark of PRCA is the **isolation** of the erythroid defect. By definition, granulopoiesis (white cells) and megakaryopoiesis (platelets) are normal. Therefore, **thrombocytopenia** (low platelet count) is not a feature of PRCA. If a patient presents with anemia along with thrombocytopenia or leukopenia, the diagnosis shifts toward Aplastic Anemia or Myelodysplastic Syndrome (MDS). **Analysis of Incorrect Options:** * **Severe Anemia:** This is the primary clinical manifestation. Patients typically present with symptomatic normocytic, normochromic anemia due to the cessation of red cell production. * **Reticulocyte count < 1%:** Since there is a maturation arrest or absence of erythroid precursors in the marrow, the output of new red cells into the blood is near zero, leading to profound reticulocytopenia (typically <0.5% or <10,000/µL). * **Normocellular Marrow:** Unlike Aplastic Anemia (where the marrow is hypocellular and fatty), the marrow in PRCA is generally normocellular. The diagnostic finding is the near-complete absence of erythroid precursors (<0.5% to 1%) amidst normal myeloid and megakaryocytic lineages. **High-Yield Clinical Pearls for NEET-PG:** * **Associations:** PRCA is strongly associated with **Thymoma** (10-15% of cases). Removal of the thymoma can lead to remission. * **Infections:** Acute self-limiting PRCA is often caused by **Parvovirus B19**, which targets the P-antigen on proerythroblasts (look for **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 595-596.

Question 1

Which of the following hematological malignancies is responsible for the finding shown in the image?

Accepted Answer

Acute myeloid leukemia (AML)

Answer

Myelodysplastic syndrome (MDS)

Answer

Childhood chronic myeloid leukemia (CML)

Answer

Acute lymphoblastic leukemia (ALL)

Question 2

A 9-year-old child presents with continuous bleeding after tonsillectomy. Bleeding time and PTT are prolonged, while platelet count and PT are normal. What is the most likely diagnosis?

Accepted Answer

Von Willebrand disease

Answer

Vitamin K deficiency

Answer

Immune thrombocytopenic purpura

Answer

Hemophilia A

Question 3

A patient of multiple myeloma presents with bony lesions. What is the best prognostic marker for the disease?

Accepted Answer

Beta-2 microglobulin

Answer

Bone marrow plasma cell percentage

Answer

Serum calcium level

Answer

Beta-1 microglobulin

Question 4

These types of RBCs are seen in which condition?

Accepted Answer

Chronic renal failure (CRF)

Answer

Hereditary spherocytosis

Answer

Myelofibrosis

Answer

Myelodysplastic syndrome

Question 5

A 60-year-old male patient complains of bleeding gums and loss of appetite. Histopathologic examination reveals basophilic leukocytosis. What condition might this patient have?

Accepted Answer

Chronic myeloid leukemia

Answer

Hodgkin lymphoma

Answer

Systemic lupus erythematosus

Answer

Bordetella pertussis infection

Question 6

In Promyelocytic Leukemia, which of the following is NOT a typical feature?

Accepted Answer

CD15 and CD34 markers are positive on the same cell population.

Answer

All-trans retinoic acid is a key component of treatment.

Answer

A characteristic chromosomal translocation is t(15;17).

Answer

It is commonly associated with Disseminated Intravascular Coagulation (DIC).

Question 7

What is the CD marker for histiocytosis?

Accepted Answer

CD 1a

Answer

CD 1b

Answer

CD 1c

Answer

CD 1d

Question 8

How will you differentiate a mediastinal mass being a thymoma or acute lymphoblastic leukemia?

Accepted Answer

Cytokeratin

Answer

CD1a

Answer

CD3

Answer

Tdt

Question 9

Diagnostic criteria of pure red cell aplasia (PRCA) includes all of the following, except:

Accepted Answer

Thrombocytopenia

Answer

Severe anemia

Answer

Reticulocyte count < 1%

Answer

Normocellular marrow

Question 10

Which of the following are characteristic markers for Hodgkin's Lymphoma?

Accepted Answer

CD15 and CD30

Answer

CD15 and CD45

Answer

CD30 and CD68

Answer

CD15 and CD3

Hematopathology — MCQs

Hematopathology — MCQs

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