Hematopathology Practice Questions

Q: A 17-year-old boy presented with a total leukocyte count of 138 × 10^9/L, with 80% blasts on the peripheral smear. Chest X-ray demonstrated a large mediastinal mass. Immunophenotyping of this patient's blasts would most likely demonstrate:

Markers associated with immature T-cell lineage. ***An immature T cell phenotype (Tdt/CD34/CD7 positive)*** - Given the age of the patient and presentation, the high percentage of blasts along with the mediastinal mass suggests **T-cell acute lymphoblastic leukemia (T-ALL)** [1], which commonly presents with an immature T cell phenotype. - The presence of **Tdt, CD34, and CD7** indicates the immature nature of the T cells, which is characteristic of this condition. *No surface antigens (null phenotype)* - A null phenotype typically indicates a **lack of differentiation**, which is not consistent with the high blast count seen here. - Most leukemias or lymphomas usually express some markers, especially in a case with significant blast population like this one. *Myeloid markers, such as CD13, CD33 and CD15* - These markers are indicative of **myeloid lineage**, which is not relevant in this case as it suggests an **acute myeloid leukemia (AML)**, not T-ALL. - The clinical context and immunophenotyping results would not support myeloid lineage in a patient with such a presentation. *B cell markers, such as CD 19, CD20 and CD22* - These are **characteristic of B-cell lineage** [1], which is not relevant in this scenario, given the presence of a mediastinal mass often associated with T-cell malignancies. - The blast cells in this patient are clearly indicative of a **T-cell disorder**, rather than a B-cell neoplasm. **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. 598-600.

Q: Prothrombin time is prolonged in all of the following conditions except:

Von Willebrand's disease. ***Von Willebrand's disease*** - **Von Willebrand's disease** primarily affects **platelet adhesion** and the **intrinsic coagulation pathway** (by carrying factor VIII), rather than the extrinsic pathway measured by PT [1]. - Patients typically have a **normal prothrombin time (PT)** and activated partial thromboplastin time (aPTT) that may be normal or slightly prolonged. *Liver disease* - The **liver** synthesizes most **coagulation factors**, including those measured by PT (factor VII from the extrinsic pathway and factors II, V, X from the common pathway) [3]. - Severe **liver disease** impairs factor synthesis, leading to **deficiencies** and a prolonged PT [1]. *Disseminated intravascular coagulation* - **DIC** involves widespread activation of the coagulation system, leading to the consumption of **clotting factors**, including those measured by PT [2]. - This **consumption coagulopathy** results in prolonged PT, aPTT, and thrombocytopenia [2]. *Warfarin therapy* - **Warfarin** is a **vitamin K antagonist** that inhibits the synthesis of vitamin K-dependent clotting factors (II, VII, IX, X) [1], [3]. - By inhibiting **factor VII** (extrinsic pathway) and the **common pathway factors**, warfarin prolongs the **prothrombin time (PT)**, which is used to monitor its anticoagulant effect [3]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 624-625. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 625-626. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 582-583.

Q: A 45-year-old female patient presented with painless supraclavicular lymphadenopathy. Biopsy revealed binucleated acidophilic owl eye appearance with floating lymphocytes in empty space, which were CD15 and CD30 positive. What is the most probable diagnosis?

Nodular sclerosis Hodgkin lymphoma (NSHL). ***Nodular sclerosis Hodgkin lymphoma*** - Characterized by a **painless supraclavicular lymphadenopathy** and the presence of **binucleated Reed-Sternberg cells** with the "owl eye" appearance. - **CD15** and **CD30 positivity** confirm the diagnosis, common in this subtype of Hodgkin lymphoma [2]. *Lymphocytic predominant Hodgkin lymphoma* - Typically shows **lymphocyte-rich background** and may present with fewer **Reed-Sternberg cells**, which differ from the described findings [3]. - **CD15** and **CD30 positivity** is generally low, contrasting with the strong positivity seen in this case. *Lymphocytic depleted lymphoma* - Characterized by a **lack of lymphocytes** and high numbers of **Reed-Sternberg cells**, not matching the floating lymphocyte description [1]. - **Usually CD15 and CD30** positive but presents with **more aggressive clinical features** than nodular sclerosis. *Mixed cellularity Hodgkin lymphoma* - This form also presents with Reed-Sternberg cells but typically involves a more **heterogeneous population** of inflammatory cells [2,3]. - Presentation of **painless lymphadenopathy** can occur, but the specific histological findings here are more indicative of nodular sclerosis. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 559-560. [2] 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. 616-618. [3] 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, p. 618.

Q: Loose fibrin accumulated in a tight clot in the coagulation pathway is caused by which factor?

Factor XIII. ***Factor XIII*** - **Factor XIII** (fibrin-stabilizing factor) is responsible for **cross-linking fibrin monomers**, converting loose fibrin into a stable, insoluble clot [1]. - This cross-linking process strengthens the clot, preventing premature degradation and ensuring effective hemostasis [2]. *Factor X* - **Factor X** (Stuart-Prower factor) is a key enzyme in the coagulation cascade that, when activated to Factor Xa, forms part of the **prothrombinase complex** that converts **prothrombin to thrombin** [2]. - It does not directly cause the cross-linking of fibrin to form a tight clot. *Factor XI* - **Factor XI** (plasma thromboplastin antecedent) is involved in the intrinsic pathway, activating **Factor IX**. - Its primary role is in propagating the coagulation cascade, not in the final stabilization of the fibrin clot. *Factor XII* - **Factor XII** (Hageman factor) initiates the intrinsic pathway of coagulation and is involved in fibrinolysis and inflammation. - It does not directly influence the stability or cross-linking of the fibrin clot structure. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, p. 130. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 582-583.

Q: Which of the following statements regarding hereditary spherocytosis is false?

Splenomegaly is not a feature of hereditary spherocytosis. ***Spherocytosis is a pathognomic finding*** - Spherocytosis is not **pathognomic** for hereditary spherocytosis as it can also occur in other conditions like autoimmune hemolytic anemia. - While **spherocytes** are a notable finding in hereditary spherocytosis, they are not exclusive to this disorder. *Anemia* - Anemia is a common result of **hemolysis** in hereditary spherocytosis due to the destruction of spherical red blood cells [1]. - Patients often present with **mild to moderate anemia** [1][2], but this is a characteristic finding, not an exception. *Splenomegaly* - Splenomegaly is frequently observed in hereditary spherocytosis due to **hypertrophy** from increased red blood cell destruction. - This finding is **typical** in cases of hereditary spherocytosis and serves as an important clinical indicator [2]. *It is due to intrinsic defect in RBC membrane skeleton* - Hereditary spherocytosis is primarily caused by an **intrinsic defect in the red blood cell membrane**, particularly affecting proteins like spectrin and ankyrin [1]. - This intrinsic defect leads to the formation of **spherocytes**, which are less deformable and prone to hemolysis [1][2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 640-642. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 597-598.

Q: A 9-year-old boy with elevation in both prothrombin time (PT) and activated partial thromboplastin time (APTT) is diagnosed with what?

Defect in common pathway. ***Defect in common pathway*** - Elevation in both **prothrombin time (PT)** and **activated partial thromboplastin time (APTT)** indicates a problem in the **common pathway**, affecting factors I, II, V, X [1]. - This defect can result from conditions such as **vitamin K deficiency** or **liver dysfunction**, which impact multiple coagulation factors. *Platelet function defect* - Typically presents with **prolonged bleeding times** but does not affect PT or APTT, which remain normal. - Symptoms often include **petechiae** or **easy bruising**, rather than the coagulation profile observed here. *Defect in extrinsic pathway* - Primarily affects **PT** only, not APTT, as this pathway involves factors VII and tissue factor [1]. - Conditions like **vitamin K deficiency** can cause prolonged PT, but APTT would not be elevated simultaneously in isolation. *Defect in intrinsic pathway* - Affects **APTT** primarily, causing a prolonged APTT while PT remains normal [1]. - Common conditions include **Hemophilia A or B**, but the simultaneous elevation of both PT and APTT rules this out. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 128-130.

Q: Which of the following statements about reticulocytes is correct?

Increased in hemolytic anemia. ***↑in hemolytic anemia*** - Reticulocyte count is typically **increased** in hemolytic anemia as the bone marrow responds to **acute blood loss** or increased hemolysis by producing more red blood cells [1]. - This reflects the body's mechanism to replace **lost RBCs**, making reticulocyte counts a useful marker in diagnosing anemia types. *Contain nucleus* - Reticulocytes do not contain a **nucleus**; they are immature red blood cells that have **already extruded** their nucleus. - Their main function is to mature into erythrocytes, which are **anucleate** cells. *Staining is done by Romanowsky stain* - Reticulocytes are typically stained using **methylene blue** or **brilliant cresyl blue**, not Romanowsky stain, which is used for **WBC and smears**. - These specific stains highlight the **reticulated cytoplasm**, indicating RNA presence, crucial for identifying reticulocytes. *Smaller than RBC* - Reticulocytes are generally **larger** than mature red blood cells (RBCs) due to their **cellular contents**, which are not fully extruded yet. - Their size is an important factor in distinguishing them from mature RBCs during evaluation. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 638-639.

Question 1

A 17-year-old boy presented with a total leukocyte count of 138 × 10^9/L, with 80% blasts on the peripheral smear. Chest X-ray demonstrated a large mediastinal mass. Immunophenotyping of this patient's blasts would most likely demonstrate:

Accepted Answer

Markers associated with immature T-cell lineage

Answer

Markers associated with myeloid lineage

Answer

Markers associated with B-cell lineage

Answer

A null phenotype (no surface antigens)

Question 2

Prothrombin time is prolonged in all of the following conditions except:

Accepted Answer

Von Willebrand's disease

Answer

Liver disease

Answer

Disseminated intravascular coagulation

Answer

Warfarin therapy

Question 3

A 45-year-old female patient presented with painless supraclavicular lymphadenopathy. Biopsy revealed binucleated acidophilic owl eye appearance with floating lymphocytes in empty space, which were CD15 and CD30 positive. What is the most probable diagnosis?

Accepted Answer

Nodular sclerosis Hodgkin lymphoma (NSHL)

Answer

Lymphocytic predominant Hodgkin lymphoma

Answer

Mixed cellularity Hodgkin lymphoma

Answer

Lymphocytic depleted Hodgkin lymphoma

Question 4

Which of the following is a false statement regarding disseminated intravascular coagulation (DIC)?

Accepted Answer

Decreased PTT

Answer

Decreased fibrinogen

Answer

Increased PT

Answer

Thrombocytopenia

Question 5

Loose fibrin accumulated in a tight clot in the coagulation pathway is caused by which factor?

Accepted Answer

Factor XIII

Answer

Factor X

Answer

Factor XI

Answer

Factor XII

Question 6

Which of the following statements regarding hereditary spherocytosis is false?

Accepted Answer

Splenomegaly is not a feature of hereditary spherocytosis

Answer

Anemia

Answer

It is due to intrinsic defect in RBC membrane skeleton

Answer

Spherocytes are the characteristic finding on peripheral blood smear

Question 7

A 9-year-old boy with elevation in both prothrombin time (PT) and activated partial thromboplastin time (APTT) is diagnosed with what?

Accepted Answer

Defect in common pathway

Answer

Platelet function defect

Answer

Defect in the extrinsic pathway

Answer

Defect in the intrinsic pathway

Question 8

Which of the following statements about reticulocytes is correct?

Accepted Answer

Increased in hemolytic anemia

Answer

Larger than RBC

Answer

Contain nucleus

Answer

Staining is done by Romanowsky stains

Question 9

Which of the following is a characteristic finding in hereditary spherocytosis?

Accepted Answer

Increased Osmotic Fragility

Answer

Increased Reticulocyte Count

Answer

Splenomegaly

Answer

Negative Direct Coombs Test

Hematopathology — MCQs

Hematopathology — MCQs

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