Bleeding Disorders — MCQs

10 questions

Disseminated intravascular coagulation (DIC) differs from thrombotic thrombocytopenic purpura. In this reference, DIC is most likely characterized by:

A 9-year-old girl has a bleeding diathesis, with prolonged activated partial thromboplastin time (APTT) but normal prothrombin time (PT) and normal platelet count. The diagnosis is?

Which of the following statements regarding von Willebrand disease is incorrect?

Glanzmann thrombasthenia is due to

Factor V mutation most commonly initially presents as:-

Following injury to a blood vessel, immediate hemostasis is achieved by:

Glanzmann thrombasthenia is due to defect in:-

A bone marrow aspirate shows blast cells with multiple Auer rods arranged in bundles ('faggot cells'). Which molecular finding would confirm acute promyelocytic leukemia?

All of the following are seen in polycythemia rubra vera except :

Q10

Adenoidectomy is contraindicated in:

Bleeding Disorders Indian Medical PG Practice Questions and MCQs

Question 1: Disseminated intravascular coagulation (DIC) differs from thrombotic thrombocytopenic purpura. In this reference, DIC is most likely characterized by:

A. Elevated reticulocyte count
B. Low levels of coagulation factors (Correct Answer)
C. Severe thrombocytopenia
D. Presence of schistocytes in the blood smear

Explanation: ***Decreased coagulation factor levels*** - DIC is characterized by an activation of the coagulation cascade, leading to increased consumption of **coagulation factors** and resulting in low levels [1]. - This process causes a paradoxical increased risk of bleeding despite a **consumption coagulopathy** scenario [1]. *Significant thrombocytopenia* - While thrombocytopenia can occur in DIC, it is not as pronounced as in **thrombotic thrombocytopenic purpura** (TTP), which features **severe thrombocytopenia** as its hallmark [2]. - DIC typically presents with **variable platelet counts**, often fluctuating based on the underlying cause. *A brisk reticulocytosis* - Reticulocytosis is common in hemolytic processes, but it is not a defining characteristic of DIC, which primarily involves dysfunction in the **coagulation cascade** rather than increased red blood cell production. - In contrast, TTP may show reticulocytosis due to hemolysis, but this does not apply directly to DIC. *Significant numbers of schistocytes* - Schistocytes are seen in microangiopathic hemolytic anemias, but **quantity and significance** vary; they may not be prominently present in DIC cases compared to TTP, which is distinguished by more pronounced schistocytes [2]. - DIC primarily leads to a **consumption coagulopathy**, whereas schistocytes more specifically indicate **mechanical hemolysis** [2].

Question 2: A 9-year-old girl has a bleeding diathesis, with prolonged activated partial thromboplastin time (APTT) but normal prothrombin time (PT) and normal platelet count. The diagnosis is?

A. Factor V deficiency
B. Haemophilia A
C. Haemophilia B
D. Von Willebrand's disease (Correct Answer)

Explanation: ***Von Willebrand's disease*** - This condition is characterized by a **deficiency or dysfunction of von Willebrand factor (vWF)**, leading to impaired platelet adhesion and stabilization of factor VIII [2], [3]. - While it primarily affects **platelet function**, it can also cause a prolonged **APTT** due to the role of vWF in protecting factor VIII from degradation, thus impacting the intrinsic coagulation pathway [3]. *Factor V deficiency* - Factor V is a component of the **common pathway** of coagulation. - A deficiency would typically prolong both the **PT and APTT** [1]. *Haemophilia A* - This is a deficiency of **Factor VIII**, which is part of the **intrinsic pathway** [1]. - Haemophilia A causes a prolonged **APTT** but a normal PT, similar to the patient's presentation. However, vWD is more common and can also present with similar findings. *Haemophilia B* - This is a deficiency of **Factor IX**, also part of the **intrinsic pathway** [1]. - Haemophilia B also causes a prolonged **APTT** but a normal PT. However, vWD is a more common inheritable bleeding disorder.

Question 3: Which of the following statements regarding von Willebrand disease is incorrect?

A. Type 2 von Willebrand disease is associated with a moderate bleeding tendency.
B. Type 3 von Willebrand disease is associated with severe deficiency of factor VIII.
C. Type 1 von Willebrand disease presents with severe bleeding since childhood. (Correct Answer)
D. Type 2 von Willebrand disease includes subtypes with varying defects in von Willebrand factor.

Explanation: ***Type 1 von Willebrand disease presents with severe bleeding since childhood.*** - This statement is incorrect because **Type 1 von Willebrand disease (vWD)** is characterized by a **partial quantitative deficiency** of von Willebrand factor (vWF), leading to **mild to moderate bleeding** that typically presents later in life or with specific challenges like surgery. - **Severe bleeding since childhood** is more characteristic of **Type 3 vWD**, which involves a near-complete absence of vWF. *Type 2 von Willebrand disease is associated with a moderate bleeding tendency.* - This statement is correct as **Type 2 vWD** involves **qualitative defects** in vWF, meaning the protein is present but doesn't function correctly [1]. - This typically results in a **moderate bleeding tendency**, which can vary depending on the specific subtype (2A, 2B, 2M, 2N). *Type 3 von Willebrand disease is associated with severe deficiency of factor VIII.* - This statement is correct because **Type 3 vWD** is characterized by a near-complete absence of vWF, which is essential for stabilizing **Factor VIII** in circulation [2]. - The severe deficiency of vWF leads to a **marked decrease in Factor VIII levels**, resulting in a bleeding phenotype that can be difficult to distinguish from severe hemophilia A. *Type 2 von Willebrand disease includes subtypes with varying defects in von Willebrand factor.* - This statement is correct as **Type 2 vWD** is subdivided into four main types (2A, 2B, 2M, 2N), each with distinct **qualitative defects** in the von Willebrand factor protein [2]. - These subtypes are differentiated by issues such as **multimerization defects**, increased platelet binding, or decreased affinity for Factor VIII [2].

Question 4: Glanzmann thrombasthenia is due to

A. Anti-GpIIb/IIIa antibodies
B. Decreased GpIIb/IIIa (Correct Answer)
C. Decreased GpIb
D. Inhibition or deficiency of ADAMTS 13

Explanation: ***Decreased GpIIb/IIIa*** - Glanzmann thrombasthenia is characterized by a **deficiency or dysfunction of the GpIIb/IIIa complex** [1], leading to impaired platelet aggregation. - The lack of this receptor prevents **fibrinogen binding** [1][2], which is essential for platelet clumping. *Anti-GpIIb/IIIa antibodies* - This option relates to **immune-mediated disorders** like **thrombocytopenic purpura**, not Glanzmann thrombasthenia. - In Glanzmann thrombasthenia, there is an actual deficiency, not antibodies against the receptor. *Inhibition or deficiency of ADAMTS 13* - This deficiency is associated with **Thrombotic Thrombocytopenic Purpura (TTP)**, impacting von Willebrand factor, not platelet aggregation directly. - TTP results in excessive platelet activation, unlike Glanzmann thrombasthenia, which results in inability to aggregate. *Decreased GpIb* - The decrease in GpIb typically relates to **Bernard-Soulier syndrome** [1], which causes defective platelet adhesion to von Willebrand factor. - Glanzmann thrombasthenia specifically involves the GpIIb/IIIa complex rather than GpIb itself. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, p. 128.

Question 5: Factor V mutation most commonly initially presents as:-

A. Thrombosis
B. Disseminated Intravascular Coagulation (DIC)
C. Pulmonary Embolism
D. Deep Vein Thrombosis (DVT) (Correct Answer)

Explanation: ***Deep Vein Thrombosis (DVT)*** - Factor V Leiden mutation is a common inherited **thrombophilia**, significantly increasing the risk of **venous thromboembolism (VTE)**. - While VTE encompasses DVT and pulmonary embolism, **DVT is the most frequent initial presentation** because it is the primary thrombotic event leading to other complications [2]. *Thrombosis* - This is a general term for the formation of a **blood clot** that obstructs blood flow. - While Factor V Leiden causes thrombosis, **DVT is a specific and common type** of thrombosis that typically presents first [1]. *Disseminated Intravascular Coagulation (DIC)* - DIC is a complex, life-threatening condition characterized by widespread activation of coagulation leading to both **thrombosis and hemorrhage**. - It is typically triggered by severe underlying conditions like sepsis or trauma, and is **not a primary presentation of Factor V Leiden mutation**. *Pulmonary Embolism (PE)* - PE occurs when a **blood clot travels to the lungs**, often originating from a DVT. - While Factor V Leiden increases PE risk, **DVT is usually the antecedent event** and thus the more common initial clinical presentation [1].

Question 6: Following injury to a blood vessel, immediate hemostasis is achieved by:

A. Fibrin deposition
B. Vasoconstriction (Correct Answer)
C. Platelet adhesion
D. Thrombosis

Explanation: ***Vasoconstriction*** - Following blood vessel injury, **vasoconstriction** occurs immediately, reducing blood flow and minimizing blood loss. - It is a **reflex response** mediated by local factors and neural mechanisms aiming to maintain hemostasis. *Fibrin deposition* - **Fibrin deposition** occurs later in the hemostatic process, primarily during the **coagulation phase** after initial vascular responses. - It is not an immediate response; rather, it's part of the **clot stabilization** process, requiring activation of the clotting cascade. *Thrombosis* - Thrombosis refers to the formation of a **blood clot** within a vessel, which happens after initial hemostatic mechanisms are activated. - It is not the **immediate** response post-injury; there is a sequence of events that lead to thrombosis after vasoconstriction and platelet activation. *Platelet adhesion* - Although platelet adhesion is crucial in hemostasis, it occurs following **vasoconstriction** and is not an immediate response to vessel injury [1][2]. - This process is part of the **primary hemostasis** phase, which cannot occur efficiently without prior initial vasoconstriction. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 581-582. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, p. 128.

Question 7: Glanzmann thrombasthenia is due to defect in:-

A. Gp VI
B. Thromboxane A2
C. Gp Ia/IIa
D. Gp IIb/IIIa (Correct Answer)

Explanation: ***Gp IIb/IIIa*** - Glanzmann thrombasthenia is a **rare, inherited bleeding disorder** characterized by a defect or deficiency in the **glycoprotein IIb/IIIa (Gp IIb/IIIa) complex** on the platelet surface [1]. - This complex is crucial for platelet aggregation as it acts as the receptor for **fibrinogen**, which links activated platelets together [1]. *Gp VI* - **Glycoprotein VI (Gp VI)** is a collagen receptor on platelets, important for initial **platelet adhesion and activation** at sites of vascular injury. - Defects in Gp VI are associated with milder bleeding disorders, not Glanzmann thrombasthenia. *Thromboxane A2* - **Thromboxane A2 (TXA2)** is a potent **vasoconstrictor** and **platelet aggregator** synthesized by platelets. - Disorders in TXA2 synthesis or response, such as aspirin-induced platelet dysfunction, cause bleeding but are biochemically distinct from Glanzmann thrombasthenia. *Gp Ia/IIa* - The **glycoprotein Ia/IIa (Gp Ia/IIa) complex** (also known as integrin ̡2̢1) is another **collagen receptor** on platelets, mediating platelet adhesion to collagen. - Defects in Gp Ia/IIa lead to a different type of mild bleeding disorder, affecting initial adhesion rather than aggregation. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 668-669.

Question 8: A bone marrow aspirate shows blast cells with multiple Auer rods arranged in bundles ('faggot cells'). Which molecular finding would confirm acute promyelocytic leukemia?

A. BCR-ABL translocation
B. PML-RARA translocation (Correct Answer)
C. AML1-ETO translocation
D. inv(16) mutation

Explanation: PML-RARA translocation - The presence of **multiple Auer rods** in bundles, described as "faggot cells," is highly characteristic of **acute promyelocytic leukemia (APL)** [1], [2]. - APL is cytogenetically defined by the **t(15;17) translocation**, which fuses the **PML (promyelocytic leukemia) gene** on chromosome 15 with the **RARA (retinoic acid receptor alpha) gene** on chromosome 17 [1], [2], [3]. *BCR-ABL translocation* - This translocation, **t(9;22)**, creates the **Philadelphia chromosome** and is the hallmark of **chronic myeloid leukemia (CML)** [3]. - While it can be seen in some acute leukemias, it does not typically present with the characteristic **Auer rods** or "faggot cells" associated with APL. *AML1-ETO translocation* - This refers to the **t(8;21)** chromosomal translocation, a common cytogenetic abnormality found in **acute myeloid leukemia (AML) with maturation**, specifically AML M2 [2]. - While AML1-ETO AML can have Auer rods, it does not typically present with the bundles ("faggot cells") seen in APL [2]. *inv(16) mutation* - The **inv(16) inversion** is associated with **acute myelomonocytic leukemia with eosinophilia (AML M4Eo)** [2]. - This subtype of AML is characterized by **abnormal eosinophils** and can have Auer rods, but it does not present with the specific "faggot cell" morphology that points to APL [2]. **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. 620-622. [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, p. 620. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 605-607.

Question 9: All of the following are seen in polycythemia rubra vera except :

A. Increased platelets
B. Increased Vit B12 binding capacity (>9000 micromols/dL)
C. Leucocytosis
D. Decreased LAP Score (Correct Answer)

Explanation: ***Decreased LAP Score*** - **LAP (Leukocyte Alkaline Phosphatase) score** is typically **increased or normal** in polycythemia vera. - A decreased LAP score is characteristic of **chronic myeloid leukemia (CML)**, which must be differentiated from polycythemia vera [2]. *Increased platelets* - **Thrombocytosis** (increased platelets) is a common feature of **polycythemia vera**, often contributing to vascular complications [1], [2]. - The unregulated proliferation of all myeloid cell lines, including megakaryocytes, leads to this increase [1], [3]. *Increased Vit B12 binding capacity (>9000 micromols/dL)* - Polycythemia vera often leads to **increased vitamin B12 levels** and **binding capacity** due to increased production of **transcobalamin I** by proliferating granulocytes. - While not a direct diagnostic criterion, it is a frequent finding supportive of the diagnosis. *Leucocytosis* - **Leukocytosis** (increased white blood cell count), particularly granulocytosis, is a common feature of polycythemia vera [1], [2]. - It results from the **clonal proliferation** of myeloid stem cells, leading to an overproduction of all myeloid lineage cells [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 614-615. [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. 626-627. [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, pp. 627-628.

Question 10: Adenoidectomy is contraindicated in:

A. SOM
B. CSOM
C. Bleeding disorder (Correct Answer)
D. None of the options

Explanation: ***Bleeding disorder*** - Adenoidectomy involves surgical removal of tissue, which carries a risk of **intraoperative and postoperative bleeding**. - In individuals with a **pre-existing bleeding disorder**, this risk is significantly elevated, potentially leading to serious complications. *SOM* - **Serous otitis media (SOM)**, or otitis media with effusion, is often caused by Eustachian tube dysfunction, which can be exacerbated by adenoid hypertrophy. - Adenoidectomy can actually be a **treatment for recurrent SOM**, as it can relieve obstruction of the Eustachian tube. *CSOM* - **Chronic suppurative otitis media (CSOM)** involves a persistent perforation of the tympanic membrane with chronic ear discharge. - While adenoid hypertrophy can contribute to Eustachian tube dysfunction and recurrent acute otitis media that might lead to CSOM, an adenoidectomy is **not directly contraindicated** for CSOM itself. *None of the options* - This option is incorrect because **bleeding disorder** is a clear contraindication for adenoidectomy due to the increased risk of hemorrhagic complications.

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