Hematology Practice Questions

Q: The following condition is not associated with an Anti-phospholipid syndrome -

Thrombocytosis. ***Thrombocytosis*** - **Antiphospholipid syndrome (APS)** is characterized by a **prothrombotic state**, leading to **thrombocytopenia** (low platelet count), not thrombocytosis (elevated platelet count) [1]. - Elevated platelet counts are not part of the diagnostic criteria or typical clinical manifestations of APS. *Neurological manifestations* - **Neurological symptoms** are common in APS and can include **stroke**, **transient ischemic attacks (TIAs)**, **seizures**, and **cognitive dysfunction**. - These manifestations are due to the prothrombotic tendency affecting the cerebral vasculature. *Venous thrombosis* - **Venous thrombosis**, particularly **deep vein thrombosis (DVT)** and **pulmonary embolism (PE)**, is a hallmark feature of APS [2]. - The presence of antiphospholipid antibodies promotes clot formation in both venous and arterial systems. *Recurrent foetal loss* - **Recurrent foetal loss** (miscarriages or stillbirths) is a classic obstetric manifestation of APS [1]. - This occurs due to thrombosis in the placental circulation, impairing fetal development and viability [2].

Q: False about Marble bone disease

Associated with Type II Renal Tubular Acidosis. ***Associated with Type II Renal Tubular Acidosis*** - **Marble bone disease**, or **osteopetrosis**, is characterized by increased bone density due to defective osteoclast function [1]. - It is **not typically associated** with Type II Renal Tubular Acidosis; instead, some forms of osteopetrosis, particularly carbonic anhydrase II deficiency, can lead to **Type I (distal) Renal Tubular Acidosis** [1]. *Pancytopenia* - **Osteopetrosis** leads to bone marrow encroachment, reducing the space available for hematopoiesis. - This often results in **pancytopenia** (anemia, leukopenia, and thrombocytopenia) due to bone marrow failure. *Treated with bone marrow transplantation* - **Bone marrow transplantation (BMT)** is a crucial treatment for severe forms of osteopetrosis, especially the infantile malignant autosomal recessive type. - BMT can introduce functional **osteoclast precursors** from the donor, which can then differentiate and restore bone resorption. *Mandible osteomyelitis* - Patients with **osteopetrosis** have bones that are dense but paradoxically brittle, making them prone to fractures and infections. - The **mandible** is particularly susceptible to **osteomyelitis** due to its dense structure and vascular compromise in osteopetrosis, often complicated by dental issues.

Q: Which of the following is false regarding transfusion-associated anaphylactic reactions?

Seen in IgG deficient individuals. ***Seen in IgG deficient individuals*** - Transfusion-associated **anaphylactic reactions** are most commonly seen in **IgA-deficient individuals** who develop **anti-IgA antibodies** and receive blood products containing IgA. - Anaphylaxis occurs when these pre-formed IgA antibodies react with donor IgA, leading to mast cell degranulation and severe allergic symptoms. *Different from allergy* - Transfusion-associated **anaphylactic reactions** are a severe form of allergic reaction, often distinguished by their **rapid onset** and life-threatening nature [1]. - While all allergies involve an immune response to an allergen, anaphylaxis represents the most extreme systemic manifestation. *Epinephrine is the drug of choice* - **Epinephrine** is indeed the **first-line treatment** for acute anaphylaxis, regardless of its cause, including transfusion-associated reactions [2]. - It acts rapidly to counteract the systemic effects of histamine and other mediators by acting on α and β adrenergic receptors [3]. *Washed blood products prevent it* - **Washing blood products** (e.g., packed red blood cells or platelets) is an effective strategy to **remove plasma proteins**, including IgA. - This is particularly crucial for patients with a known **IgA deficiency and anti-IgA antibodies** to prevent severe anaphylactic reactions.

Q: A patient presents with MCV = 60, Hb = 5 gm%, MCHC = 20 & PCV = 32%. What is the MOST COMMON cause of this type of anemia in developing countries?

C) Hookworm infection. ***Hookworm infection*** - The presented lab values (MCV = 60, Hb = 5 gm%, MCHC = 20) indicate severe **microcytic hypochromic anemia**, which is a classic presentation of **iron deficiency anemia** [1], [2]. - In developing countries, **chronic blood loss** due to **hookworm infection** is the most common cause of sustained iron deficiency, leading to this type of anemia [2], [3]. *Phenytoin* - **Phenytoin** is an anticonvulsant that can cause **megaloblastic anemia** due to **folate deficiency**, not microcytic hypochromic anemia. - Its effects on red blood cells typically result in an **increased MCV**, not a decreased one. *Blind loop syndrome* - **Blind loop syndrome** leads to bacterial overgrowth in the small intestine, which can cause **vitamin B12 deficiency** due to bacterial consumption of B12. - This deficiency results in **megaloblastic anemia** (high MCV), not microcytic hypochromic anemia. *Chronic kidney disease with normal iron studies* - **Chronic kidney disease** primarily causes **normocytic normochromic anemia** due to decreased erythropoietin production [4]. - While it causes anemia, the red blood cell indices (MCV, MCHC) would typically be within the normal range, unlike the **microcytic hypochromic** values observed here.

Q: A 20-year-old woman with a family history of von Willebrand disease is found to have an activated partial thromboplastin time (aPTT) of 78 (normal = 32) on routine testing prior to cholecystectomy. Further investigation reveals a prothrombin time (PT) of 13 (normal = 12), a platelet count of 350,000/mm³, and an abnormal bleeding time. Which of the following should be administered in the perioperative period?

Desmopressin (DDAVP). ***Desmopressin (DDAVP)*** - **Desmopressin** is the first-line treatment for **von Willebrand disease (vWD)** [1], especially for type 1, which this patient's profile suggests (prolonged aPTT, normal PT, normal platelet count, and abnormal bleeding time). - It works by stimulating the release of endogenous **von Willebrand factor (vWF)** and **factor VIII** from endothelial cells [1]. *Factor VIII* - While Factor VIII deficiency can cause prolonged aPTT, this patient's presentation with a family history of **vWD**, normal PT, and abnormal bleeding time points specifically to a **vWF** defect rather than isolated **Factor VIII** deficiency [1]. - Administration of direct **Factor VIII** is not the primary treatment for **vWD**, although vWF concentrate (which contains Factor VIII) may be used for severe cases [1]. *Platelets* - The patient's **platelet count is normal** (350,000/mm³), indicating that a platelet transfusion is not necessary. - **vWD** is a disorder of platelet adhesion, not a problem with platelet quantity. *Vitamin K* - **Vitamin K** is essential for the synthesis of coagulation factors II, VII, IX, and X, as well as proteins C and S. - A deficiency in **Vitamin K** typically results in a prolonged **PT** [2], which is normal in this patient.

Q: Which of these is not a risk factor for thromboembolism -

Regular exercise. ***Regular exercise*** - **Regular exercise** is a protective factor against thromboembolism, as it improves blood circulation and reduces venous stasis. - It also helps maintain a healthy weight and cardiovascular fitness, lowering overall risk. *Superficial thrombophlebitis* - While typically less serious than deep vein thrombosis (DVT), **superficial thrombophlebitis** can extend into deeper veins or serve as a risk marker for DVT. - Inflammation and clot formation in superficial veins can sometimes trigger or coexist with more significant thromboembolic events [1]. *Myocardial infarction* - A **myocardial infarction** increases the risk for thromboembolism due to cardiac wall motion abnormalities and subsequent eddy currents causing thrombus formation within the ventricle [2]. - The damaged heart muscle can also release pro-coagulant factors, further elevating the risk. *Estrogen therapy* - **Estrogen therapy**, particularly in higher doses or certain formulations, is known to increase the risk for venous and arterial thromboembolism. - This is due to stimulating the production of clotting factors and reducing the activity of natural anticoagulants.

Q: An 80-year-old asymptomatic man presents with a total leukocyte count of 100,000, with 80% lymphocytes and 20% PMCs. What is the most probable diagnosis?

CLL. CLL - The finding of a **very high total leukocyte count** predominantly composed of **lymphocytes** (80%), particularly in an **asymptomatic elderly man**, is highly characteristic of **Chronic Lymphocytic Leukemia (CLL)** [1]. - CLL is often diagnosed incidentally in older patients due to routine blood tests showing **marked lymphocytosis** [1]. CML - **Chronic Myeloid Leukemia (CML)** would typically present with a high leukocyte count primarily consisting of **granulocytes** (neutrophils, eosinophils, basophils), and their precursors, not lymphocytes [2]. - CML is usually associated with the **Philadelphia chromosome (BCR-ABL1 fusion gene)** and often causes symptoms like fatigue, weight loss, and splenomegaly [2]. TB - **Tuberculosis (TB)** can cause a change in leukocyte count, but it typically presents with a **mild to moderate leukocytosis** with a **neutrophilic predominance** or **monocytosis**, and can also cause **lymphopenia** in severe cases, not the marked lymphocytosis seen here [1]. - Patients with TB are usually **symptomatic** with fever, night sweats, cough, and weight loss [3]. HIV - **HIV infection** primarily targets **CD4+ T-lymphocytes**, leading to a progressive decline in their count, resulting in **lymphopenia**, not the extreme lymphocytosis observed in this case [1]. - While HIV can cause various hematological abnormalities, significant, sustained lymphocytosis is not a typical hallmark [1].

Q: Commonest site of extramedullary relapse of ALL:

CNS. ***CNS*** - The **central nervous system (CNS)** is the most common site for extramedullary relapse in **acute lymphoblastic leukemia (ALL)**, particularly in children. - This is due to the "sanctuary" status of the CNS, where chemotherapy drugs often have **poor penetration**, allowing leukemic cells to evade treatment [1]. *Testis* - While the **testis** is another significant site for extramedullary relapse in ALL, it is less common than the CNS. - Testicular relapse is more frequently seen in **boys** and can be part of a systemic relapse or an isolated event. *Liver* - **Hepatic involvement** in ALL is more often part of initial disease presentation or a widespread systemic relapse, rather than an isolated extramedullary relapse site. - While leukemic cells can infiltrate the liver, it is not considered a common "sanctuary" site for *isolated* relapse compared to the CNS. *Lung* - **Pulmonary involvement** in ALL typically indicates widespread disease or leukostasis, rather than an isolated extramedullary relapse. - The lung is rarely a primary site for isolated extramedullary relapse in ALL compared to sites like the CNS or testes.

Q: First investigation to be done in a patient with thrombocytopenia:

Platelet count. Platelet count - The definition of **thrombocytopenia** is a low **platelet count**. Therefore, a platelet count is the direct measure to confirm the presence and severity of thrombocytopenia [3]. - This test is fundamental in initial diagnosis and monitoring, as it directly assesses the number of **platelets** available for clotting. *aPTT* - **aPTT (activated partial thromboplastin time)** primarily assesses the **intrinsic and common pathways** of coagulation, not platelet quantity [1]. - While important for evaluating overall coagulation, it does not directly measure or confirm **thrombocytopenia**. *Prothrombin time* - **Prothrombin time (PT)** evaluates the **extrinsic and common pathways** of coagulation, providing information about factors like **Factor VII** and **Warfarin** effect [1]. - Like aPTT, it assesses **coagulation factor function** rather than platelet numbers. *Bleeding time* - **Bleeding time** assesses **platelet function** and **vessel wall interaction**, providing an indication of primary hemostasis [2]. - Although it can be prolonged in **thrombocytopenia**, it is an indirect measure and is less specific than a direct platelet count; it is also prone to variability and is not the first diagnostic test for thrombocytopenia itself.

Question 1

The following condition is not associated with an Anti-phospholipid syndrome -

Accepted Answer

Thrombocytosis

Answer

Neurological manifestations

Answer

Venous thrombosis

Answer

Recurrent foetal loss

Question 2

False about Marble bone disease

Accepted Answer

Associated with Type II Renal Tubular Acidosis

Answer

Pancytopenia

Answer

Treated with bone marrow transplantation

Answer

Mandible osteomyelitis

Question 3

Splenectomy is least useful in

Accepted Answer

Hereditary nonspherocytic haemolytic anaemia

Answer

Congenital elliptocytosis

Answer

Thalassemia major

Answer

Congenital spherocytic anaemia

Question 4

Which of the following is false regarding transfusion-associated anaphylactic reactions?

Accepted Answer

Seen in IgG deficient individuals

Answer

Different from allergy

Answer

Epinephrine is the drug of choice

Answer

Washed blood products prevent it

Question 5

A patient presents with MCV = 60, Hb = 5 gm%, MCHC = 20 & PCV = 32%. What is the MOST COMMON cause of this type of anemia in developing countries?

Accepted Answer

C) Hookworm infection

Answer

A) Phenytoin

Answer

B) Blind loop syndrome

Answer

D) Chronic kidney disease with normal iron studies

Answer

Question 6

A 20-year-old woman with a family history of von Willebrand disease is found to have an activated partial thromboplastin time (aPTT) of 78 (normal = 32) on routine testing prior to cholecystectomy. Further investigation reveals a prothrombin time (PT) of 13 (normal = 12), a platelet count of 350,000/mm³, and an abnormal bleeding time. Which of the following should be administered in the perioperative period?

Accepted Answer

Desmopressin (DDAVP)

Answer

Factor VIII

Answer

Platelets

Answer

Vitamin K

Question 7

Which of these is not a risk factor for thromboembolism -

Accepted Answer

Regular exercise

Answer

Superficial thrombophlebitis

Answer

Myocardial infarction

Answer

Estrogen therapy

Question 8

An 80-year-old asymptomatic man presents with a total leukocyte count of 100,000, with 80% lymphocytes and 20% PMCs. What is the most probable diagnosis?

Accepted Answer

CLL

Answer

CML

Answer

TB

Answer

HIV

Question 9

Commonest site of extramedullary relapse of ALL:

Accepted Answer

CNS

Answer

Testis

Answer

Liver

Answer

Lung

Question 10

First investigation to be done in a patient with thrombocytopenia:

Accepted Answer

Platelet count

Answer

aPTT

Answer

Prothrombin time

Answer

Bleeding time

Hematology — MCQs

Hematology — MCQs

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