Hematology Practice Questions

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: Most common cause of febrile non haemolytic transfusion reaction?

HLA mismatch. ***HLA mismatch*** - **Febrile non-hemolytic transfusion reactions (FNHTR)** are primarily caused by the recipient's antibodies reacting against donor leukocyte antigens (HLAs) [1]. - This reaction leads to the release of **pyrogenic cytokines** from donor leukocytes stored in the blood product, causing fever and chills [1]. *Rh mismatch* - **Rh mismatch** primarily causes **hemolytic transfusion reactions**, characterized by red blood cell destruction, not just fever without hemolysis [1]. - This type of reaction is typically severe and involves antibody-mediated red cell lysis [1]. *ABO mismatch* - **ABO mismatch** leads to the most severe and often fatal **acute hemolytic transfusion reactions**, involving rapid intravascular hemolysis [1]. - This is a direct immune response against incompatible red blood cell antigens, resulting in hemoglobinuria, renal failure, and shock, not just fever [1]. *All of the options* - This option is incorrect because while all listed conditions relate to transfusion, only **HLA mismatch** commonly causes FNHTRs. - **Rh and ABO mismatches** are associated with distinct and more severe hemolytic reactions.

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.

Q: A 59-year-old male came with Hb 18.0 gm/dl on three occasions. The resident doctor wants to exclude Polycythemia Vera. Which of the following is the most relevant investigation :

Hematocrit. **Hematocrit** - The **hematocrit** measures the percentage of red blood cells in the blood and is directly reflective of the **red cell mass**, which is crucially elevated in polycythemia vera [1]. - A persistently high hemoglobin level of 18.0 gm/dL warrants further evaluation of the red cell mass, and **hematocrit** is the most direct and initial step to confirm true erythrocytosis before pursuing more specific tests [1]. *Reticulocyte count* - **Reticulocyte count** measures the number of immature red blood cells and primarily assesses the bone marrow's response to anemia, not polycythemia. - In polycythemia vera, the erythropoiesis is unregulated and uncontrolled, but a high reticulocyte count is not a primary diagnostic criterion. *JAK2 V617F mutation testing* - This is a highly specific test for **Polycythemia Vera**, as the **JAK2 V617F mutation** is present in about 95% of patients with the condition [1]. - While essential for definitive diagnosis, it should be performed after demonstrating an unexplained persistent increase in **red cell mass** or hemoglobin/hematocrit, as suggested by the initial blood tests [1]. *Serum erythropoietin (EPO) levels* - **Serum EPO levels** are typically suppressed or low in **Polycythemia Vera** due to the constitutive activation of the JAK2 pathway, which makes erythropoiesis independent of EPO. - While an important diagnostic marker, it is usually assessed after confirming an elevated red cell mass and before more specific genetic testing.

Q: A 39-year-old man is undergoing resuscitation with blood products for an upper GI bleed. He is suspected of having a hemolytic transfusion reaction. Which of the following is appropriate in the management of this patient?

Fluids and mannitol. ***Fluids and mannitol*** - **Aggressive intravenous fluids** are crucial to maintain renal perfusion and prevent acute kidney injury by flushing out free hemoglobin [1]. - **Mannitol** is an osmotic diuretic that promotes renal excretion of hemoglobin and prevents tubular obstruction; it should be used cautiously to avoid fluid overload [1]. *Removal of nonessential foreign body irritants, for example, Foley catheter* - While **infection control** is generally important, removing a Foley catheter is not a primary or direct intervention for managing a **hemolytic transfusion reaction**. - A Foley catheter actually assists in monitoring **urine output**, which is critical for assessing renal function during a hemolytic transfusion reaction [1]. *0.1 M HCl infusion* - **Hydrochloric acid (HCl) infusion** would cause severe **acidosis** and is not indicated in the management of a hemolytic transfusion reaction. - The focus is on **maintaining blood pressure**, **renal perfusion**, and addressing potential **coagulopathy**, not altering systemic pH with strong acids. *Fluid restriction* - **Fluid restriction** would be detrimental in a patient with a hemolytic transfusion reaction, as it can worsen **hypovolemia**, **renal hypoperfusion**, and accelerate acute kidney injury. - **Aggressive fluid hydration** is essential to help excrete hemolyzed products and maintain kidney function [1].

Q: Platelet transfusion is not indicated in:

Immunogenic Thrombocytopenia. ***Immunogenic Thrombocytopenia*** - In **immune thrombocytopenia (ITP)**, antibodies destroy platelets, making transfused platelets also susceptible to destruction, thus offering minimal benefit [1]. - Platelet transfusions are generally avoided in ITP unless there is **life-threatening bleeding** that is refractory to other treatments like corticosteroids or IVIG. *DIC* - **Disseminated intravascular coagulation (DIC)** involves widespread activation of the clotting cascade, leading to consumption of platelets and clotting factors [1]. - Platelet transfusions are often indicated in DIC when there is **significant bleeding** or a high risk of bleeding, especially with platelet counts below 20-50 x 10^9/L [2]. *Dilutional Thrombocytopenia* - This condition occurs due to **massive transfusion of packed red blood cells (PRBCs)** or fluids that lack platelets, diluting the patient's own circulating platelets. - Platelet transfusions are often indicated to **replenish platelet counts** and prevent bleeding in patients receiving large volume transfusions. *Aplastic Anemia* - **Aplastic anemia** is characterized by pancytopenia, including profound thrombocytopenia due to bone marrow failure. - Platelet transfusions are frequently necessary to **prevent or manage bleeding complications** in patients with severe aplastic anemia, especially during episodes of active bleeding or before invasive procedures.

Q: All of the following statements are true about Hemolytic uramic syndrome except -

Positive coomb's test. ***Positive Coomb's test*** - A **positive Coomb's test** indicates an **autoimmune hemolytic anemia**, where antibodies bind to red blood cells, causing their destruction. - HUS involves **microangiopathic hemolytic anemia**, which is typically **non-immune mediated** and thus has a negative Coomb's test [2]. *Thrombocytopenia* - **Thrombocytopenia** (low platelet count) is a key feature of HUS, caused by the consumption of platelets within widespread microthrombi in the microvasculature [1], [2]. - This leads to both bleeding complications and the characteristic microangiopathic hemolytic anemia. *Hypofibrinogenemia* - **Hypofibrinogenemia** can occur in severe HUS due to the consumption of fibrinogen during the formation of extensive microthrombi. - While not universally present, reduced fibrinogen levels reflect the ongoing thrombotic process. *Uremia* - **Uremia**, or elevated blood urea nitrogen (BUN) and creatinine, is a hallmark of HUS due to **acute kidney injury** [1]. - The microvascular damage in the kidneys leads to reduced glomerular filtration and accumulation of waste products.

Q: Rosenthal's syndrome is due to deficiency of

XI. XI - **Rosenthal's syndrome**, also known as **Hemophilia C**, is an autosomal recessive disorder caused by a deficiency of **Factor XI (FXI)**. - This deficiency leads to a mild bleeding disorder, often characterized by **post-traumatic or postsurgical bleeding**, but spontaneous bleeding is rare. IX - Deficiency of **Factor IX** causes **Hemophilia B** (Christmas disease), an X-linked recessive bleeding disorder [1]. - Hemophilia B typically causes **moderate to severe bleeding** symptoms, including spontaneous joint and muscle bleeds [1]. VIII - Deficiency of **Factor VIII** causes **Hemophilia A** (classic hemophilia), the most common severe X-linked recessive bleeding disorder [1]. - Patients with Hemophilia A experience diverse bleeding manifestations, including **hemarthroses** (joint bleeding) and **deep muscle hematomas** [1]. XII - Deficiency of **Factor XII (Hageman factor)** is associated with a **prolonged activated partial thromboplastin time (aPTT)** but generally does not cause a bleeding disorder. - Patients with F XII deficiency are often **asymptomatic** and do not experience abnormal bleeding, though some may have an increased risk of thrombosis.

Q: Cause of ITP is

Antibody to platelets. The cause of ITP is: ***Antibody to platelets*** - **Immune thrombocytopenic purpura (ITP)** is an autoimmune disorder [1] characterized by the destruction of platelets due to the presence of **autoantibodies**, primarily targeting platelet surface glycoproteins like **GPIIb/IIIa**. - These antibodies lead to premature destruction or increased consumption [1] of platelets by the reticuloendothelial system, particularly in the spleen, resulting in **thrombocytopenia**. *Vasculitis* - **Vasculitis** is inflammation of the blood vessels, which can cause symptoms like purpura but typically does not primarily cause isolated severe thrombocytopenia as seen in ITP. - While it can lead to bleeding manifestations, the underlying mechanism is vascular inflammation, not direct platelet destruction by antibodies. *Antibody to vascular epithelium* - Antibodies to **vascular endothelium** are seen in conditions such as some forms of vasculitis or autoimmune disorders like lupus, but they directly target vessel walls, not platelets. - This typically leads to endothelial damage and inflammation, rather than isolated thrombocytopenia from platelet destruction. *Antibody to clotting factors* - Antibodies to **clotting factors** (e.g., Factor VIII inhibitors) cause **hemophilia-like bleeding disorders** by interfering with the coagulation cascade. - This mechanism results in impaired clot formation, not primarily in low platelet counts as is characteristic of ITP.

Question 1

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 2

Commonest site of extramedullary relapse of ALL:

Accepted Answer

CNS

Answer

Testis

Answer

Liver

Answer

Lung

Question 3

Most common cause of febrile non haemolytic transfusion reaction?

Accepted Answer

HLA mismatch

Answer

Rh mismatch

Answer

ABO mismatch

Answer

All of the options

Question 4

First investigation to be done in a patient with thrombocytopenia:

Accepted Answer

Platelet count

Answer

aPTT

Answer

Prothrombin time

Answer

Bleeding time

Question 5

A 59-year-old male came with Hb 18.0 gm/dl on three occasions. The resident doctor wants to exclude Polycythemia Vera. Which of the following is the most relevant investigation :

Accepted Answer

Hematocrit

Answer

Reticulocyte count

Answer

JAK2 V617F mutation testing

Answer

Serum erythropoietin (EPO) levels

Question 6

A 39-year-old man is undergoing resuscitation with blood products for an upper GI bleed. He is suspected of having a hemolytic transfusion reaction. Which of the following is appropriate in the management of this patient?

Accepted Answer

Fluids and mannitol

Answer

Removal of nonessential foreign body irritants, for example, Foley catheter

Answer

0.1 M HCl infusion

Answer

Fluid restriction

Question 7

Platelet transfusion is not indicated in:

Accepted Answer

Immunogenic Thrombocytopenia

Answer

DIC

Answer

Dilutional Thrombocytopenia

Answer

Aplastic Anemia

Question 8

All of the following statements are true about Hemolytic uramic syndrome except -

Accepted Answer

Positive coomb's test

Answer

Thrombocytopenia

Answer

Hypofibrinogenemia

Answer

Uraemia

Question 9

Rosenthal's syndrome is due to deficiency of

Accepted Answer

XI

Answer

IX

Answer

VIII

Answer

XII

Question 10

Cause of ITP is

Accepted Answer

Antibody to platelets

Answer

Vasculitis

Answer

Antibody to vascular epithelium

Answer

Antibody to clotting factors

Hematology — MCQs

Hematology — MCQs

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