Hematology Practice Questions

Q: What is the first-line treatment for severe or chronic idiopathic thrombocytopenia purpura (ITP)?

Corticosteroid therapy. ***Corticosteroid therapy*** - **Corticosteroids** are the cornerstone of first-line treatment for severe or chronic ITP due to their ability to suppress the **autoimmune destruction of platelets** [1]. - They work by reducing antibody production, improving platelet survival, and stabilizing endothelial integrity, thereby **decreasing bleeding risk** [1]. *Platelet transfusion* - **Platelet transfusions** are generally reserved for **life-threatening hemorrhage** in ITP, as transfused platelets are quickly destroyed by the same autoantibodies. - They are not a first-line treatment for chronic management because they do not address the underlying **immune-mediated destruction**. *Observation and monitoring* - **Observation and monitoring** may be appropriate for patients with **mild ITP** (platelet count >30,000/µL) and no significant bleeding symptoms. - However, for **severe or chronic ITP**, active treatment is required to prevent serious bleeding complications. *Splenectomy* - **Splenectomy** is a treatment option for **refractory ITP**, typically considered after failure of first-line therapies like corticosteroids [1]. - The spleen is the primary site of antiplatelet antibody production and platelet destruction, so its removal can be effective but carries surgical risks and is not an initial approach [1].

Q: Which of the following statements is true regarding Hemophilia A?

Deficiency of factor VIII. ***Serum levels of factor VIII are decreased*** - Hemophilia A is characterized by a **deficiency of factor VIII** [1], which leads to decreased serum levels of this factor. - This deficiency results in a **prolonged activated partial thromboplastin time (aPTT)** [3] but normal prothrombin time (PT). *Deficiency of factor IX* - This escribes **Hemophilia B**, which is caused by a deficiency of factor IX, not factor VIII as in Hemophilia A [1]. - Hemophilia A specifically refers to the **deficiency of factor VIII** [1][2], where factor IX is not involved. *PT increased* - In Hemophilia A, the **prothrombin time (PT)** is usually normal because the intrinsic pathway is not affected [3]. - The primary test affected is the **activated partial thromboplastin time (aPTT)**, which is prolonged due to factor VIII deficiency [3]. *FIT decreased* - The term "FIT" is not standard in the context of hemophilia; it might refer to some other tests or assessments not directly relevant to factor levels. - The relevant lab finding in Hemophilia A is the **decrease in factor VIII** [2], not a direct measure of "FIT".

Q: Which of the following can result in dactylitis

Sickle Cell Anemia. ***Sickle Cell Anemia*** - **Dactylitis**, often referred to as **hand-foot syndrome**, is a common early manifestation of **sickle cell anemia** in infants and young children [2]. - It results from **vaso-occlusion** in the small bones of the hands and feet, leading to **inflammation, pain, and swelling**. *Hemophilia* - Hemophilia is a bleeding disorder characterized by a deficiency in certain **clotting factors**, leading to uncontrolled bleeding. - It primarily causes **hemarthrosis** (bleeding into joints) and muscle hematomas, but not dactylitis. *Measles* - Measles is a viral infection characterized by a distinctive **rash, fever, cough, coryza, and conjunctivitis**. - It does not directly cause dactylitis; musculoskeletal symptoms are not typical of measles. *Von Willebrand disease* - This is the most common **inherited bleeding disorder**, caused by a deficiency or dysfunction of **von Willebrand factor** [1]. - Similar to hemophilia, it primarily manifests with **mucocutaneous bleeding** and **menorrhagia**, not dactylitis.

Q: In which of the following conditions is marked bleeding typically observed?

Thrombocytopenia. ***ALL*** - **Acute Lymphoblastic Leukemia (ALL)** often presents with significant **thrombocytopenia**, leading to **marked bleeding** due to the low platelet count. - The disease disrupts normal hematopoiesis in the bone marrow, contributing to leukopenia and anemia, which may also result in bleeding complications [1]. *Haemophilia B* - This condition is characterized by a deficiency of **Factor IX**, leading to bleeding, but typically presents with **mild to moderate** bleeding rather than marked bleeding [1]. - Bleeding often occurs following trauma or surgery, unlike the more significant bleeding seen in ALL [1]. *VMA disease* - VMA disease (likely referring to **Vanillylmandelic Acid**), associated with **neuroblastoma**, does not primarily cause bleeding issues but rather is related to catecholamine excretion. - Symptoms are more aligned with **tumor-related effects** rather than coagulopathy or bleeding diathesis. *Haemophilia A* - Similar to Haemophilia B, it involves a deficiency of a clotting factor (**Factor VIII**) and can cause bleeding, but it usually results in **moderate bleeding** rather than marked bleeding [1]. - Bleeding is generally more controlled, relating to specific injury rather than spontaneous significant hemorrhage [1].

Q: In cobalamin deficiency which is not seen

Microcytic anemia. Microcytic anemia - Cobalamin deficiency typically leads to macrocytic anemia due to impaired DNA synthesis, not microcytic anemia [1]. - Microcytic anemia is usually associated with iron deficiency, thalassemia, or anemia of chronic disease [1]. Long tract signs - Long tract signs are common in cobalamin deficiency due to posterior column and corticospinal tract involvement leading to symptoms like spasticity. - They indicate involvement of pathways that are affected by vitamin B12 deficiency. Loss of proprioception - Loss of proprioception can occur in cobalamin deficiency due to damage to the dorsal columns of the spinal cord. - It is a common clinical finding indicating the involvement of sensory pathways. Rhomberg sign - A positive Rhomberg sign indicates impaired proprioception, which can happen in cobalamin deficiency. - It reflects difficulty maintaining balance, emphasizing sensory dysfunction associated with the deficiency.

Q: CAR-T cell therapy (Chimeric Antigen Receptor T-cell therapy) is being investigated for the treatment of which malignancy?

Acute Lymphoblastic Leukemia. ***Acute Lymphoblastic Leukemia*** - **CAR T-cell therapy** has shown remarkable success, particularly in treating refractory or relapsed **B-cell acute lymphoblastic leukemia (ALL)** in children and young adults. - The therapy targets the **CD19 antigen** found on malignant B-cells, leading to their destruction by engineered T-cells. *Renal Cell Carcinoma* - While immune therapies are used for **renal cell carcinoma (RCC)**, traditional CAR T-cell therapy targeting specific antigens has not yet achieved widespread clinical success for this solid tumor. - RCC often presents with a **heterogeneous antigenic landscape**, making it challenging for single-target CAR T-cells. *Pancreatic Cancer* - **Pancreatic cancer** is a challenging malignancy due to its dense stroma and immunosuppressive microenvironment, which limits T-cell infiltration and efficacy. - CAR T-cell therapy for pancreatic cancer is still largely in **early-stage clinical trials**, facing significant hurdles in solid tumor treatment. *Glioblastoma Multiforme* - **Glioblastoma multiforme (GBM)** is an aggressive brain tumor with unique challenges for CAR T-cell therapy, including the **blood-brain barrier** and tumor heterogeneity. - Research is ongoing to develop CAR T-cells that can effectively target GBM, often using **regional delivery methods** or targeting multiple antigens.

Q: Chronic hemolytic anaemia is associated with which of the following -

Black Pigment stone of the gall bladder. ***Black Pigment stone of the gall bladder*** - Chronic hemolytic anemia leads to increased **bilirubin**, particularly unconjugated bilirubin, which can result in the formation of **black pigment stones** [1]. - These stones are associated with conditions causing **excess bilirubin production**, such as sickle cell disease and thalassemia. *Brown Pigment stone of the gall bladder* - Brown pigment stones are primarily associated with **infection** and **biliary tract disorders**, not directly with chronic hemolytic anemia. - They are mainly composed of **calcium bilirubinate**, which arises in cases of **bacterial infections** or parasitic infestations. *Intestinal Obstruction* - While hemolytic anemia can have various complications, it is **not directly linked with intestinal obstruction**. - Obstruction typically arises from **mechanical causes** such as adhesions, tumors, or hernias, rather than from hemolytic processes. *Uric acid Renal Calculus* - Uric acid stones are formed due to conditions leading to **hyperuricemia** and are not a primary consequence of chronic hemolytic anemia. - They are often associated with **gout** and **certain metabolic disorders**, rather than hemolytic processes.

Q: Therapeutic phlebotomy is not done in which of the following conditions?

CML. CML - **Chronic Myeloid Leukemia (CML)** is typically treated with targeted therapies like **Tyrosine Kinase Inhibitors** (e.g., Imatinib), not phlebotomy [1]. - Therapeutic phlebotomy is ineffective in managing the **hypercellularity** or symptoms associated with this condition compared to other conditions [1]. *Polycythemia vera* - Therapeutic phlebotomy is a key treatment in **Polycythemia vera** to reduce **hyperviscosity** symptoms. - This condition features increased red blood cell mass, which is directly addressed by phlebotomy. *Hemochromatosis* - In **Hemochromatosis**, phlebotomy is employed to lower **iron overload** by removing excess iron from the body. - This reduces the risk of complications such as **liver cirrhosis** and **diabetes** associated with iron excess. *Porphyria cutanea tarda* - Therapeutic phlebotomy is sometimes used in cases of **Porphyria cutanea tarda** to manage iron levels as a potential precipitating factor [2]. - It helps alleviate symptoms and prevent complications associated with **photosensitivity** and skin lesions [2].

Q: Gaisbock syndrome is known as:

Spurious Polycythemia. ***Spurious Polycythemia*** - Gaisbock syndrome is characterized by an increase in **red blood cells** due to **dehydration** and is a form of **spurious or relative polycythemia** [1]. - It typically occurs in individuals with **high hematocrit levels** without true pathological erythrocytosis [1]. *High Altitude Erythrocytosis* - This condition is caused by **hypoxia** at high altitudes leading to increased **erythropoietin**, resulting in genuine **erythrocytosis** [2]. - Unlike Gaisbock syndrome, it reflects a true physiological response to reduced oxygen levels [2]. *Polycythemia Vera* - Polycythemia vera is a **myeloproliferative disorder** characterized by increased red blood cell mass due to intrinsic bone marrow changes. - It entails elevated **erythropoietin** levels and features such as **splenomegaly** and other cytogenetic changes, which are not present in Gaisbock syndrome [1][3]. *Primary Familial Polycythemia* - This hereditary condition results in increased red blood cells due to genetic mutations leading to overproduction of erythrocytes. - It is distinct from Gaisbock syndrome as it typically manifests from genetic predisposition rather than environmental factors such as dehydration.

Q: Thrombocythemia is characterized by an elevated platelet count.

Elevated platelet count. Elevated platelet count - Thrombocythemia is a condition specifically defined by an abnormally high number of platelets (thrombocytes) in the blood [2]. - This elevated count can lead to issues with both bleeding and clotting [2]. Low platelets - Low platelets, also known as thrombocytopenia, is the opposite of thrombocythemia [1]. - This condition is associated with an increased risk of bleeding [1]. Neutrophilia - Neutrophilia refers to an elevated count of neutrophils, a type of white blood cell, which is typically seen in bacterial infections. - It does not directly describe the platelet count. Monocytosis - Monocytosis indicates an increase in monocytes, another type of white blood cell, often seen in chronic infections or inflammatory conditions. - This term is unrelated to platelet levels.

Question 1

What is the first-line treatment for severe or chronic idiopathic thrombocytopenia purpura (ITP)?

Accepted Answer

Corticosteroid therapy

Answer

Splenectomy

Answer

Platelet transfusion

Answer

Observation and monitoring

Question 2

Which of the following statements is true regarding Hemophilia A?

Accepted Answer

Deficiency of factor VIII

Answer

PT is typically prolonged

Answer

PTT is typically normal

Answer

Serum levels of factor VIII are increased

Question 3

Which of the following can result in dactylitis

Accepted Answer

Sickle Cell Anemia

Answer

Hemophilia

Answer

Measles

Answer

Von Willebrand disease

Question 4

In which of the following conditions is marked bleeding typically observed?

Accepted Answer

Thrombocytopenia

Answer

Von Willebrand disease

Answer

Hemophilia A

Answer

Hemophilia B

Question 5

In cobalamin deficiency which is not seen

Accepted Answer

Microcytic anemia

Answer

Loss of proprioception

Answer

Rhomberg sign

Answer

Long tract signs

Question 6

CAR-T cell therapy (Chimeric Antigen Receptor T-cell therapy) is being investigated for the treatment of which malignancy?

Accepted Answer

Acute Lymphoblastic Leukemia

Answer

Renal Cell Carcinoma

Answer

Pancreatic Cancer

Answer

Glioblastoma Multiforme

Question 7

Chronic hemolytic anaemia is associated with which of the following -

Accepted Answer

Black Pigment stone of the gall bladder

Answer

Brown Pigment stone of the gall bladder

Answer

Uric acid Renal Calculus

Answer

Intestinal Obstruction

Question 8

Therapeutic phlebotomy is not done in which of the following conditions?

Accepted Answer

CML

Answer

Polycythemia vera

Answer

Hemochromatosis

Answer

Porphyria cutanea tarda

Question 9

Gaisbock syndrome is known as:

Accepted Answer

Spurious Polycythemia

Answer

Primary Familial Polycythemia

Answer

High Altitude Erythrocytosis

Answer

Polycythemia Vera

Question 10

Thrombocythemia is characterized by an elevated platelet count.

Accepted Answer

Elevated platelet count

Answer

Low platelets

Answer

Neutrophilia

Answer

Monocytosis

Hematology — MCQs

Hematology — MCQs

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