Hematology Practice Questions

Q: A 45-year-old female with isolated thrombocytopenia (platelet count 20,000) requires the best test to evaluate bone marrow response. What is the appropriate test?

IPF. ### IPF - **Immature Platelet Fraction (IPF)** is a direct measure of the proportion of newly produced, young platelets in the peripheral blood, reflecting the current rate of **megakaryopoiesis** and bone marrow's response to thrombocytopenia. - A high IPF in the setting of decreased peripheral platelet count indicates an **adequate bone marrow response** to platelet destruction or consumption [1]. *Plt-associated IgG Ab* - **Platelet-associated IgG antibodies** are relevant for diagnosing **immune thrombocytopenic purpura (ITP)**, by indicating platelet destruction [1]. - While this test helps diagnose the cause of thrombocytopenia, it does not directly evaluate the **bone marrow's production capacity**. *vWF activity* - **Von Willebrand factor (vWF) activity** measures the function of vWF, which is crucial for **platelet adhesion** and aggregation. - This test is used to diagnose **von Willebrand disease**, a cause of bleeding disorders, but it does not assess bone marrow platelet production [1]. *RPI* - The **reticulocyte production index (RPI)** is a measure of the effective bone marrow **erythroid response** to anemia. - While useful for evaluating red blood cell production, the RPI does not provide information about the **bone marrow's platelet production**.

Q: A 55-year-old woman presents with fatigue, pallor, numbness and tingling in her hands and feet, and a high mean corpuscular volume (MCV). A peripheral smear shows hypersegmented neutrophils and oval macrocytes. What is the most likely deficiency?

Vitamin B12. ***Vitamin B12*** - **Fatigue**, **pallor**, and **pancytopenia** are characteristic of megaloblastic anemia, which can be caused by **Vitamin B12 deficiency** [1]. - **Numbness** and **tingling** (neuropathy) are specific to **Vitamin B12 deficiency**, as it affects myelin synthesis, and the peripheral smear findings of **hypersegmented neutrophils** and **oval macrocytes** indicate impaired DNA synthesis during erythropoiesis [1]. *Iron* - **Iron deficiency anemia** typically presents with a **microcytic hypochromic anemia** (low MCV), in contrast to the high MCV seen here [1]. - Neurological symptoms like numbness and tingling are not characteristic of **iron deficiency**. *Folate* - **Folate deficiency** also causes **macrocytic anemia** with **hypersegmented neutrophils** and **oval macrocytes**, similar to B12 deficiency [2]. - However, **folate deficiency** does not typically cause the **neurological symptoms** (numbness and tingling) described, which are a key differentiating factor [1]. *Vitamin C* - **Vitamin C deficiency** leads to **scurvy**, characterized by **gingival bleeding**, **petechiae**, and impaired wound healing. - It can cause **anemia**, but it is usually **normocytic or macrocytic** due to impaired iron absorption or megaloblastic changes, not the primary cause of the neurological symptoms or the specific peripheral smear findings seen here.

Q: Which of the following is the most appropriate initial treatment for a patient with newly diagnosed essential thrombocythemia?

Hydroxyurea. ***Hydroxyurea*** - **Hydroxyurea** is a first-line treatment for essential thrombocythemia (ET), particularly in high-risk patients, due to its effectiveness in reducing **platelet counts** and preventing **thrombotic events**. [1] - It works by inhibiting DNA synthesis, thus suppressing megakaryocyte proliferation and platelet production in the **bone marrow**. *Anagrelide* - **Anagrelide** is an alternative agent that specifically lowers platelet counts by inhibiting megakaryocyte maturation and differentiation. - It is typically reserved for patients who are **intolerant to hydroxyurea** or when hydroxyurea fails to achieve adequate platelet control. *Interferon alfa* - **Interferon alfa** can be used to treat ET, especially in younger patients or those with a desire for future pregnancy, as it is non-mutagenic. - Its use is often limited by significant **side effects** like fatigue, flu-like symptoms, and mood disturbances, making it less ideal for initial therapy in most cases. *Plateletpheresis* - **Plateletpheresis** is a procedure used for rapid, temporary reduction of extremely high platelet counts in patients with **acute thrombotic** or **hemorrhagic complications**. - It is an emergency measure and not a chronic, long-term treatment for essential thrombocythemia due to its temporary effect and the need for specialized equipment.

Q: A 55-year-old male with fatigue and a palpable spleen is found to have a high white cell count predominantly consisting of mature lymphocytes. What is the most likely diagnosis?

Chronic lymphocytic leukemia. ***Chronic lymphocytic leukemia*** - The presence of **fatigue**, a **palpable spleen**, and an elevated white cell count predominantly consisting of **mature lymphocytes** is highly characteristic of CLL [1]. - CLL is a common leukemia in older adults, where **monoclonal B-lymphocytes** accumulate in the blood, bone marrow, and lymphoid organs [1]. *Acute lymphoblastic leukemia* - This leukemia typically presents with **immature lymphocytes (blasts)** and a more aggressive clinical course, often seen in children [2]. - While it causes fatigue and can lead to organomegaly, the predominance of **mature lymphocytes** in the peripheral blood rules out ALL [2]. *Myelodysplastic syndrome (ineffective hematopoiesis and cytopenias)* - This syndrome is characterized by **ineffective hematopoiesis** and **cytopenias** (low blood cell counts), rather than a high white cell count. - It involves dysplasia in one or more myeloid cell lines, and the peripheral smear would show abnormal cell morphology, not a predominance of mature lymphocytes. *Hairy cell leukemia (characterized by "hairy" appearing cells)* - While it presents with **splenomegaly** and can cause fatigue, the characteristic cells have **"hairy" cytoplasmic projections** and are rarely found in high numbers in the peripheral blood initially. - The diagnosis is confirmed by flow cytometry and bone marrow biopsy, and the classic picture often includes **pancytopenia**, not just a high white cell count from mature lymphocytes.

Q: In anemia of chronic disease, which iron parameter is typically elevated?

Ferritin. ***Ferritin*** - In **anemia of chronic disease (ACD)**, inflammation leads to increased production of **hepcidin**, which blocks iron release from stores [1]. - As a result, iron is trapped within macrophages and liver cells, causing **elevated ferritin levels**, which reflects increased iron stores despite functional iron deficiency. *Serum iron* - **Serum iron** levels are typically **decreased** in ACD because hepcidin inhibits iron absorption from the gut and release from macrophages [1]. - This reduction in circulating iron contributes to the anemia, as less iron is available for erythropoiesis. *Total iron-binding capacity* - **Total iron-binding capacity (TIBC)**, which primarily reflects **transferrin levels**, is usually **decreased** or normal in ACD [2]. - This is in contrast to iron deficiency anemia, where transferrin production increases to try and capture more iron. *Transferrin* - **Transferrin levels** are typically **decreased** or normal in ACD due to inflammation's suppressive effect on liver protein synthesis. - Reduced transferrin contributes to the decreased TIBC and further limits the transport of iron to erythroid precursors.

Q: A 50-year-old male presents with fatigue and pallor. His peripheral smear shows hypersegmented neutrophils and macro-ovalocytes. What is the most likely diagnosis?

Folate deficiency anemia. ***Folate deficiency anemia*** - The presence of **hypersegmented neutrophils** and **macro-ovalocytes** on peripheral smear is characteristic of **megaloblastic anemia**, which can be caused by folate deficiency. - **Fatigue** and **pallor** are common symptoms of anemia. *Iron deficiency anemia* - This typically presents with **microcytic, hypochromic** red blood cells, which is contrary to the macro-ovalocytes seen in this patient. - Iron deficiency does not cause **hypersegmented neutrophils**. *Thalassemia* - Thalassemia usually causes **microcytic, hypochromic anemia** and is characterized by abnormal hemoglobin production. - It does not involve **hypersegmented neutrophils** or **macro-ovalocytes**. *Chronic myeloid leukemia* - This condition is characterized by an overproduction of **granulocytes**, often with a **left shift** in the myeloid lineage. - It does not typically present with **hypersegmented neutrophils** and **macro-ovalocytes** as the primary diagnostic features.

Q: A beta-thalassemia patient was given 125 units of packed red blood cells. Which additional drug should be administered?

Desferroxamine. ***Desferroxamine*** - Patients with **beta-thalassemia** often require frequent **packed red blood cell transfusions** to manage severe anemia. - These transfusions lead to **iron overload** (hemosiderosis), necessitating iron chelation therapy with drugs like **Desferroxamine** to prevent organ damage. *Cytarabine* - **Cytarabine** is a **chemotherapeutic agent** primarily used in the treatment of various leukemias, particularly **acute myeloid leukemia**. - It is not indicated for the management of **iron overload** in thalassemia patients. *Hydroxyurea* - **Hydroxyurea** is used to increase **fetal hemoglobin** production in conditions like **sickle cell anemia** and some forms of beta-thalassemia [1]. - While it can reduce transfusion requirements in some thalassemia patients, its primary role is not to address **iron overload** already caused by transfusions. *Butyrates* - **Butyrates** are being investigated for their potential to induce **fetal hemoglobin** production, similar to hydroxyurea, in hemoglobinopathies. - They do not directly address **iron overload** and are not standard therapy for this complication.

Q: A 60-year-old man presents with fatigue, weight loss, and bone pain. Laboratory tests reveal anemia, hypercalcemia, and renal insufficiency. What is the most likely diagnosis?

Multiple myeloma. ***Multiple myeloma*** - The constellation of **fatigue (due to anemia)**, **weight loss**, **bone pain**, **hypercalcemia**, and **renal insufficiency** is characteristic of multiple myeloma, often described by the mnemonic **CRAB criteria** (Calcium elevation, Renal failure, Anemia, Bone lesions) [1, 2]. - This plasma cell malignancy leads to overproduction of **monoclonal immunoglobulins** (M-proteins) which can cause kidney damage, and osteolytic lesions leading to bone pain and hypercalcemia [1, 2]. *Chronic lymphocytic leukemia* - While it can present with fatigue and weight loss, specific features like **bone pain**, **hypercalcemia**, and **renal insufficiency** are not typical primary manifestations. [3] - Diagnosis usually involves **lymphocytosis** and the presence of **clonal B-cells** with specific immunophenotypes. *Non-Hodgkin lymphoma* - Patients often present with **lymphadenopathy**, **fever**, **night sweats**, and **weight loss**, but **hypercalcemia** and **renal insufficiency** due to direct bone involvement or myeloma kidney are less common. - Bone pain might occur if there's extensive **bone marrow involvement**, but it's not the primary feature compared to myeloma. *Primary hyperparathyroidism* - This condition primarily causes **hypercalcemia** and can lead to **bone pain** (osteitis fibrosa cystica) and **renal stones/insufficiency**. - However, **anemia** and significant **weight loss** are not typical presenting features; severe anemia is more indicative of a hematologic malignancy.

Question 1

A 45-year-old female with isolated thrombocytopenia (platelet count 20,000) requires the best test to evaluate bone marrow response. What is the appropriate test?

Accepted Answer

IPF

Answer

Plt-associated IgG Ab

Answer

vWF activity

Answer

RPI

Question 2

A 55-year-old woman presents with fatigue, pallor, numbness and tingling in her hands and feet, and a high mean corpuscular volume (MCV). A peripheral smear shows hypersegmented neutrophils and oval macrocytes. What is the most likely deficiency?

Accepted Answer

Vitamin B12

Answer

Iron

Answer

Folate

Answer

Vitamin C

Question 3

Which of the following is the most appropriate initial treatment for a patient with newly diagnosed essential thrombocythemia?

Accepted Answer

Hydroxyurea

Answer

Anagrelide

Answer

Interferon alfa

Answer

Plateletpheresis

Question 4

A 55-year-old male with fatigue and a palpable spleen is found to have a high white cell count predominantly consisting of mature lymphocytes. What is the most likely diagnosis?

Accepted Answer

Chronic lymphocytic leukemia

Answer

Acute lymphoblastic leukemia

Answer

Myelodysplastic syndrome (ineffective hematopoiesis and cytopenias)

Answer

Hairy cell leukemia (characterized by "hairy" appearing cells)

Question 5

In anemia of chronic disease, which iron parameter is typically elevated?

Accepted Answer

Ferritin

Answer

Serum iron

Answer

Total iron-binding capacity

Answer

Transferrin

Question 6

A 50-year-old male presents with fatigue and pallor. His peripheral smear shows hypersegmented neutrophils and macro-ovalocytes. What is the most likely diagnosis?

Accepted Answer

Folate deficiency anemia

Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Chronic myeloid leukemia

Question 7

A beta-thalassemia patient was given 125 units of packed red blood cells. Which additional drug should be administered?

Accepted Answer

Desferroxamine

Answer

Cytarabine

Answer

Hydroxyurea

Answer

Butyrates

Question 8

Which of the following conditions is not associated with venous and arterial thrombotic events?

Accepted Answer

Idiopathic Thrombocytopenic Purpura (ITP), a condition primarily causing bleeding.

Answer

Heparin-Induced Thrombocytopenia (HIT), a condition associated with immune-mediated thrombosis.

Answer

Paroxysmal Nocturnal Hemoglobinuria (PNH), a condition linked to hemolysis and thrombosis.

Answer

Disseminated Intravascular Coagulation (DIC), a condition involving widespread clotting and bleeding.

Question 9

A 60-year-old man presents with fatigue, weight loss, and bone pain. Laboratory tests reveal anemia, hypercalcemia, and renal insufficiency. What is the most likely diagnosis?

Accepted Answer

Multiple myeloma

Answer

Chronic lymphocytic leukemia

Answer

Non-Hodgkin lymphoma

Answer

Primary hyperparathyroidism

Question 10

A 54-year-old male presents with fever, confusion, and petechiae. Laboratory results show decreased platelets, increased PT/INR, increased D-dimer, and decreased fibrinogen. Analyze and determine the diagnosis and appropriate initial management.

Accepted Answer

Disseminated intravascular coagulation; treat underlying cause

Answer

Thrombocytopenic purpura; plasma exchange

Answer

Sepsis; broad-spectrum antibiotics

Answer

ITP; corticosteroids

Hematology — MCQs

Hematology — MCQs

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