Hematology Practice Questions

Q: A boy after playing football complaining fatigue and abdominal pain. He also had a history of hand swelling in past. On ultrasonography, he has shrunken spleen. What is the likely diagnosis of this patient?

Sickle cell anemia. ### Sickle cell anemia - This patient's history of **hand swelling** (dactylitis), current **fatigue**, and **abdominal pain** are classic symptoms of sickle cell crises in **sickle cell anemia**. - The finding of a **shrunken spleen** on ultrasonography is consistent with **autosplenectomy**, a common complication of sickle cell disease due to repeated infarctions [1]. *Acute pancreatitis* - While acute pancreatitis can cause **severe abdominal pain**, it typically presents with elevated amylase/lipase, and does not explain the history of **hand swelling** or **shrunken spleen**. - There is no specific connection between playing football and developing acute pancreatitis in this context. *Iron deficiency anemia* - **Iron deficiency anemia** can cause **fatigue**, but it does not typically cause **abdominal pain**, **hand swelling**, or **autosplenectomy**. - A definitive diagnosis would require iron studies, which are not mentioned here. *Intermittent porphyria* - **Intermittent porphyria** can cause **abdominal pain** and neurological symptoms, but it does not manifest with **hand swelling** or **splenic atrophy**. - Its clinical presentation is distinctly different from the symptoms described.

Q: Factor V mutation most commonly initially presents as:-

Deep Vein Thrombosis (DVT). ***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].

Q: Which of the following is not true regarding Von Willebrand disease?

Hemarthrosis is the usual presentation. **Hemarthrosis is the usual presentation** - **Hemarthrosis** (bleeding into joints) is characteristic of severe factor deficiencies, such as **hemophilia A or B**, but is uncommon in von Willebrand disease (vWD) [2]. - vWD typically presents with **mucocutaneous bleeding** (e.g., easy bruising, nosebleeds, heavy menstrual bleeding) due to impaired platelet adhesion [2]. *Normal platelet count* - Patients with von Willebrand disease usually have a **normal platelet count**, as the issue is with the function or quantity of **von Willebrand factor (vWF)**, not the number of platelets [3]. - vWF primarily mediates platelet adhesion and protects **factor VIII** from degradation, so platelet production itself is unaffected [1]. *Quantitative defects are seen in subtypes 1 and 3 von Willebrand disease* - **Type 1 vWD** involves a partial **quantitative deficiency** of vWF, meaning reduced levels of otherwise normal vWF. - **Type 3 vWD** is characterized by a severe or near-complete **absence of vWF**, representing the most severe quantitative defect. *Produced by endothelial cells* - **Von Willebrand factor (vWF)** is primarily synthesized and stored in **endothelial cells** (in Weibel-Palade bodies) and also in **megakaryocytes**. - Its production by endothelial cells allows for its release into the bloodstream and subendothelial matrix to facilitate **hemostasis**.

Q: Which of the following hematological problems may be precipitated by parvovirus?

Aplastic crisis. ***Aplastic crisis*** - Parvovirus B19 preferentially infects and destroys **erythroid progenitor cells** in the bone marrow, leading to a temporary halt in red blood cell production [1]. - This can precipitate an **aplastic crisis**, especially in individuals with underlying chronic hemolytic anemias (e.g., **sickle cell disease** or **hereditary spherocytosis**), where red blood cell lifespan is already shortened [2]. *Hemolytic crisis* - A hemolytic crisis involves an **accelerated destruction of red blood cells**, leading to a sudden drop in hemoglobin. - While parvovirus can indirectly worsen anemia, it primarily affects red cell production rather than directly increasing their destruction, acting more as a trigger for a production problem than a hemolytic one [1]. *Thrombocytopenia* - **Thrombocytopenia** is characterized by a low platelet count, which can lead to bleeding disorders. - While viral infections can sometimes cause mild myelosuppression affecting platelets, parvovirus B19 is not typically associated with severe or primary thrombocytopenia. *Pancytopenia* - **Pancytopenia** is a reduction in all three blood cell lines (red blood cells, white blood cells, and platelets). - While parvovirus can cause a transient aplastic crisis (affecting red cells), it rarely causes a severe, sustained suppression of all cell lines to be classified as true pancytopenia, though some transient leukopenia may occur [1].

Q: Which of the following is the best treatment for refractory ITP?

Splenectomy. ***Splenectomy*** - **Splenectomy** is considered the most effective treatment for **refractory immune thrombocytopenic purpura (ITP)**, especially in patients who have failed multiple lines of medical therapy [1]. - The spleen is the primary site of **platelet destruction** and **antibody production** against platelets in ITP, so its removal can lead to a sustained increase in platelet count [1]. *Platelet transfusion* - While platelet transfusions can temporarily increase platelet counts, they are generally reserved for **acute, life-threatening bleeding** in ITP, not as a definitive treatment for refractory cases. - Transfused platelets are rapidly destroyed by the same **autoantibodies** that target endogenous platelets, making their effect short-lived. *Azathioprine* - **Azathioprine** is an **immunosuppressant** that can be used in ITP, but it is typically a **second-line** or **third-line agent** and its response takes time [1]. - It is not considered the "best" treatment for refractory ITP compared to splenectomy, which offers a more immediate and often sustained response [1]. *Prednisolone* - **Prednisolone** (corticosteroids) is often the **first-line treatment** for ITP due to its rapid immunosuppressive effects. - However, for **refractory ITP** (meaning it has not responded adequately to initial treatments), corticosteroids alone are usually insufficient to achieve long-term remission [1].

Q: A 65-year-old female with polycythemia vera on hydroxyurea for 2 years presents with painful bilateral leg ulcers for 3 months. She has well-controlled disease (Hct 42%), but recent CBC shows platelets 80,000/μL, WBC 3,500/μL. She also has diabetes (HbA1c 7.2%), peripheral vascular disease, and venous insufficiency. Doppler shows biphasic flow in dorsalis pedis. Most appropriate next step is:

Begin interferon-alpha. The patient's **leg ulcers** and **cytopenias** (leukopenia and thrombocytopenia) are likely due to **hydroxyurea toxicity**, despite controlled polycythemia vera (PV). **Interferon-alpha** is an appropriate alternative myelosuppressive agent for PV, especially in cases of hydroxyurea intolerance or toxicity, allowing for resolution of adverse effects while maintaining disease control. Continuing hydroxyurea is **contraindicated** as the ulcers are strongly suggestive of a drug-related adverse effect, and her cytopenias indicate bone marrow suppression. Aggressive **wound care** is necessary but will not resolve the underlying cause if hydroxyurea is the culprit. While **ruxolitinib** is approved for PV, it is typically reserved for patients who are **intolerant to or whose disease is refractory to hydroxyurea**, and who also have **splenomegaly or symptoms** that are not present here. Furthermore, ruxolitinib can cause **cytopenias**, which might worsen her current blood counts. **Busulfan** is an alkylating agent that can be used in PV, but it is generally reserved for **elderly patients** who are unable to tolerate other drugs due to its potential for severe adverse effects including **myelosuppression** and **pulmonary fibrosis**. Given her current cytopenias, busulfan could exacerbate her bone marrow suppression.

Q: Hyperviscosity is seen in all, except:

MGUS. ***MGUS*** - **Monoclonal gammopathy of undetermined significance (MGUS)** is characterized by a low concentration of **M-protein** in the blood, which is generally not sufficient to cause significant hyperviscosity. - While it is a precursor to multiple myeloma, the lack of end-organ damage and lower M-protein levels mean hyperviscosity syndrome is **rarely seen** in MGUS. *Cryoglobulinemia* - **Cryoglobulins** are immunoglobulins that precipitate at cold temperatures, leading to increased blood viscosity, especially in cooler body parts. - This precipitation can cause symptoms like **Raynaud's phenomenon**, **cutaneous vasculitis**, and **neuropathy**. *Lymphoma* - Certain lymphomas, particularly those producing **large amounts of monoclonal immunoglobulins** (e.g., Waldenström macroglobulinemia, which is a low-grade B-cell lymphoma), can lead to hyperviscosity syndrome [1]. - The elevated protein levels directly increase the **viscosity of the blood**, impairing blood flow [1]. *Multiple myeloma* - Multiple myeloma involves the overproduction of **monoclonal immunoglobulins (M-proteins)** by plasma cells, leading to high protein concentrations in the blood [1]. - These elevated proteins significantly increase blood viscosity, contributing to the symptoms of **hyperviscosity syndrome**, such as visual disturbances, neurological symptoms, and bleeding diathesis [1].

Q: All of the following decrease in iron deficiency anemia except:

TIBC. ***TIBC*** - In **iron deficiency anemia**, the body attempts to maximize iron absorption and transport, leading to an **increase** in **Total Iron Binding Capacity (TIBC)** [1]. - TIBC reflects the amount of **transferrin** available to bind iron; more transferrin is produced when iron stores are low [1]. *Serum iron* - **Serum iron** measures the iron circulating in the blood bound to transferrin. - In **iron deficiency anemia**, the overall amount of circulating iron is **decreased** due to insufficient iron stores [1]. *Ferritin* - **Ferritin** is a storage protein for iron, reflecting the body's iron stores [2]. - In **iron deficiency anemia**, iron stores are depleted, resulting in a **decreased** serum ferritin level [1]. *Transferrin saturation* - **Transferrin saturation** is the percentage of transferrin binding sites occupied by iron. - In **iron deficiency anemia**, with low serum iron and increased TIBC, the percentage of binding sites occupied by iron is **lowered** [1].

Q: A patient presented with headache and fever. His investigations revealed hemoglobin of 16g/dl, TLC of 21,000/uL, platelet count of 375,000. His DLC showed Neutrophils (25%), Lymphocytes (20%), Metamyelocytes and myelocytes 40% and eosinophils 5%. Which of the following is the next best investigation in this patient?

Philadelphia chromosome. ***Philadelphia chromosome*** - The presence of **metamyelocytes and myelocytes** (immature myeloid forms) in peripheral blood, along with **leukocytosis** (TLC 21,000/uL), is highly suggestive of **Chronic Myeloid Leukemia (CML)**. - The **Philadelphia chromosome (BCR-ABL1 fusion gene)** is the hallmark genetic abnormality in CML, and its detection is crucial for diagnosis and guiding targeted therapy with tyrosine kinase inhibitors [1]. *JAK 2 mutation* - **JAK2 mutations** are primarily associated with other **myeloproliferative neoplasms** such as **Polycythemia Vera**, **Essential Thrombocythemia**, and **Primary Myelofibrosis** [2]. - While leukocytosis can occur in these conditions, the specific presence of a high percentage of early myeloid precursors (metamyelocytes and myelocytes) points away from primary JAK2-driven disorders and more towards CML. *Bone marrow biopsy* - A **bone marrow biopsy** is usually performed after a strong suspicion of a hematological malignancy has been established, often following preliminary investigations like flow cytometry or cytogenetics on peripheral blood. - While it will be part of the diagnostic work-up for CML, identifying the Philadelphia chromosome on peripheral blood is the **next best initial step** to confirm the specific diagnosis and plan further management. *EPO level* - Serum **Erythropoietin (EPO)** levels are primarily used in the diagnosis and differentiation of **polycythemia** (high red blood cell count). - In this patient, the hemoglobin is within the normal range (16 g/dL), and the clinical picture is dominated by leukocytosis with immature myeloid forms, making EPO levels less relevant as the next investigative step.

Q: A 40-year-old male experiences hot flushes during bathing. Hb: 20 g/dL, Platelet: 89,000/mL, WBC: 30,000/mL. Investigation revealed JAK2 mutation. What is the most likely diagnosis?

PCV. PCV - **Polycythemia vera (PCV)** is characterized by an elevated red blood cell count (**Hb: 20 g/dL**), often accompanied by thrombocytosis and leukocytosis [2]. - The presence of **JAK2V617F mutation** and **aquagenic pruritus** (hot flushes during bathing) are classic diagnostic features of PCV [2]. *PMF* - **Primary myelofibrosis (PMF)** typically presents with **pancytopenia** or **anemia with leukocytosis/thrombocytosis**, often with **splenomegaly** due to extramedullary hematopoiesis [1]. - While a **JAK2 mutation** can be present, the prominent feature of **extreme erythrocytosis** observed here is not typical for PMF [1]. *CML* - **Chronic myeloid leukemia (CML)** is defined by the presence of the **Philadelphia chromosome (BCR-ABL1 fusion gene)**, which is not mentioned in the patient's presentation. - CML primarily involves a dramatic increase in **granulocytes** and their precursors, with **anemia** being common, but not the marked erythrocytosis seen here. *Essential thrombocythemia* - **Essential thrombocythemia (ET)** is characterized primarily by a persistent and unexplained **elevated platelet count**. - Although it can be associated with a **JAK2 mutation**, the presence of an **exceptionally high hemoglobin level** and **leukocytosis** points away from a primary diagnosis of ET.

Question 1

A boy after playing football complaining fatigue and abdominal pain. He also had a history of hand swelling in past. On ultrasonography, he has shrunken spleen. What is the likely diagnosis of this patient?

Accepted Answer

Sickle cell anemia

Answer

Acute pancreatitis

Answer

Iron deficiency anemia

Answer

Intermittent porphyria

Question 2

Factor V mutation most commonly initially presents as:-

Accepted Answer

Deep Vein Thrombosis (DVT)

Answer

Thrombosis

Answer

Disseminated Intravascular Coagulation (DIC)

Answer

Pulmonary Embolism

Question 3

Which of the following is not true regarding Von Willebrand disease?

Accepted Answer

Hemarthrosis is the usual presentation

Answer

Normal platelet count

Answer

Quantitative defects are seen in subtypes 1 and 3 von Willebrand disease

Answer

Produced by endothelial cells

Question 4

Which of the following hematological problems may be precipitated by parvovirus?

Accepted Answer

Aplastic crisis

Answer

Hemolytic crisis

Answer

Thrombocytopenia

Answer

Pancytopenia

Question 5

Which of the following is the best treatment for refractory ITP?

Accepted Answer

Splenectomy

Answer

Platelet transfusion

Answer

Azathioprine

Answer

Prednisolone

Question 6

A 65-year-old female with polycythemia vera on hydroxyurea for 2 years presents with painful bilateral leg ulcers for 3 months. She has well-controlled disease (Hct 42%), but recent CBC shows platelets 80,000/μL, WBC 3,500/μL. She also has diabetes (HbA1c 7.2%), peripheral vascular disease, and venous insufficiency. Doppler shows biphasic flow in dorsalis pedis. Most appropriate next step is:

Accepted Answer

Begin interferon-alpha

Answer

Continue hydroxyurea with aggressive wound care

Answer

Switch to ruxolitinib

Answer

Switch to busulfan

Question 7

Hyperviscosity is seen in all, except:

Accepted Answer

MGUS

Answer

Cryoglobulinemia

Answer

Lymphoma

Answer

Multiple myeloma

Question 8

All of the following decrease in iron deficiency anemia except:

Accepted Answer

TIBC

Answer

Serum iron

Answer

Ferritin

Answer

Transferrin saturation

Question 9

A patient presented with headache and fever. His investigations revealed hemoglobin of 16g/dl, TLC of 21,000/uL, platelet count of 375,000. His DLC showed Neutrophils (25%), Lymphocytes (20%), Metamyelocytes and myelocytes 40% and eosinophils 5%. Which of the following is the next best investigation in this patient?

Accepted Answer

Philadelphia chromosome

Answer

JAK 2 mutation

Answer

Bone marrow biopsy

Answer

EPO level

Question 10

A 40-year-old male experiences hot flushes during bathing. Hb: 20 g/dL, Platelet: 89,000/mL, WBC: 30,000/mL. Investigation revealed JAK2 mutation. What is the most likely diagnosis?

Accepted Answer

PCV

Answer

PMF

Answer

CML

Answer

Essential thrombocythemia

Hematology — MCQs

Hematology — MCQs

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