Hematology Practice Questions

Q: A 25-year-old lady presented with anemia, jaundice, and recurrent joint pains. All of the following are true except:

She can present with pulmonary bleeds. ***She can present with pulmonary bleeds*** - The symptoms of **anemia**, **jaundice**, and recurrent **joint pains** in a 25-year-old suggest **sickle cell anemia**. Pulmonary bleeds are not a typical or common presentation of sickle cell disease; rather, patients are more prone to **acute chest syndrome**, which involves pulmonary infiltrates, but not usually frank bleeding [2]. - While various complications can affect the lungs in sickle cell disease, **pulmonary hemorrhage** is rare and not a characteristic feature. *HbA will be undetectable* - In **sickle cell anemia (Hb SS)**, the body exclusively produces **hemoglobin S (HbS)**, meaning **adult hemoglobin (HbA)**, the normal form, is indeed undetectable [3], [4]. - This is because the patient is homozygous for the **sickle gene**, preventing the synthesis of normal beta-globin chains [4]. *She may have retinopathy* - **Sickle cell retinopathy** is a common complication due to **vaso-occlusion** in the retinal vessels, leading to ischemia, neovascularization, and potentially vision loss. - This can manifest as various stages of proliferative retinopathy, often requiring treatment to preserve vision. *Hydroxyurea would help her* - **Hydroxyurea** is a medication used to reduce the frequency and severity of **sickle cell crises** and mitigate complications [1]. - It works by increasing the production of **fetal hemoglobin (HbF)**, which interferes with the polymerization of HbS and improves red blood cell function [1].

Q: Autoimmune hemolytic anemia is seen in: A) Sickle cell anemia B) Chronic lymphocytic leukemia (CLL) C) Acute myelocytic leukemia (AML) D) Multiple myeloma

Chronic lymphocytic leukemia (CLL). ***Chronic lymphocytic leukemia (CLL)*** - **CLL** is strongly associated with **autoimmune hemolytic anemia** due to immune system dysregulation that leads to production of **autoantibodies** against red blood cells [1]. - The malignant B-lymphocytes in CLL cause **immunologic dysfunction**, making AIHA one of the most common autoimmune complications of this disease [1]. *Sickle cell anemia* - **Sickle cell anemia** causes hemolysis through **intrinsic red blood cell defects** due to abnormal hemoglobin S structure, not autoimmune mechanisms. - The hemolysis is **mechanical** and occurs when sickled cells become rigid and fragile, rather than being mediated by autoantibodies. *Acute myelocytic leukemia (AML)* - **AML** is a rapidly progressing cancer of the myeloid blood cell line, primarily affecting early progenitor cells. - Autoimmune hemolytic anemia is **rarely associated** with AML; the primary hematologic issue is **pancytopenia** due to bone marrow suppression. *Multiple myeloma* - **Multiple myeloma** is a plasma cell dyscrasia characterized by proliferation of malignant plasma cells and production of **monoclonal proteins** [2]. - While other hematologic abnormalities can occur, **AIHA is not a common complication** of multiple myeloma [2].

Q: All of the following are true about lupus anticoagulant except:

Recurrent bleeding episodes. ***Recurrent bleeding episodes*** - Lupus anticoagulant (LA) is an **autoantibody** that paradoxically increases the risk of **thrombosis** (clotting), not bleeding. - While it prolongs clotting tests in vitro, its in vivo effect is **procoagulant**, leading to conditions like **deep vein thrombosis**, **pulmonary embolism**, and **stroke**. *Prolongation of only aPTT* - This statement is false because LA typically causes a **prolongation of phospholipid-dependent clotting tests**, most commonly the **activated partial thromboplastin time (aPTT)**. - However, it can also affect other tests such as the dilute Russell viper venom time (dRVVT) and kaolin clotting time. *Recurrent second trimester abortion* - Lupus anticoagulant is a key component of **antiphospholipid syndrome (APS)**, a condition strongly associated with **recurrent pregnancy loss**, particularly in the second and third trimesters. - The presence of LA contributes to **placental thrombosis**, leading to fetal demise. *Can be associated without any clinical symptoms* - Many individuals test positive for lupus anticoagulant incidentally without ever developing **thrombotic events** or other clinical manifestations of antiphospholipid syndrome. - The presence of LA alone does not automatically equate to a diagnosis of APS; clinical criteria must also be met.

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: 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.

Question 1

A 25-year-old lady presented with anemia, jaundice, and recurrent joint pains. All of the following are true except:

Accepted Answer

She can present with pulmonary bleeds

Answer

HbA will be undetectable

Answer

She may have retinopathy

Answer

Hydroxyurea would help her

Question 2

Autoimmune hemolytic anemia is seen in:
A) Sickle cell anemia
B) Chronic lymphocytic leukemia (CLL)
C) Acute myelocytic leukemia (AML)
D) Multiple myeloma

Accepted Answer

Chronic lymphocytic leukemia (CLL)

Answer

Acute myelocytic leukemia (AML)

Answer

Multiple myeloma

Answer

Sickle cell anemia

Question 3

All of the following are true about lupus anticoagulant except:

Accepted Answer

Recurrent bleeding episodes

Answer

Prolongation of only aPTT

Answer

Recurrent second trimester abortion

Answer

Can be associated without any clinical symptoms

Question 4

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 5

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 6

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

Accepted Answer

Aplastic crisis

Answer

Hemolytic crisis

Answer

Thrombocytopenia

Answer

Pancytopenia

Question 7

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

Accepted Answer

Splenectomy

Answer

Platelet transfusion

Answer

Azathioprine

Answer

Prednisolone

Question 8

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 9

Hyperviscosity is seen in all, except:

Accepted Answer

MGUS

Answer

Cryoglobulinemia

Answer

Lymphoma

Answer

Multiple myeloma

Question 10

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

Hematology — MCQs

Hematology — MCQs

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