Hematology Practice Questions

Q: What is the threshold absolute neutrophil count that defines severe neutrophil depletion?

500. 500 - An absolute neutrophil count (ANC) of less than 500 cells/µL is defined as severe neutropenia. - At this level, the risk of serious bacterial and fungal infections significantly increases due to compromised immune defense. 100 - An ANC of less than 100 cells/µL is considered profound neutropenia, representing an even higher risk of severe infection. - While very low, it is not the general threshold for defining severe neutropenia, which is typically 500 cells/µL. 250 - An ANC of 250 cells/µL falls within the range of severe neutropenia, but the standard threshold for defining severe neutropenia is generally accepted as 500 cells/µL. - This count still indicates a very high risk of infection, but 500 cells/µL is the more common cutoff for "severe." 1000 - An ANC of 1000 cells/µL is considered mild neutropenia and does not meet the criteria for severe neutrophil depletion. - While it's lower than normal, the risk of serious infection is considerably lower compared to ANCs below 500 cells/µL.

Q: Which of the following presents as mediastinal enlargement?

Hodgkin lymphoma. ***Hodgkin lymphoma*** - **Hodgkin lymphoma** frequently presents with **mediastinal involvement**, particularly the **nodular sclerosis subtype**, leading to mediastinal enlargement [1]. - This enlargement is often detected on chest X-rays and can cause symptoms due to compression of nearby structures [1]. *T-cell Acute Lymphoblastic Leukemia* - While T-cell ALL can cause a **mediastinal mass**, it is a **leukemia** typically characterized by widespread bone marrow involvement and circulating blast cells, not primarily a solid mediastinal enlargement. - The mediastinal involvement is usually a manifestation of **thymic infiltration** by leukemic cells, but the primary disease is systemic. *Primary mediastinal large B-cell lymphoma* - This is a distinct subtype of **diffuse large B-cell lymphoma (DLBCL)** that **primarily arises in the mediastinum** and presents as a large mediastinal mass. - It is histologically and clinically distinct from Hodgkin lymphoma, though both can cause mediastinal enlargement [1]. *Chronic Myeloid Leukemia* - **CML** is a myeloproliferative neoplasm characterized by the **Philadelphia chromosome** and primarily affects the bone marrow and spleen. - While extramedullary hematopoiesis can occur, mediastinal enlargement is not a typical or common presentation of CML.

Q: Polycythemia may be a feature of all of the following except:

Gastric carcinoma. ***Gastric carcinoma*** - **Gastric carcinoma** is not typically associated with paraneoplastic polycythemia. Paraneoplastic syndromes in gastric cancer are rare but may include **Trousseau's syndrome** (migratory thrombophlebitis) or **acanthosis nigricans**. - While anemia is a common feature due to chronic blood loss, polycythemia is not a known association. *Hepatoma* - **Hepatocellular carcinoma (Hepatoma)** can produce **erythropoietin (EPO)**, leading to secondary polycythemia as a paraneoplastic syndrome [1]. - This occurs due to the unregulated production of EPO by the tumor cells, stimulating red blood cell production in the bone marrow [1]. *Hypernephroma* - **Renal cell carcinoma (Hypernephroma)** is well-known for its paraneoplastic manifestations, including the production of **erythropoietin** [1]. - This **ectopic EPO production** stimulates the bone marrow, resulting in secondary polycythemia in a significant percentage of patients [1]. *Cushing's syndrome* - **Cushing's syndrome**, while primarily involving hypercortisolism, can sometimes present with a **mild polycythemia** or an increase in red blood cell mass [2]. - This is thought to be due to direct stimulatory effects of **excess glucocorticoids** on erythropoiesis or indirectly through increased erythropoietin sensitivity in the bone marrow.

Q: Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except

High C4 complement levels. High C4 complement levels - **High C4 complement levels** are not typically associated with an increased risk or development of lymphoma in Sjögren's syndrome. In fact, **low C4** (due to consumption) is more commonly linked to complications like vasculitis or cryoglobulinemia, which can sometimes precede lymphoma. - Decreased complement levels, particularly **C4**, are often seen in Sjögren's patients with **lymphoproliferative disorders** or **cryoglobulinemia**, making elevated levels an unlikely indicator of lymphoma. Leukopenia - **Leukopenia** (low white blood cell count) can be a significant indicator of developing **lymphoma** in patients with Sjögren's syndrome. It may reflect bone marrow infiltration or a systemic effect of the underlying disease. - Persistent or worsening **leukopenia** should prompt further investigation for lymphoproliferative disorders. Cryoglobulinemia - **Cryoglobulinemia** is a well-established risk factor and a paraneoplastic manifestation strongly associated with the development of **B-cell non-Hodgkin lymphoma** in Sjögren's syndrome [1]. - The presence of **cryoglobulins** indicates abnormal B-cell activity, which can evolve into overt lymphoma. Persistent parotid gland enlargement - **Persistent parotid gland enlargement** in a Sjögren's patient is a critical warning sign for potential **lymphoma**, especially **mucosa-associated lymphoid tissue (MALT) lymphoma** [1]. - This symptom warrants a **biopsy** to rule out malignancy, as it often signifies increased lymphoid proliferation within the salivary glands.

Q: Causes of deep venous thrombosis include all of the following, EXCEPT:

Hypothyroidism. ***Hypothyroidism*** - **Hypothyroidism** is generally associated with a hypercoagulable state due to various mechanisms, but it is not considered a primary direct cause of **deep venous thrombosis (DVT)**. - While some studies show an association with increased **cardiovascular risk**, it is not listed as a common or significant independent risk factor for DVT compared to other conditions. *Prolonged surgery* - **Prolonged surgery** significantly increases the risk of DVT due to **immobility**, leading to venous stasis, and **surgical trauma** contributing to endothelial injury and activation of coagulation [1]. - Anesthesia can also cause **vasodilation** and further pooling of blood in the lower extremities, exacerbating stasis [1]. *Paroxysmal Nocturnal Hemoglobinuria (PNH)* - **PNH** is a rare acquired hematological disorder characterized by increased susceptibility to complement-mediated hemolysis and a very high risk of **thrombosis**, including DVT, often in unusual locations. - The thrombotic tendency in PNH is due to the absence of **glycosylphosphatidylinositol-anchored proteins** like **CD55** and **CD59** on blood cells, which normally protect against complement activation. *Oral contraceptives* - **Oral contraceptives**, particularly those containing **estrogen**, significantly increase the risk of DVT by causing a **procoagulant state**. - Estrogen can increase the synthesis of **coagulation factors** (e.g., factors VII, VIII, X, and fibrinogen) and decrease levels of natural anticoagulants (e.g., antithrombin).

Q: Best blood product to be given in a patient of multiple clotting factor deficiency with active bleeding:

Fresh frozen plasma. ***Fresh frozen plasma*** - **Fresh frozen plasma (FFP)** contains all coagulation factors, including labile factors V and VIII, making it the best choice for patients with multiple clotting factor deficiencies and active bleeding. - It rapidly replenishes clotting factors, which is critical in scenarios of **acute hemorrhage** due to global coagulopathy. *Whole blood* - **Whole blood** contains red blood cells, plasma, and platelets, but its clotting factor concentration is lower than FFP and deteriorates over storage. - It is preferred for massive hemorrhage with significant blood volume loss, but less effective for isolated clotting factor deficiencies without substantial volume depletion. *Packed RBCs* - **Packed red blood cells (PRBCs)** are primarily used to increase oxygen-carrying capacity by raising hemoglobin levels in anemic patients. - They lack significant amounts of clotting factors and are therefore not effective in treating active bleeding due to coagulation factor deficiencies. *Cryoprecipitate* - **Cryoprecipitate** contains specific clotting factors, namely factor VIII, von Willebrand factor, fibrinogen, and factor XIII. - While useful for deficiencies in these specific factors (e.g., hemophilia A, DIC with low fibrinogen), it does not provide a broad spectrum of all clotting factors needed for general multiple factor deficiencies.

Q: A 25-year-old patient came to medicine OPD with complaints of sore throat and a slightly itchy rash. On examination, pharyngeal erythema with bilateral tender cervical lymphadenopathy is noted. Throat swab is sent for microbiological examination. Peripheral blood film revealed the following finding. Which of the following malignant conditions are not associated with the above infection?

B cell lymphoma (immunocompetent patient). ***B cell lymphoma (immunocompetent patient)*** - The image shows **atypical lymphocytes**, characteristic of infectious mononucleosis, typically caused by the **Epstein-Barr Virus (EBV)**. EBV is associated with several malignancies, but primary B-cell lymphoma in an immunocompetent patient is not a direct consequence of EBV infection, unlike post-transplant lymphoproliferative disorder. - While EBV infects B cells, leading to their proliferation, a normal immune system usually controls this. The specific malignant transformation to B-cell lymphoma without underlying immunodeficiency is not a direct, well-established association with EBV infection. It's more commonly linked to specific genetic translocations or immunodeficiency. *Hodgkin disease* - **Epstein-Barr Virus (EBV)** has a strong association with a significant proportion of classic Hodgkin lymphoma cases, particularly the mixed-cellularity and lymphocyte-depleted subtypes. - EBV DNA can be found in the **Reed-Sternberg cells** characteristic of Hodgkin lymphoma. *Burkitt's lymphoma* - **Endemic Burkitt's lymphoma**, prevalent in equatorial Africa, is strongly linked to **Epstein-Barr Virus (EBV)** and chronic malaria. - EBV contributes to the pathogenesis by promoting B-cell proliferation, which can lead to the characteristic **MYC translocation**. *Nasopharyngeal carcinoma* - **Epstein-Barr Virus (EBV)** is consistently associated with all histological types of nasopharyngeal carcinoma (NPC), particularly the undifferentiated type. - EBV infection is considered a **necessary cofactor** in the development of NPC, with viral DNA found in almost all tumor cells.

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.

Question 1

What is the threshold absolute neutrophil count that defines severe neutrophil depletion?

Accepted Answer

500

Answer

100

Answer

250

Answer

1000

Question 2

Which of the following presents as mediastinal enlargement?

Accepted Answer

Hodgkin lymphoma

Answer

T-cell Acute Lymphoblastic Leukemia

Answer

Primary mediastinal large B-cell lymphoma

Answer

Chronic Myeloid Leukemia

Question 3

Polycythemia may be a feature of all of the following except:

Accepted Answer

Gastric carcinoma

Answer

Hepatoma

Answer

Cushing's syndrome

Answer

Hypernephroma

Question 4

Development of Lymphoma in Sjogren's syndrome is suggested by all of the following except

Accepted Answer

High C4 complement levels

Answer

Leukopenia

Answer

Cryoglobulinemia

Answer

Persistent parotid gland enlargement

Question 5

Causes of deep venous thrombosis include all of the following, EXCEPT:

Accepted Answer

Hypothyroidism

Answer

Prolonged surgery

Answer

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Answer

Oral contraceptives

Question 6

Best blood product to be given in a patient of multiple clotting factor deficiency with active bleeding:

Accepted Answer

Fresh frozen plasma

Answer

Whole blood

Answer

Packed RBCs

Answer

Cryoprecipitate

Question 7

A 25-year-old patient came to medicine OPD with complaints of sore throat and a slightly itchy rash. On examination, pharyngeal erythema with bilateral tender cervical lymphadenopathy is noted. Throat swab is sent for microbiological examination. Peripheral blood film revealed the following finding. Which of the following malignant conditions are not associated with the above infection?

Accepted Answer

B cell lymphoma (immunocompetent patient)

Answer

Hodgkin disease

Answer

Burkitt's lymphoma

Answer

Nasopharyngeal carcinoma

Question 8

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 9

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 10

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

Hematology — MCQs

Hematology — MCQs

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