Hematology Practice Questions

Q: A 16-year-old adolescent boy with a history of severe hemophilia A is undergoing an elective inguinal hernia repair. Which of the following is the best option for preventing or treating a bleeding complication in the setting of this disease?

Combination of ε-aminocaproic acid and desmopressin. ***Combination of ε-aminocaproic acid and desmopressin*** - For **mild hemophilia A**, **desmopressin** can aid in increasing factor VIII levels temporarily [1]. **ε-aminocaproic acid (EACA)** is an **antifibrinolytic agent** which is useful for mucosal bleeding conditions or as an adjunct during dental procedures or minor surgeries to prevent clot breakdown. - While desmopressin alone may not fully prevent surgical bleeding in hemophilia A, the addition of EACA helps stabilize the clot, reducing the risk of bleeding for minor surgical procedures like inguinal hernia repair [1]. *Combination of desmopressin and fresh-frozen plasma* - **Desmopressin** is useful for **mild hemophilia A** as it releases stored factor VIII [1]. **Fresh-frozen plasma (FFP)** provides all clotting factors. - Using FFP carries a higher risk of **volume overload** and **transfusion-related acute lung injury (TRALI)** compared to factor concentrates or desmopressin alone. *Factor IX concentrate* - **Factor IX concentrate** is used to treat **hemophilia B**, which is a deficiency in factor IX, not factor VIII. - Administering factor IX concentrate to a patient with hemophilia A would not correct their coagulation defect or prevent bleeding [1]. *Fresh-frozen plasma* - While **fresh-frozen plasma (FFP)** contains factor VIII, it is in **non-concentrated amounts**, making it less effective for severe hemophilia A. - FFP also carries a risk of transfusion reactions and **volume overload**, especially when large quantities are needed.

Q: International prognostic index for lymphomas includes the following prognostic factors, EXCEPT:

Hemoglobin and albumin. ***Hemoglobin and albumin*** - While hemoglobin and albumin can be indicators of overall health and nutritional status, they are **not part of the standard International Prognostic Index (IPI)** for lymphomas. - The IPI specifically focuses on factors directly related to tumor burden and patient vitality, not general systemic markers like these. *LDH* - **Lactate dehydrogenase (LDH)** is a crucial component of the IPI, reflecting tumor burden and aggressiveness [1]. - Elevated LDH levels indicate a higher risk and are associated with a poorer prognosis [1]. *Number of extralymphatic sites involved* - The **number of extralymphatic sites involved** is a key prognostic factor in the IPI. - Involvement of more than one extralymphatic site indicates more widespread disease and a worse prognosis. *Stage of disease* - The **stage of disease**, as defined by the Ann Arbor staging system, is an essential element of the IPI [1]. - Advanced stages (III or IV) are associated with a poorer prognosis compared to early stages [1].

Q: Which of the following types of leukemia is administered prophylactic methotrexate for CNS prophylaxis –

ALL. ***ALL*** - **Acute Lymphoblastic Leukemia (ALL)** has a high propensity for **central nervous system (CNS) involvement**, necessitating prophylactic intrathecal methotrexate to prevent CNS relapse [1]. - Prophylactic treatment of the CNS is a standard component of ALL treatment protocols due to the risk of leukemic cell infiltration into the brain and spinal cord [1]. *AML* - **Acute Myeloid Leukemia (AML)** has a lower incidence of CNS involvement compared to ALL, so prophylactic CNS treatment is generally not routine unless specific risk factors are present. - While CNS involvement can occur in AML, it is more commonly treated with systemic chemotherapy that has CNS penetration or intrathecal treatment only when CNS disease is confirmed. *CLL* - **Chronic Lymphocytic Leukemia (CLL)** rarely involves the CNS, and prophylactic CNS treatment is not part of standard management. - When CNS involvement does occur in CLL, it is an aggressive, late-stage complication and typically requires specific, targeted therapy rather than prophylaxis. *CML* - **Chronic Myeloid Leukemia (CML)** has an extremely low risk of CNS involvement, especially in the chronic phase, and therefore, prophylactic CNS treatment is not administered. - CNS involvement in CML is usually seen during a blast crisis and is rare, making prophylaxis unnecessary.

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.

Question 1

A 16-year-old adolescent boy with a history of severe hemophilia A is undergoing an elective inguinal hernia repair. Which of the following is the best option for preventing or treating a bleeding complication in the setting of this disease?

Accepted Answer

Combination of ε-aminocaproic acid and desmopressin

Answer

Combination of desmopressin and fresh-frozen plasma

Answer

Factor IX concentrate

Answer

Fresh-frozen plasma

Question 2

International prognostic index for lymphomas includes the following prognostic factors, EXCEPT:

Accepted Answer

Hemoglobin and albumin

Answer

LDH

Answer

Number of extralymphatic sites involved

Answer

Stage of disease

Question 3

Which of the following types of leukemia is administered prophylactic methotrexate for CNS prophylaxis –

Accepted Answer

ALL

Answer

AML

Answer

CLL

Answer

CML

Question 4

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

Accepted Answer

500

Answer

100

Answer

250

Answer

1000

Question 5

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 6

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

Accepted Answer

Gastric carcinoma

Answer

Hepatoma

Answer

Cushing's syndrome

Answer

Hypernephroma

Question 7

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 8

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 9

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 10

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

Hematology — MCQs

Hematology — MCQs

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