Hematology Practice Questions

Q: Priapism in a young male could occur because of

leukaemia. ***Leukaemia*** - In leukaemia, especially **myeloid leukaemia**, immature white blood cells can accumulate in the **corpus cavernosa**, leading to stasis and **venous occlusion**. [1] - This cellular congestion prevents venous outflow from the penis, causing a prolonged and **painful erection (priapism)**. *Carcinoid tumour of appendix* - A carcinoid tumour of the appendix is typically associated with **carcinoid syndrome**, which involves symptoms like flushing and diarrhoea. - It does **not directly cause priapism**, as its mediators (e.g., serotonin) do not typically induce this specific local vascular event. *Testicular cancer* - Testicular cancer primarily manifests as a **painless lump in the testis** and can cause symptoms related to metastasis, but **priapism is not a typical direct presenting symptom**. - While some cancers can cause paraneoplastic syndromes, testicular cancer is not associated with priapism. *Penile cancer* - Penile cancer typically presents as a **lesion, ulcer, or mass on the penis**, often associated with pain, bleeding, or discharge. - While it affects the penis, it does **not typically cause prolonged erections (priapism)**, but rather local tissue destruction or obstruction.

Q: Umbilical cord blood stem cells are used to treat all the following diseases except :

Parkinsonism. ***Parkinsonism*** - While stem cell research for Parkinson's disease is ongoing, current treatments using **umbilical cord blood stem cells** have not shown efficacy or routine use for this neurodegenerative disorder. - Parkinson's disease primarily affects **dopaminergic neurons** in the substantia nigra, which are not effectively replaced or regenerated by umbilical cord blood stem cells in a therapeutic context. *Leukemia* - **Umbilical cord blood stem cells** are a well-established source of **hematopoietic stem cells** used in transplantation to treat various forms of leukemia. - These stem cells can engraft in the bone marrow and differentiate into healthy blood cells, replacing cancerous cells after chemotherapy or radiation. *Osteoporosis* - Research is exploring the use of **mesenchymal stem cells** from umbilical cord blood for bone regeneration and treatment of osteoporosis. - These stem cells have the potential to differentiate into **osteoblasts**, which are essential for bone formation and repair. *Diabetes* - Experimental treatments and clinical trials are investigating the use of **umbilical cord blood stem cells** to treat Type 1 Diabetes by modulating the immune system and potentially regenerating insulin-producing beta cells. - The immunomodulatory properties of these stem cells may help in preventing the autoimmune destruction of **pancreatic beta cells**.

Q: A patient has recurrent abdominal pain and jaundice. The blood investigations reveal reticulocytosis and hyperbilirubinemia. What is the clinical diagnosis?

Hereditary spherocytosis. ***Hereditary spherocytosis*** - The combination of **recurrent abdominal pain**, **jaundice**, **reticulocytosis**, and **hyperbilirubinemia** is highly suggestive of hereditary spherocytosis, which causes chronic hemolytic anemia [1, 3]. - **Jaundice** and **abdominal pain** can result from pigment gallstones (due to chronic hemolysis) and splenic enlargement, both common in this condition [2, 3]. *Mirizzi's syndrome* - Characterized by **extrinsic compression of the common hepatic duct** by a stone impacted in the cystic duct or gallbladder neck. - While it causes jaundice and abdominal pain, it does not typically present with **reticulocytosis** or **hemolysis** [1]. *Sclerosing cholangitis* - A chronic cholestatic liver disease characterized by **inflammation and fibrosis** of the bile ducts. - While it causes jaundice and abdominal pain, it is not associated with **reticulocytosis** or signs of **hemolysis** [1]. *Choledochal cyst* - A **congenital dilation of the bile ducts**, leading to recurrent abdominal pain, jaundice, and a palpable mass. - It does not involve **hemolysis** or **reticulocytosis**, making it less likely in this context.

Q: Which of the following statements is correct about Paroxysmal Nocturnal Hemoglobinuria (PNH)?

Flow cytometry is the best investigation. ***Flow cytometry is the best investigation*** - **Flow cytometry** is the gold standard for diagnosing PNH as it directly detects the absence of **GPI-anchored proteins (CD55 and CD59)** on the surface of red blood cells, granulocytes, and monocytes. - This method is highly sensitive and specific, allowing for the precise quantification of PNH clones even when present in small numbers. *Sucrose lysis test is used for the confirmation* - The **sucrose lysis test** (or sugar-water test) was an older screening test for PNH, but it is **not specific or sensitive enough** for confirmation. - It works by inducing complement activation on PNH cells in low ionic strength sucrose solution [1], but it has a high rate of false positives and negatives. *The most common cause of death is heart failure* - The most common cause of death in patients with PNH is **thrombosis**, particularly in unusual sites such as hepatic, cerebral, or abdominal veins, due to the prothrombotic state caused by the disease. - While heart complications can occur, they are not the leading cause of mortality. *It is an inherited disorder* - PNH is an **acquired clonal disorder** of hematopoietic stem cells, not an inherited genetic disorder [1]. - It results from a somatic mutation in the **PIG-A gene** in a hematopoietic stem cell, leading to the deficiency of GPI-anchored proteins.

Q: An 18-year-old male presents to the OPD with gum bleeding, fever, low total leukocyte count (TLC), and low platelet count. General examination is unremarkable. Further investigations reveal a low reticulocyte count, absent megakaryocytes, and no immature cells in the bone marrow. What is the most likely diagnosis?

Aplastic anemia. ***Aplastic anemia*** - This condition is characterized by **pancytopenia** (low TLC and platelet count, implied low red blood cell count by low reticulocyte count) due to **bone marrow failure**. - The absence of **megakaryocytes** and other immature cells in the bone marrow confirms the **hypocellularity** typical of aplastic anemia. *Immune Thrombocytopenic Purpura (ITP)* - While ITP presents with **low platelet count** and potential gum bleeding [1], the bone marrow typically shows **normal or increased megakaryocytes**. - ITP does not explain the **low total leukocyte count** or low reticulocyte count seen in this patient [1]. *Myelodysplastic Syndrome (MDS)* - MDS involves **ineffective hematopoiesis** and can present with cytopenias, but the bone marrow is usually **hypercellular or normocellular** with dysplastic changes. - The absence of immature cells and overall hypoplasia does not fit the typical picture of MDS. *Acute Myeloid Leukemia (AML)* - AML is characterized by an overproduction of **immature myeloid cells (blasts)** [3], which are conspicuously absent in this patient's bone marrow description. - While AML can cause pancytopenia, the presence of **immature cells** in the bone marrow is its hallmark [3]. Gum hypertrophy is also a common clinical sign in AML [2].

Q: Which of the following is considered a poor prognostic marker in multiple myeloma (MM)?

B2 microglobulins. ***B2 microglobulins*** - Elevated levels of **B2 microglobulin** are a significant indicator of increased tumor burden and are used in the **International Staging System (ISS)** for multiple myeloma, correlating with shorter survival [1]. - This protein is present on the surface of most nucleated cells and its accumulation reflects **renal impairment** or increased cell turnover. *Serum Creatinine* - While elevated **serum creatinine** can indicate **renal insufficiency**, a common complication in MM, it is not a direct measure of tumor burden or aggression in the same way as B2 microglobulin [1]. - **Renal failure** can be a poor prognostic factor in MM, but creatinine itself is a marker of organ damage rather than disease progression or malignancy. *Hypercalcemia* - **Hypercalcemia** is a common complication in MM due to increased **bone resorption**, but it is generally manageable and not considered a primary prognostic marker for disease aggressiveness itself. - While severe hypercalcemia can contribute to overall morbidity, its presence doesn't directly stage the disease or predict survival as significantly as B2 microglobulin. *Telomerase* - **Telomerase** is an enzyme involved in maintaining telomere length and is often overexpressed in various cancers, including MM, allowing cells to proliferate indefinitely. - While it plays a role in the **pathogenesis** of MM, its utility as a prognostic marker in routine clinical practice is not as established or as widely used as B2 microglobulin.

Q: All are increased in IDA except

Transferrin saturation. ***Transferrin saturation*** - In **iron deficiency anemia (IDA)**, there is insufficient iron to saturate transferrin, leading to a **decreased** transferrin saturation. This is the exception among the given options. - Transferrin saturation is calculated as (serum iron / TIBC) x 100, and both **serum iron** and its percentage saturation are low in IDA. *TIBC* - **Total iron-binding capacity (TIBC)** is typically **increased** in IDA as the liver produces more transferrin in an attempt to capture more iron [1]. - This elevated TIBC reflects the body's compensatory mechanism to maximize available iron uptake. *Soluble transferrin receptor* - **Soluble transferrin receptor (sTfR)** levels are **elevated** in IDA because iron-deficient erythroblasts upregulate the production of transferrin receptors on their surface as they try to scavenge more iron. - The elevated sTfR is a sensitive and specific marker for **iron deficiency**, particularly useful in differentiating IDA from anemia of chronic disease [1]. *Erythropoietin* - **Erythropoietin (EPO)** levels are **increased** in IDA due to the kidney's response to the decreased oxygen-carrying capacity of the blood (anemia) [1]. - EPO stimulates the bone marrow to produce more red blood cells, which exacerbates the demand for iron, often leading to further iron depletion if iron stores are low.

Q: What is/are the characterstics of Iron defficiency Anemaia(IDA)?

All of the options. ***All of the options*** - **Iron deficiency anemia (IDA)** characteristically presents with a combination of these markers due to a true depletion of the body's iron stores [2]. - A comprehensive evaluation of iron studies, including **TIBC**, **ferritin**, **serum iron**, and **transferrin saturation**, is essential for an accurate diagnosis of IDA [3]. *Increased TIBC* - **Total iron-binding capacity (TIBC)** is typically **elevated in IDA** as the body attempts to maximize iron absorption and transport by increasing the production of transferrin [1]. - Transferrin, the primary iron-binding protein, is less saturated with iron, leading to an **increased capacity to bind more iron**. *Low serum ferritin* - **Serum ferritin** is a direct measure of **iron storage** in the body and is considered the most sensitive and specific marker for iron deficiency. - In IDA, **ferritin levels are markedly decreased**, indicating depleted iron reserves. *Low serum iron* - **Serum iron** measures the amount of iron circulating in the blood, primarily bound to transferrin [4]. - In IDA, the **absolute amount of circulating iron is reduced** due to insufficient iron supply [1]. *Low transferrin saturation* - **Transferrin saturation** represents the percentage of transferrin binding sites occupied by iron. - In IDA, due to **low serum iron** and **high transferrin (indicated by increased TIBC)**, the transferrin saturation is significantly reduced.

Q: A 62-year-old male patient with heart failure is scheduled for a heart transplant. His renal function test is deranged, and haemoglobin is $6 \mathrm{gm} \%$. The physician ordered 2 units of whole blood. Four hours after transfusion, he developed severe respiratory distress. On examination, he is hypoxemic, has tachycardia and his mean arterial pressure is elevated. Which of the following are the best investigations for the above scenario? 1. Chest X-ray 2. Brain natriuretic peptide (BNP) level 3. Absolute neutrophil count 4. Leucocyte antibodies 5. Platelets

1 and 2. ***1 and 2*** - A **Chest X-ray** would help identify signs of **pulmonary edema** and **cardiomegaly** [1], which are characteristic of transfusion-associated circulatory overload (**TACO**) due to his underlying heart failure exacerbated by fluid from the transfusion. [2] - An elevated **Brain Natriuretic Peptide (BNP) level** is a key biomarker for heart failure [1] and would support a diagnosis of **TACO** by indicating increased ventricular stretch and volume overload. *3 and 5* - An **absolute neutrophil count** is primarily relevant for assessing infection or inflammatory conditions, which are not the primary focus given the acute respiratory distress post-transfusion in a heart failure patient. - **Platelets** are important for coagulation assessments but do not directly explain acute respiratory distress and hypoxemia in the context of post-transfusion events like TACO. *4 and 5* - **Leukocyte antibodies** (such as anti-HLA antibodies) are typically investigated in cases of **transfusion-related acute lung injury (TRALI)**, but the elevated blood pressure and underlying heart failure point more strongly towards **TACO**. - **Platelets** are not a primary investigation for acute respiratory distress following transfusion in a patient with heart failure. *2 only* - While an elevated **BNP level** is highly indicative of heart failure exacerbation and TACO [1], a **Chest X-ray** is also crucial for visualizing the pulmonary edema and assessing the extent of circulatory overload [2]. - Relying solely on BNP might miss co-occurring pulmonary issues or provide an incomplete picture of the patient's acute respiratory distress.

Q: Which of the following is not likely to be seen in a patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)?

Leukemia. Leukemia - While PNH can transform into **acute myeloid leukemia (AML)** in a small percentage of cases, it is not a common or direct presentation, making it the *least likely* immediate finding among the options. - The primary pathophysiology of PNH involves a defect in hematopoietic stem cells leading to complement-mediated destruction, not malignant proliferation of myeloid or lymphoid cells as seen in leukemia. *Thrombosis* - **Thrombosis** is a major cause of morbidity and mortality in PNH, occurring due to complement activation and platelet activation on the surface of GPI-deficient cells. - It most commonly affects unusual sites like the **hepatic** or **mesenteric veins**, and cerebral venous sinuses. *Aplastic anemia* - **Aplastic anemia** is closely associated with PNH, as both with conditions can arise from a defect in hematopoietic stem cells. - PNH clones are often detectable in patients with aplastic anemia, and some cases of PNH evolve from or into aplastic anemia. *Hemolysis* - **Hemolysis** is a hallmark of PNH, caused by the absence of **GPI-anchored proteins (CD55 and CD59)** on red blood cells, making them susceptible to complement-mediated destruction [1]. - This leads to intravascular hemolysis, resulting in characteristic symptoms like **dark urine** (hemoglobinuria), especially in the morning [1].

Question 1

Priapism in a young male could occur because of

Accepted Answer

leukaemia

Answer

carcinoid tumour of appendix

Answer

testicular cancer

Answer

penile cancer

Question 2

Umbilical cord blood stem cells are used to treat all the following diseases except :

Accepted Answer

Parkinsonism

Answer

Leukemia

Answer

Osteoporosis

Answer

Diabetes

Question 3

A patient has recurrent abdominal pain and jaundice. The blood investigations reveal reticulocytosis and hyperbilirubinemia. What is the clinical diagnosis?

Accepted Answer

Hereditary spherocytosis

Answer

Mirizzi's syndrome

Answer

Sclerosing cholangitis

Answer

Choledochal cyst

Question 4

Which of the following statements is correct about Paroxysmal Nocturnal Hemoglobinuria (PNH)?

Accepted Answer

Flow cytometry is the best investigation

Answer

Sucrose lysis test is used for the confirmation

Answer

The most common cause of death is heart failure

Answer

It is an inherited disorder

Question 5

An 18-year-old male presents to the OPD with gum bleeding, fever, low total leukocyte count (TLC), and low platelet count. General examination is unremarkable. Further investigations reveal a low reticulocyte count, absent megakaryocytes, and no immature cells in the bone marrow. What is the most likely diagnosis?

Accepted Answer

Aplastic anemia

Answer

Immune Thrombocytopenic Purpura (ITP)

Answer

Myelodysplastic Syndrome (MDS)

Answer

Acute Myeloid Leukemia (AML)

Question 6

Which of the following is considered a poor prognostic marker in multiple myeloma (MM)?

Accepted Answer

B2 microglobulins

Answer

Serum Creatinine

Answer

Hypercalcemia

Answer

Telomerase

Question 7

All are increased in IDA except

Accepted Answer

Transferrin saturation

Answer

TIBC

Answer

Soluble transferrin receptor

Answer

Erythropoietin

Question 8

What is/are the characterstics of Iron defficiency Anemaia(IDA)?

Accepted Answer

All of the options

Answer

Increased TIBC

Answer

Low serum ferritin

Answer

Low serum iron

Answer

Low transferrin saturation

Question 9

A 62-year-old male patient with heart failure is scheduled for a heart transplant. His renal function test is deranged, and haemoglobin is $6 \mathrm{gm} \%$. The physician ordered 2 units of whole blood. Four hours after transfusion, he developed severe respiratory distress. On examination, he is hypoxemic, has tachycardia and his mean arterial pressure is elevated. Which of the following are the best investigations for the above scenario?

1. Chest X-ray
2. Brain natriuretic peptide (BNP) level
3. Absolute neutrophil count
4. Leucocyte antibodies
5. Platelets

Accepted Answer

1 and 2

Answer

3 and 5

Answer

4 and 5

Answer

2 only

Question 10

Which of the following is not likely to be seen in a patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)?

Accepted Answer

Leukemia

Answer

Thrombosis

Answer

Aplastic anemia

Answer

Hemolysis

Hematology — MCQs

Hematology — MCQs

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