Hematology Practice Questions

Q: The most classic dietary trigger for acute hemolytic anemia in G6PD deficiency is which of the following?

Fava beans. **Fava beans** - **Fava beans** contain high concentrations of **vicine and convicine**, which are converted into **divicine and isouramil**. These compounds produce **reactive oxygen species** that overwhelm the erythrocyte's antioxidant defense mechanisms, especially in individuals with **G6PD deficiency** [2]. - In individuals with **G6PD deficiency**, the inability to produce sufficient **NADPH** results in inadequate levels of **reduced glutathione**, making red blood cells vulnerable to **oxidative stress** induced by these fava bean metabolites, leading to **hemolysis** [1, 3]. *Viral upper respiratory infections only* - While **infections (bacterial or viral)** can trigger **hemolytic crises** in G6PD deficient individuals by causing **oxidative stress** and inflammation, they are not a dietary trigger [3, 4]. - The question specifically asks for a **dietary trigger**, which excludes infections as the primary cause. *Vitamin C supplements* - While **large doses of vitamin C (ascorbic acid)** can act as a **pro-oxidant** and potentially induce some oxidative stress, it is generally not considered a classic or strong trigger for acute hemolytic anemia in G6PD deficiency at typical supplement doses. - **Fava beans** are far more potent and well-documented as a dietary trigger for this condition [2]. *Folic acid deficiency* - **Folic acid deficiency** does not directly cause acute hemolytic anemia in G6PD deficiency; it typically leads to **megaloblastic anemia** due to impaired DNA synthesis [4]. - While folic acid supplementation is often given to G6PD deficient patients with chronic hemolysis to support increased red blood cell production, its deficiency does not induce oxidative hemolysis.

Q: B-cell prolymphocytic leukemia patients differ from those with B-cell chronic lymphocytic leukemia in:

Having a shorter survival. Having a shorter survival - **B-cell prolymphocytic leukemia (B-PLL)** is characterized by a significantly more **aggressive** clinical course and **poorer prognosis** compared to B-cell chronic lymphocytic leukemia (B-CLL). - This aggressive nature leads to a **shorter overall survival** for patients with B-PLL. *Presenting at a younger age* - **B-CLL** typically presents in **older adults**, with a median age of diagnosis around 70 years [1]. - While B-PLL can occur at various ages, it is not consistently associated with a **younger presentation** compared to B-CLL. *Having a lower total leucocyte count* - **B-PLL** is classically associated with a **very high total leukocyte count**, often exceeding 100 x 10^9/L, due to the proliferation of prolymphocytes. - **B-CLL** can also have elevated leukocyte counts, but they are generally **not as dramatically high** as in B-PLL. *Having prominent lymphadenopathy* - **B-CLL** is characterized by prominent and widespread **lymphadenopathy** and **splenomegaly** [1]. - While B-PLL can involve lymph nodes and spleen, it often presents with **massive splenomegaly** and **less prominent lymphadenopathy** in comparison to B-CLL [1].

Q: A 40-year-old male presents with weakness, weight loss, and abdominal pain, accompanied by diffuse hyperpigmentation, a palpable liver edge, and polyarthritis of the wrist and hips. His fasting blood sugar level is 185 mg/dl. What is the most likely diagnosis?

Hemochromatosis. ***Hemochromatosis*** - The constellation of **hyperpigmentation**, diabetes (**fasting blood sugar 185 mg/dl**), **polyarthritis**, and **hepatomegaly** (palpable liver edge) is highly characteristic of **hemochromatosis** [1]. - **Iron overload** in hereditary hemochromatosis leads to deposition in various organs, causing organ damage and the classic triad of **bronze diabetes** (hyperpigmentation + diabetes) and **cirrhosis** [1]. *Type I Diabetes Mellitus* - While the patient has **elevated blood sugar**, Type I diabetes is typically an **autoimmune destruction of pancreatic beta cells** and does not cause **hyperpigmentation**, **hepatomegaly**, or widespread **polyarthritis** as primary features. - Onset is usually in childhood or early adulthood, and prominent symptoms often include **polydipsia, polyuria, and weight loss**, without the complex systemic manifestations described. *Pancreatic Carcinoma* - Pancreatic carcinoma can cause **weight loss**, **abdominal pain**, and sometimes **diabetes** due to pancreatic dysfunction. - However, it does not typically present with diffuse **hyperpigmentation**, **polyarthritis**, or significant **hepatomegaly** as described, unless there are widespread metastases to the liver, which would be a later stage phenomenon. *Addison's Disease* - **Addison's disease** (adrenal insufficiency) is characterized by **hyperpigmentation**, **weakness**, and **weight loss**, which align with some symptoms. - However, it does not cause **elevated blood sugar**, **hepatomegaly**, or **polyarthritis**, which are key findings in this patient.

Q: Which of the following is least likely to cause iron deficiency anemia?

Excess of meat in the diet. ***Chronic blood loss*** - Chronic blood loss is a **significant cause** of iron deficiency anemia as it depletes iron stores over time [1]. - Conditions such as **peptic ulcers** or heavy menstrual bleeding can lead to significant blood loss, contributing to anemia [1], [2]. *Achlorhydria* - Achlorhydria refers to the **absence of stomach acid**, which can impair iron absorption, potentially leading to deficiency. - It is important but is **not a direct cause** of anemia, rather a contributing factor. *Excess of meat in the diet* - A diet high in meat actually provides **heme iron**, which is readily absorbed and can prevent deficiency rather than cause it [2]. - Iron deficiency is more likely in diets **low in meat** or in vegetarians unless iron supplements are included. *Extensive surgical removal of the proximal small bowel* - This can lead to **malabsorption** of nutrients, including iron, thus contributing to iron deficiency anemia indirectly [2]. - However, it can also cause deficiencies in other nutrients, not solely related to iron.

Q: Which of the following methods can be used to prevent graft-versus-host disease in bone marrow transplantation?

All of the options. ***All of the options*** - **T-cell removal** from the donor marrow significantly reduces the number of alloreactive T-cells that can cause GVHD. - **Prior immunosuppression** prepares the recipient's immune system to reduce graft rejection, while **post-procedure immunosuppression** helps manage any remaining alloreactive cells [1]. *T-cell removal* - This method directly targets the cells primarily responsible for initiating **graft-versus-host disease (GVHD)**. - While effective, it can also increase the risk of **graft failure** and **leukemic relapse** due to the loss of graft-versus-leukemia effects. *Post procedure immune suppression* - Administering **immunosuppressive drugs** after transplantation helps to suppress the recipient's immune response and prevent donor immune cells from attacking recipient tissues [1]. - Common regimens include **cyclosporine**, **methotrexate**, or **tacrolimus**, which are tapered over time to minimize side effects [2, 3]. *Prior immune suppression* - Suppressing the **recipient's immune system** before transplantation helps to create an environment where the donor cells are less likely to be rejected and reduces recipient T-cell mediated responses against the graft [1]. - This is typically part of the **conditioning regimen** given before the bone marrow infusion.

Q: Microcytic anemia is not typically associated with which of the following conditions?

Leukopenia. Leukopenia - **Leukopenia** is a decrease in white blood cell count and is not directly associated with the pathogenesis of microcytic anemia. - While both can occur in certain systemic conditions (e.g., bone marrow suppression), leukopenia itself does not cause or result from a microcytic anemia. *Osteomyelitis* - **Osteomyelitis**, a bone infection, can lead to **anemia of chronic disease**, which often manifests as a normocytic, normochromic anemia but can become microcytic over time due to impaired iron utilization [1]. - Inflammation in osteomyelitis can cause a functional iron deficiency, leading to smaller red blood cells [1]. *Papillary necrosis* - **Papillary necrosis** is a renal condition often associated with chronic analgesic abuse, sickle cell disease, or diabetes. - It can cause **renal failure** or chronic kidney disease, which is a common cause of **anemia of chronic disease**, sometimes presenting as microcytic initially or upon progression [1], [2]. *Rheumatoid arthritis* - **Rheumatoid arthritis** is a chronic inflammatory disorder that frequently causes **anemia of chronic disease** [3]. - This type of anemia is typically **normocytic**, but with prolonged severe inflammation, it can evolve into a **microcytic** and hypochromic anemia due to altered iron metabolism [1], [2].

Q: A 22-year-old man presents to the emergency department with dyspnea, palpitations, and a headache, which developed shortly after taking trimethoprim-sulfamethoxazole for a urinary tract infection. Laboratory studies reveal a normochromic, normocytic anemia, and a peripheral blood smear shows Heinz bodies. Which of the following is the most likely cause of this patient's anemia?

Glucose-6-phosphate dehydrogenase deficiency. ***Glucose-6-phosphate dehydrogenase deficiency*** - The patient's symptoms (dyspnea, palpitations, headache) along with **normochromic, normocytic anemia** and the presence of **Heinz bodies** after taking **trimethoprim-sulfamethoxazole** are highly indicative of **hemolytic anemia** due to G6PD deficiency [1], [2]. - Trimethoprim-sulfamethoxazole is an **oxidative stressor** that can trigger hemolysis in individuals with G6PD deficiency, as they cannot produce sufficient **NADPH** to protect red blood cells from oxidative damage [1]. *Lead poisoning* - Lead poisoning typically causes a **microcytic, hypochromic anemia** and is associated with **basophilic stippling** on a peripheral blood smear, not Heinz bodies. - Symptoms usually include **abdominal pain**, **neuropathy**, and **cognitive deficits**, which are not clearly described here. *Folate deficiency* - Folate deficiency leads to a **macrocytic, megaloblastic anemia**, characterized by **hypersegmented neutrophils** and large red blood cells. - It would not typically present with Heinz bodies or be exacerbated by trimethoprim-sulfamethoxazole in this manner. *Hereditary spherocytosis* - This condition is characterized by **spherocytes** on the peripheral smear and increased osmotic fragility, often leading to chronic hemolytic anemia. - While it causes hemolytic anemia, the presence of **Heinz bodies** specifically after an oxidative stressor (like trimethoprim-sulfamethoxazole) points more strongly to G6PD deficiency [2].

Q: All of the following are causes of microcytic hypochromic anemia except:

Fanconi's anemia. ***Fanconi anaemia*** - Fanconi anemia is a type of **aplastic anemia** caused by bone marrow failure, which does not typically result in **microcytic hypochromic anemia**. - The anemia in Fanconi's is usually **normocytic** due to inadequate production of red blood cells rather than defective hemoglobin production. *Iron deficiency* - Iron deficiency anemia is the most common cause of **microcytic hypochromic anemia**, characterized by decreased **iron stores** affecting hemoglobin synthesis [1]. - Common clinical features include **fatigue**, **pallor**, and **koilonychia** (spoon nails). *Lead poisoning* - Lead poisoning leads to microcytic hypochromic anemia by causing **inhibition of heme synthesis**, resulting in the accumulation of **protoporphyrin**. - Alongside anemia, symptoms may include **abdominal pain** and **neurological dysfunction**. *Thalassemia* - Thalassemia is a genetic disorder that also causes **microcytic hypochromic anemia** due to inadequate formation of **alpha or beta globin chains**. - Patients often present with **chronic anemia** and **splenomegaly** due to hemolysis.

Q: What is the treatment for chemotherapy-induced agranulocytopenia?

Granulocyte colony-stimulating factor (G-CSF). ***Granulocyte colony-stimulating factor (G-CSF)*** - **G-CSF** directly stimulates the production and maturation of **granulocytes** (including neutrophils) in the bone marrow, - This helps to **increase neutrophil counts** and shorten the duration of **chemotherapy-induced agranulocytopenia**, reducing the risk of infection. *Packed red blood cells* - **Packed red blood cells (PRBCs)** are used to treat **anemia**, not agranulocytopenia. - They increase **hemoglobin** levels but have no effect on neutrophil count. *Platelet transfusion* - **Platelet transfusions** are administered to treat **thrombocytopenia** (low platelet count) and prevent bleeding. - They do not address the low neutrophil count seen in **agranulocytopenia**. *Granulocyte-macrophage colony-stimulating factor (GM-CSF), less commonly used.* - **GM-CSF** stimulates the production of both **granulocytes** and **macrophages**. - While it can increase neutrophil counts, **G-CSF** is generally preferred due to its more specific action and lower incidence of side effects for neutropenia.

Q: A girl who developed abdominal pain and presented to the emergency department with a seizure after taking sulphonamides is likely to have a diagnosis of:

Acute intermittent porphyria due to other triggers. ***Acute intermittent porphyria due to other triggers*** - The combination of **abdominal pain**, **seizure**, and the **ingestion of sulfonamides** (a known trigger) is highly suggestive of an acute porphyric attack [1]. - **Sulfonamides** induce δ-aminolevulinate synthase, leading to an overproduction of neurotoxic porphyrin precursors [1]. *Congenital erythropoietic porphyria with skin manifestations* - This condition primarily presents with **severe photosensitivity**, **blistering skin lesions**, and **hemolytic anemia**, rather than acute neurological symptoms [1]. - While it is a type of porphyria, its clinical presentation and triggers are distinct from the neurovisceral symptoms described. *Infectious mononucleosis with fever and lymphadenopathy* - Characterized by **fever**, **fatigue**, **sore throat**, and **lymphadenopathy**, symptoms not aligned with the severe abdominal pain and seizure observed. - This condition is caused by the Epstein-Barr virus and is not triggered by sulfonamide use. *Kawasaki disease with prolonged fever and rash* - This is an **acute vasculitis** in children, involving **prolonged fever**, **rash**, **conjunctivitis**, and lymphadenopathy, but not seizures or abdominal pain caused by sulfonamides. - It primarily affects the cardiovascular system and is not related to porphyria or drug-induced attacks.

Question 1

The most classic dietary trigger for acute hemolytic anemia in G6PD deficiency is which of the following?

Accepted Answer

Fava beans

Answer

Viral upper respiratory infections only

Answer

Vitamin C supplements

Answer

Folic acid deficiency

Question 2

B-cell prolymphocytic leukemia patients differ from those with B-cell chronic lymphocytic leukemia in:

Accepted Answer

Having a shorter survival

Answer

Presenting at a younger age

Answer

Having a lower total leucocyte count

Answer

Having prominent lymphadenopathy

Question 3

A 40-year-old male presents with weakness, weight loss, and abdominal pain, accompanied by diffuse hyperpigmentation, a palpable liver edge, and polyarthritis of the wrist and hips. His fasting blood sugar level is 185 mg/dl. What is the most likely diagnosis?

Accepted Answer

Hemochromatosis

Answer

Type I Diabetes Mellitus

Answer

Pancreatic Carcinoma

Answer

Addison's Disease

Question 4

Which of the following is least likely to cause iron deficiency anemia?

Accepted Answer

Excess of meat in the diet

Answer

Chronic blood loss

Answer

Achlorhydria

Answer

Extensive surgical removal of the proximal small bowel

Question 5

Which of the following methods can be used to prevent graft-versus-host disease in bone marrow transplantation?

Accepted Answer

All of the options

Answer

T-cell removal

Answer

Post procedure immune suppression

Answer

Prior immune suppression

Question 6

Microcytic anemia is not typically associated with which of the following conditions?

Accepted Answer

Leukopenia

Answer

Osteomyelitis

Answer

Papillary necrosis

Answer

Rheumatoid arthritis

Question 7

A 22-year-old man presents to the emergency department with dyspnea, palpitations, and a headache, which developed shortly after taking trimethoprim-sulfamethoxazole for a urinary tract infection. Laboratory studies reveal a normochromic, normocytic anemia, and a peripheral blood smear shows Heinz bodies. Which of the following is the most likely cause of this patient's anemia?

Accepted Answer

Glucose-6-phosphate dehydrogenase deficiency

Answer

Folate deficiency

Answer

Lead poisoning

Answer

Hereditary spherocytosis

Question 8

All of the following are causes of microcytic hypochromic anemia except:

Accepted Answer

Fanconi's anemia

Answer

Thalassemia

Answer

Iron deficiency anemia

Answer

Lead poisoning

Question 9

What is the treatment for chemotherapy-induced agranulocytopenia?

Accepted Answer

Granulocyte colony-stimulating factor (G-CSF)

Answer

Packed red blood cells

Answer

Platelet transfusion

Answer

Granulocyte-macrophage colony-stimulating factor (GM-CSF), less commonly used.

Question 10

A girl who developed abdominal pain and presented to the emergency department with a seizure after taking sulphonamides is likely to have a diagnosis of:

Accepted Answer

Acute intermittent porphyria due to other triggers

Answer

Congenital erythropoietic porphyria with skin manifestations

Answer

Infectious mononucleosis with fever and lymphadenopathy

Answer

Kawasaki disease with prolonged fever and rash

Hematology — MCQs

Hematology — MCQs

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