Hematology Practice Questions

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: 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: 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.

Q: Which of the following is more likely associated with a platelet disorder rather than hemophilia A?

Epistaxis. ***Epistaxis*** - **Epistaxis**, or nosebleeds, is commonly associated with **platelet disorders** due to poor **platelet function** or **thrombocytopenia** [1]. - It occurs due to **vascular fragility** and inadequate platelet response, unlike hemophilia A, which primarily affects coagulation factors [1]. *Late rebleeding* - **Late rebleeding** is more characteristic of **coagulation disorders** like hemophilia A, where hemophilic patients may experience delayed bleeding after injury. - It results from factors' prolonged activity rather than intrinsic platelet dysfunction. *Hemarthrosis* - **Hemarthrosis** (bleeding into joints) is a classic manifestation of hemophilia A, specifically due to deficiencies in **coagulation factors** [1]. - This is not typical for platelet disorders, which do not significantly affect joints [1]. *Prolonged partial thromboplastin time* - A **prolonged partial thromboplastin time (PTT)** is indicative of deficiencies in intrinsic pathway factors, which is more aligned with hemophilia rather than platelet disorders. - Platelet disorders typically do not affect PTT but may prolong bleeding time due to poor platelet function [2].

Q: A 15-year-old boy suffering from Hodgkin's disease with lymph node involvement. Which treatment option is likely to have a high success rate?

Chemotherapy. ***Chemotherapy*** - **Hodgkin's disease** (lymphoma) is a systemic malignancy, and chemotherapy is the cornerstone of treatment, especially for widespread disease like lymph node involvement [1]. - Combination chemotherapy regimens, such as **ABVD** (Adriamycin, Bleomycin, Vinblastine, Dacarbazine), achieve high cure rates in pediatric Hodgkin's lymphoma. *Surgical resection of lymph nodes* - Surgical resection is generally **not curative** for Hodgkin's disease because it is a systemic malignancy, meaning cancer cells can be present in other lymph nodes or organs not visible [1]. - It might be used for **biopsy** or to debulk very large masses causing symptoms, but not as primary curative therapy [1]. *Targeted immunotherapy* - While immunotherapy agents like **Brentuximab vedotin** are used in Hodgkin's lymphoma, they are typically reserved for **relapsed/refractory disease** or in specific high-risk settings, not usually as first-line monotherapy. - Traditional chemotherapy remains the primary curative approach for initial presentation with lymph node involvement [1]. *Surgery combined with immunotherapy* - This combination is **not the standard primary treatment** for Hodgkin's lymphoma with lymph node involvement. - Surgery has a limited role, and while immunotherapy has a role, it's often in conjunction with chemotherapy or for specific cases, not typically as the initial combined approach with surgery.

Q: A female with recurrent abortion and isolated prolonged activated partial thromboplastin time (APTT) is most likely associated with.

Lupus anticoagulant. ***Lupus anticoagulant*** - **Lupus anticoagulant** is an antibody that paradoxically prolongs **APTT** [1] in vitro but is associated with a **prothrombotic state** in vivo, leading to recurrent abortions. - It is a key feature of **Antiphospholipid Syndrome (APS)**, which classically presents with **recurrent pregnancy loss and thrombotic events**. *DIC* - **Disseminated Intravascular Coagulation (DIC)** involves widespread activation of the clotting cascade, leading to both **thrombosis** and **hemorrhage**. - While it can cause prolonged **APTT**, it would also be characterized by **thrombocytopenia**, **prolonged PT**, and elevated **D-dimer** [2], which are not mentioned. *Von Willebrand disease* - **Von Willebrand disease (vWD)** is a common **bleeding disorder** due to deficiency or dysfunction of **von Willebrand factor** [3]. - It can cause a prolonged **APTT** (if factor VIII levels are significantly low) and bleeding, but it is not typically associated with **recurrent abortions** due to thrombosis [3]. *Hemophilia* - **Hemophilia** (A or B) is an **X-linked recessive bleeding disorder** characterized by a deficiency of **Factor VIII (Hemophilia A)** or **Factor IX (Hemophilia B)**. - It causes a prolonged **APTT** and significant bleeding episodes, but it is not associated with **thrombosis** or **recurrent abortions**.

Question 1

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 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

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

Accepted Answer

Leukopenia

Answer

Osteomyelitis

Answer

Papillary necrosis

Answer

Rheumatoid arthritis

Question 4

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 5

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 6

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 7

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

Question 8

Which of the following is more likely associated with a platelet disorder rather than hemophilia A?

Accepted Answer

Epistaxis

Answer

Late rebleeding

Answer

Hemarthrosis

Answer

Prolonged activated partial thromboplastin time

Question 9

A 15-year-old boy suffering from Hodgkin's disease with lymph node involvement. Which treatment option is likely to have a high success rate?

Accepted Answer

Chemotherapy

Answer

Surgical resection of lymph nodes

Answer

Targeted immunotherapy

Answer

Surgery combined with immunotherapy

Question 10

A female with recurrent abortion and isolated prolonged activated partial thromboplastin time (APTT) is most likely associated with.

Accepted Answer

Lupus anticoagulant

Answer

Hemophilia

Answer

Von Willebrand disease

Answer

DIC

Hematology — MCQs

Hematology — MCQs

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