Hematology (anemias, clotting disorders) Practice Questions

Q: A 54-year-old woman with a 30-pack-year smoking history presents with 6 months of progressive exertional dyspnea and a chronic productive cough. She denies orthopnea or paroxysmal nocturnal dyspnea. Vitals are notable for oxygen saturation of 91% on room air. Chest auscultation reveals diffuse expiratory wheezes and a prolonged expiratory phase. Spirometry is performed; post-bronchodilator testing reveals an FEV1/FVC ratio of 0.62 with an FEV1 of 58% predicted, and repeat testing after bronchodilator administration shows less than 12% and less than 200 mL improvement in FEV1. Which of the following spirometric patterns is most consistent with this patient's presentation?

FEV1/FVC ratio <0.70 with FEV1 <80% predicted, not fully reversible after bronchodilator. ***FEV1/FVC ratio <0.70 with FEV1 <80% predicted, not fully reversible after bronchodilator*** - This patient presents with hallmark signs of **Chronic Obstructive Pulmonary Disease (COPD)**, defined by a **post-bronchodilator FEV1/FVC ratio 0.70 with proportional reduction in FEV1 and FVC* - This pattern is characteristic of **restrictive lung disease**, where both volumes decrease but the ratio remains normal or increased. - It is inconsistent with this patient's presentation of **obstructive symptoms** like wheezing and a prolonged expiratory phase. *FEV1/FVC ratio 12% and >200 mL improvement after bronchodilator* - This describes **reversible airway obstruction**, which is the diagnostic hallmark of **asthma** rather than COPD. - While COPD patients may show some improvement, a full reversal or a significant **bronchodilator response** as defined here points toward an alternative diagnosis. *FEV1/FVC ratio >0.70 with FVC <80% predicted and normal TLC on plethysmography* - A normal **FEV1/FVC ratio** excludes the primary diagnosis of an obstructive disease like **COPD**. - A normal **Total Lung Capacity (TLC)** on plethysmography would further rule out **restrictive lung disease**, suggesting a normal variant or poor effort rather than pathology. *Normal FEV1/FVC ratio with a scooped flow-volume loop on the expiratory limb only* - A **scooped expiratory limb** is classic for obstruction, but it must be accompanied by a **decreased FEV1/FVC ratio** to meet diagnostic criteria. - A normal ratio would indicate that the airflow limitation is not clinically significant enough to qualify as **COPD**.

Q: A 45-year-old woman presents with confusion and fever. Labs show: hemoglobin 7.8 g/dL, platelets 15,000/μL, creatinine 2.8 mg/dL, LDH 890 U/L, total bilirubin 3.1 mg/dL (indirect), undetectable haptoglobin. Peripheral smear shows numerous schistocytes. ADAMTS13 activity is 8% (normal >67%). She has no diarrheal illness. Coagulation studies (PT/aPTT) are normal. Evaluate the most appropriate immediate therapeutic intervention.

Immediate plasma exchange with FFP replacement. ***Immediate plasma exchange with FFP replacement*** - The patient exhibits the classic pentad of **Thrombotic Thrombocytopenic Purpura (TTP)**: microangiopathic hemolytic anemia (**schistocytes**, high LDH, low haptoglobin), thrombocytopenia, fever, renal dysfunction, and confusion. - **Plasma exchange (PEX)** is the definitive immediate treatment as it removes autoantibodies against **ADAMTS13** and restores the deficient enzyme levels to prevent further microthrombi formation. *Platelet transfusion for severe thrombocytopenia* - Platelet transfusions are generally **contraindicated** in TTP as they can "fuel the fire" by worsening **microvascular thrombosis** and exacerbating organ ischemia. - This intervention is reserved only for patients with life-threatening **hemorrhage**. *High-dose corticosteroids and rituximab* - Although these are used to suppress the production of **ADAMTS13 autoantibodies**, they are **adjunctive therapies** and cannot replace the immediate need for PEX. - Corticosteroids do not provide the necessary replacement of the **ADAMTS13 protease** required to break down large vWF multimers. *Eculizumab for atypical hemolytic uremic syndrome* - While atypical HUS shares features with TTP, the significantly low **ADAMTS13 activity (<10%)** confirms TTP, making eculizumab (a **C5 inhibitor**) inappropriate here. - Atypical HUS is typically caused by **complement dysregulation**, whereas this case is driven by a protease deficiency. *Supportive care and dialysis for renal failure* - While the patient has elevated creatinine, dialysis is only a **supportive measure** for renal failure and does not address the systemic **microangiopathy** driving the TTP. - Relying on supportive care alone in TTP is associated with a **mortality rate** exceeding 90%.

Question 1

A 58-year-old woman with a 35-pack-year smoking history presents with progressive dyspnea on exertion over 6 months, a chronic productive cough, and two hospitalizations for respiratory infections in the past year. She uses an albuterol inhaler as needed. Her mMRC dyspnea score is 2 and CAT score is 18. Spirometry is performed. Post-bronchodilator FEV₁/FVC is 0.58 and FEV₁ is 48% of predicted. She has no wheezing at rest and her chest X-ray shows hyperinflation with flattened diaphragms. Which of the following changes to her maintenance regimen is most appropriate at this time?

Accepted Answer

Add a long-acting muscarinic antagonist and a long-acting beta-2 agonist

Answer

Initiate high-dose inhaled corticosteroid monotherapy

Answer

Begin oral theophylline as a bronchodilator adjunct

Answer

Prescribe a short-acting muscarinic antagonist scheduled four times daily

Answer

Start long-term oral corticosteroids to reduce exacerbation frequency

Question 2

A 54-year-old woman with a 30-pack-year smoking history presents with 6 months of progressive exertional dyspnea and a chronic productive cough. She denies orthopnea or paroxysmal nocturnal dyspnea. Vitals are notable for oxygen saturation of 91% on room air. Chest auscultation reveals diffuse expiratory wheezes and a prolonged expiratory phase. Spirometry is performed; post-bronchodilator testing reveals an FEV1/FVC ratio of 0.62 with an FEV1 of 58% predicted, and repeat testing after bronchodilator administration shows less than 12% and less than 200 mL improvement in FEV1. Which of the following spirometric patterns is most consistent with this patient's presentation?

Accepted Answer

FEV1/FVC ratio <0.70 with FEV1 <80% predicted, not fully reversible after bronchodilator

Answer

FEV1/FVC ratio >0.70 with proportional reduction in FEV1 and FVC

Answer

FEV1/FVC ratio <0.70 with FEV1 >12% and >200 mL improvement after bronchodilator

Answer

FEV1/FVC ratio >0.70 with FVC <80% predicted and normal TLC on plethysmography

Answer

Normal FEV1/FVC ratio with a scooped flow-volume loop on the expiratory limb only

Question 3

A 19-year-old college student returns from a mission trip to Kenya. Three weeks later, he develops fatigue, dark urine, and jaundice. Labs show: hemoglobin 8.1 g/dL (baseline 14 g/dL), MCV 92 fL, reticulocyte count 9%, total bilirubin 4.8 mg/dL (indirect), LDH 720 U/L. Peripheral smear shows bite cells and Heinz bodies. Malaria smears are negative. He reports taking primaquine for malaria prophylaxis. Which of the following is the most likely underlying condition?

Accepted Answer

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Acute malaria with hemolytic crisis despite negative smear

Answer

Hereditary spherocytosis unmasked by oxidative stress

Answer

Autoimmune hemolytic anemia triggered by infection

Question 4

A 35-year-old woman with lupus anticoagulant and history of two miscarriages is now 10 weeks pregnant. She had a DVT 3 years ago. She is currently asymptomatic. Her obstetrician requests recommendations for antithrombotic management throughout pregnancy and postpartum period. Synthesize the optimal comprehensive management strategy.

Accepted Answer

Prophylactic LMWH plus aspirin throughout pregnancy, therapeutic LMWH postpartum

Answer

Therapeutic dose LMWH throughout pregnancy and 6 weeks postpartum

Answer

Warfarin with INR 2-3 after first trimester, then switch to LMWH at term

Answer

Prophylactic dose LMWH throughout pregnancy, continue 6 weeks postpartum

Answer

Aspirin 81 mg daily only throughout pregnancy

Question 5

A 45-year-old woman presents with confusion and fever. Labs show: hemoglobin 7.8 g/dL, platelets 15,000/μL, creatinine 2.8 mg/dL, LDH 890 U/L, total bilirubin 3.1 mg/dL (indirect), undetectable haptoglobin. Peripheral smear shows numerous schistocytes. ADAMTS13 activity is 8% (normal >67%). She has no diarrheal illness. Coagulation studies (PT/aPTT) are normal. Evaluate the most appropriate immediate therapeutic intervention.

Accepted Answer

Immediate plasma exchange with FFP replacement

Answer

High-dose corticosteroids and rituximab

Answer

Eculizumab for atypical hemolytic uremic syndrome

Answer

Platelet transfusion for severe thrombocytopenia

Answer

Supportive care and dialysis for renal failure

Question 6

A 70-year-old man with metastatic pancreatic cancer on chemotherapy develops sudden dyspnea and hypoxia. CT pulmonary angiogram confirms bilateral pulmonary emboli. Platelet count is 45,000/μL (down from 180,000/μL two weeks ago). He has normal renal and liver function. Oncologist reports he has an estimated survival of 6-8 months. Evaluate the optimal long-term anticoagulation strategy.

Accepted Answer

Low molecular weight heparin (LMWH) for cancer-associated thrombosis

Answer

Apixaban with dose reduction for thrombocytopenia

Answer

Unfractionated heparin infusion continued indefinitely

Answer

Inferior vena cava filter placement without anticoagulation

Answer

Warfarin with INR goal 2-3

Question 7

A 24-year-old African American woman presents with severe bone pain and fever. She has sickle cell disease. Temperature is 39.2°C (102.6°F), blood pressure 110/65 mmHg. Hemoglobin is 6.1 g/dL (baseline 8.5 g/dL), reticulocyte count 0.5%, WBC 3,200/μL. Chest X-ray is clear. Blood cultures are pending. Analyze the clinical presentation and determine the priority intervention.

Accepted Answer

Simple transfusion for aplastic crisis and broad-spectrum antibiotics

Answer

Immediate bone marrow transplant evaluation

Answer

Exchange transfusion for acute chest syndrome

Answer

Intravenous immunoglobulin for parvovirus B19

Answer

Hydroxyurea initiation and pain management

Question 8

A 55-year-old man with chronic kidney disease (GFR 25 mL/min) develops deep vein thrombosis. He is started on low molecular weight heparin (LMWH) and warfarin. After 5 days, his INR is 2.8, but before stopping LMWH, laboratory studies show: platelet count decreased from 280,000/μL to 85,000/μL. He has no bleeding. Peripheral smear shows no schistocytes. Analyze the most likely diagnosis and appropriate action.

Accepted Answer

Heparin-induced thrombocytopenia (HIT); stop all heparin and start argatroban

Answer

Warfarin-induced thrombocytopenia; stop warfarin and continue LMWH

Answer

Disseminated intravascular coagulation; begin supportive care

Answer

Immune thrombocytopenic purpura; start prednisone

Answer

Uremic platelet dysfunction; initiate desmopressin therapy

Question 9

A 32-year-old woman presents with fatigue and dyspnea. Labs show hemoglobin 7.2 g/dL, MCV 68 fL, ferritin 8 ng/mL, and total iron binding capacity 450 μg/dL. She was started on oral ferrous sulfate 325 mg three times daily. After 4 weeks, her hemoglobin is unchanged at 7.3 g/dL. She reports taking the medication as prescribed. What factor most likely explains the lack of response?

Accepted Answer

Ongoing blood loss exceeding replacement

Answer

Celiac disease causing malabsorption

Answer

Anemia of chronic disease coexisting with iron deficiency

Answer

Concurrent calcium supplement intake

Answer

Thalassemia trait misdiagnosed as iron deficiency

Question 10

A 65-year-old man with atrial fibrillation on warfarin (INR goal 2-3) presents to the emergency department after a fall with head trauma. His INR is 8.5. CT head shows a small subdural hematoma with no midline shift. He is alert and neurologically intact. Blood pressure is 145/85 mmHg. What is the most appropriate immediate management?

Accepted Answer

Administer prothrombin complex concentrate (PCC) and vitamin K 10 mg IV

Answer

Administer platelets and cryoprecipitate

Answer

Administer vitamin K 10 mg IV and fresh frozen plasma

Answer

Observe and repeat INR in 6 hours

Answer

Give vitamin K 5 mg PO only

Hematology (anemias, clotting disorders) — MCQs

Hematology (anemias, clotting disorders) — MCQs

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