Hematology (anemias, clotting disorders) Practice Questions

Q: A 28-year-old woman with menorrhagia presents with easy bruising and epistaxis. Platelet count is 18,000/μL. PT and aPTT are normal. Peripheral smear shows decreased platelets with normal morphology. Bone marrow biopsy reveals increased megakaryocytes. She was treated with prednisone 1 mg/kg for 4 weeks without response. What is the most appropriate next step?

Rituximab therapy. ***Rituximab therapy*** - This patient has **Immune Thrombocytopenic Purpura (ITP)** and has failed first-line **corticosteroid** therapy; **Rituximab** is a preferred second-line monoclonal antibody directed against **CD20** on B-cells. - It is effective in reducing the production of **anti-platelet antibodies** and is often chosen before surgical interventions like splenectomy in modern clinical practice. *Splenectomy* - While **splenectomy** is a valid second-line treatment, it is an invasive procedure generally deferred until pharmacological options like **Rituximab** or **TPO-receptor agonists** are considered. - It carries a lifelong risk of **overwhelming post-splenectomy infection (OPSI)**, requiring preoperative vaccinations against encapsulated organisms. *Intravenous immunoglobulin (IVIG)* - **IVIG** is used for **acute management** to rapidly increase platelet counts in cases of severe bleeding or prior to surgery. - Its effects are **temporary** (lasting only 1–2 weeks) and it is not an appropriate long-term strategy for patients who are steroid-refractory. *Romiplostim (thrombopoietin receptor agonist)* - **Romiplostim** is a second-line option that stimulates **megakaryocytes** via the **TPO receptor** to increase platelet production. - Though effective, it requires weekly subcutaneous injections and is often ranked alongside or after **Rituximab** depending on patient preference and clinician experience. *Increase prednisone to 2 mg/kg* - Increasing the dose of **prednisone** beyond 1 mg/kg is not recommended as it significantly increases **systemic toxicity** without improving the remission rate. - Failure to respond to 1 mg/kg after 4 weeks defines the patient as **steroid-refractory**, necessitating a switch to second-line agents rather than dose escalation.

Q: A 45-year-old African American man presents with fatigue and jaundice. Laboratory studies show hemoglobin 8.5 g/dL, MCV 88 fL, reticulocyte count 8%, total bilirubin 4.2 mg/dL (predominantly indirect), and LDH 650 U/L. Peripheral blood smear shows spherocytes and polychromasia. Direct Coombs test is positive. He recently started taking methyldopa for hypertension. What is the most appropriate next step in management?

Discontinue methyldopa and observe. ***Discontinue methyldopa and observe*** - The patient presents with **drug-induced Warm Autoimmune Hemolytic Anemia (WAIHA)**, as evidenced by a **positive Direct Coombs test**, anemia, and jaundice shortly after starting **methyldopa**. - Methyldopa induces the production of true **autoantibodies** against red blood cell antigens; the most appropriate and often curative first step is the **withdrawal of the offending drug**. *Start prednisone 1 mg/kg daily* - **Corticosteroids** are the first-line treatment for **primary (idiopathic) WAIHA**, but they are not the immediate priority when a clear drug-induced cause is identified. - Most cases of **methyldopa-induced hemolysis** resolve gradually within weeks after the medication is stopped without requiring systemic steroids. *Perform splenectomy* - **Splenectomy** is a **second-line intervention** reserved for patients with WAIHA who are refractory to or dependent on high doses of corticosteroids. - It is inappropriate in the initial management of **drug-induced hemolysis** where the trigger can be easily removed. *Initiate rituximab therapy* - **Rituximab**, an anti-CD20 monoclonal antibody, is utilized for **refractory AIHA** or cases where patients fail initial steroid therapy. - It is an expensive treatment with potential side effects and is not indicated as the **next step** before assessing response to drug discontinuation. *Begin plasmapheresis immediately* - **Plasmapheresis** is generally ineffective in WAIHA because **IgG antibodies** are primarily located on the RBC surface and have a large volume of distribution. - This procedure is typically reserved for extreme cases of **Thrombotic Thrombocytopenic Purpura (TTP)** or occasionally Cold Agglutinin Disease, but not drug-induced WAIHA.

Question 1

A 28-year-old woman with menorrhagia presents with easy bruising and epistaxis. Platelet count is 18,000/μL. PT and aPTT are normal. Peripheral smear shows decreased platelets with normal morphology. Bone marrow biopsy reveals increased megakaryocytes. She was treated with prednisone 1 mg/kg for 4 weeks without response. What is the most appropriate next step?

Accepted Answer

Rituximab therapy

Answer

Intravenous immunoglobulin (IVIG)

Answer

Romiplostim (thrombopoietin receptor agonist)

Answer

Splenectomy

Answer

Increase prednisone to 2 mg/kg

Question 2

A 45-year-old African American man presents with fatigue and jaundice. Laboratory studies show hemoglobin 8.5 g/dL, MCV 88 fL, reticulocyte count 8%, total bilirubin 4.2 mg/dL (predominantly indirect), and LDH 650 U/L. Peripheral blood smear shows spherocytes and polychromasia. Direct Coombs test is positive. He recently started taking methyldopa for hypertension. What is the most appropriate next step in management?

Accepted Answer

Discontinue methyldopa and observe

Answer

Initiate rituximab therapy

Answer

Start prednisone 1 mg/kg daily

Answer

Begin plasmapheresis immediately

Answer

Perform splenectomy

Hematology (anemias, clotting disorders) — MCQs

Hematology (anemias, clotting disorders) — MCQs

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