Sickle Cell Disease
Author and Disclosure Information [Show]

Vivien A. Sheehan, MD, PhD
Assistant Professor of Pediatrics, Baylor College of Medicine/Texas Children’s Hematology Center, Houston, TX

Question 1 of 5

A 23-year-old African American woman with homozygous hemoglobin SS (HbSS) disease presents to initiate care. She has been on monthly simple transfusion therapy since she was found to have a transcranial Doppler (TCD) velocity of 220 cm/sec in her right middle cerebral artery at age 7 years. She has not had an overt stroke, and her last magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) evaluation, at age 16, was negative for moyamoya or significant stenosis. There were mild ischemic changes in the frontal lobe consistent with silent infarcts. She has no allo- or autoantibodies, and is on an iron chelator, with a ferritin level under 1000 ng/mL. She wants to know when she can discontinue transfusion therapy.

How should the physician respond to this patient's question regarding stopping transfusion therapy?

She can stop now, as she is an adult and is no longer at risk for stroke

She can safely switch to hydroxyurea at any time, as it has been shown conclusively to prevent stroke with efficacy equal to that of chronic transfusions

She can undergo dural inversion therapy and discontinue transfusions

She should continue transfusion therapy indefinitely

Hospital Physician: Hematology/Oncology. 2017 January;12(1)

This quiz is not accredited for CME.

More from Board Review Questions: Hematology/Oncology


Autoimmune Hemolytic Anemia
Gastrointestinal Stromal Tumors
Testicular Cancer
Management of Advanced Melanoma
Castration-Resistant Prostate Cancer
Transfusion Reactions (part 2)
Transfusion Reactions (part 1)
Plasma Cell Disorders (part 3)
Plasma Cell Disorders (part 2)
Chronic Myeloid Leukemia
Aplastic Anemia
Small Cell Lung Cancer
Management of Metastatic Gastric Cancer
Transfusion Medicine
Management of Colorectal Cancer in Older Adults
Urothelial Carcinoma: Muscle-Invasive and Metastatic Disease
Acute Myeloid Leukemia
Pancreatic Adenocarcinoma: Advanced Unresectable and Metastatic Disease
HER2-Positive Breast Cancer
Aggressive B-Cell Non-Hodgkin Lymphoma
Von Willebrand Disease: Assessment and Diagnostic Testing
Von Willebrand Disease: Clinical Management
Pancreatic Adenocarcinoma: Neoadjuvant and Adjuvant Treatment
Late Pulmonary Complications After Hematopoietic Stem Cell Transplantation
Polycythemia Vera and Essential Thrombocythemia: Current Management
Treatment of Biliary Tract Cancers
Early Pulmonary Complications After Hematopoietic Stem Cell Transplantation
Hairy Cell Leukemia
Genomic Testing in Women with Early-Stage Hormone Receptor–Positive, HER2-Negative Breast Cancer
Immune Thrombocytopenia
Cancer-Related Fatigue
Disseminated Intravascular Coagulation
Advanced Stage and Relapsed/Refractory Hodgkin Lymphoma
Adjuvant Chemotherapy for Colon Cancer
Molecular Markers and Targeted Therapies in the Management of Non-Small Cell Lung Cancer
Metastatic Cutaneous Melanoma: Targeted Therapy and Immunotherapy
Thrombosis in Pregnancy
Locally Advanced Pancreatic Cancer
Early-Stage Hodgkin Lymphoma
Neoadjuvant and Adjuvant Therapy for Gastric Cancer
Hemophilia A and B
Mantle Cell Lymphoma
Soft Tissue Sarcoma
Renal Cell Carcinoma
Management of Locally Advanced Rectal Adenocarcinoma
Adjuvant Systemic Therapy for Early-Stage Breast Cancer
Chronic Myeloid Leukemia
Plasma Cell Disorders
Myelodysplastic Syndromes
Acute and Chronic Graft-versus-Host Disease