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Cross-Sex Hormone Therapy Complicated by Frequent ...
Cross-Sex Hormone Therapy Complicated by Frequent Sickle Cell Vaso-Occlusive Crises
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This case report describes a 25-year-old Black transgender woman with HbSS sickle cell disease (SCD) receiving cross-sex hormone therapy (CSHT) with estrogen for male-to-female transition. She had a history of acute chest syndrome, chronic left upper extremity DVT, and recurrent painful vaso-occlusive crises (VOC). She was admitted with severe back and leg pain consistent with prior VOC, along with leukocytosis and hemoglobin slightly below baseline. She improved after transfusion, opioid pain control, and supportive care, and was discharged on oral opioids.<br /><br />The report highlights an important clinical uncertainty: both SCD and exogenous estrogen are independently associated with increased thromboembolic risk, but the effect of estrogen-based CSHT on VOC frequency in transgender patients with SCD is unknown. In this patient, VOCs became frequent enough to require nine hospitalizations in one year while she continued CSHT. Despite discussion of possible increased risk, she consistently chose to remain on hormone therapy because it was central to her identity and quality of life.<br /><br />The authors note that current literature is very limited. Existing reports on transgender patients with SCD are sparse, and older estrogen-related data in cisgender women often involve ethinyl estradiol, which is not the same as the 17-β estradiol typically used in CSHT. Therefore, those findings cannot be directly applied to transgender women.<br /><br />The case emphasizes the need for more research on VOC risk in transgender people with SCD, better guidance for managing hormone therapy in this population, and strong patient advocacy. It also underscores how treatment decisions in transgender patients with SCD are deeply tied to self-identity and overall quality of life.
Asset Subtitle
Emily Jane Taylor
Meta Tag
Author List
Cindy L. Tsui, Emily Jane Taylor
Category
Clinical Vignettes
Concept
Gender-Affirming Hormone Therapy
Concept
Estrogen Therapy
Concept
Sickle Cell Disease
Concept
Vaso-occlusive Pain
Concept
Thromboembolic Risk
Distinguished
Non-Finalist
Presenter Organization
NYU Langone Health
Presenting Author
Emily Jane Taylor
Track
Adult
Keywords
sickle cell disease
HbSS
cross-sex hormone therapy
estrogen
transgender woman
vaso-occlusive crisis
thromboembolic risk
acute chest syndrome
deep vein thrombosis
patient advocacy
Gender-Affirming Hormone Therapy
Estrogen Therapy
Sickle Cell Disease
Vaso-occlusive Pain
Thromboembolic Risk
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