Journal
BLOOD
Volume 137, Issue 15, Pages 2001-2009Publisher
AMER SOC HEMATOLOGY
DOI: 10.1182/blood.2021010898
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Funding
- American-Italian Cancer Foundation PostDoctoral Research Fellowship
- National Institutes of Health, National Heart, Lung, and Blood Institute [R35HL135795]
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Pure red cell aplasia (PRCA) is a rare hematological disorder with multiple etiologies and diverse clinical features. Prompt recognition of pathogenetic clues is crucial for diagnosis and treatment, with treatment options varying depending on the clinical scenario. Personalized therapeutic recommendations are essential for effective management of PRCA.
Pure red cell aplasia (PRCA) is a rare hematological disorder with multiple etiologies. The multifaceted nature of this disease is emphasized by the variety of concomitant clinical features. Classic idiopathic presentation aside, prompt recognition of pathogenetic clues is important because of their diagnostic and therapeutic implications. As a consequence, treatment of PRCA is diverse and strictly dependent on the presented clinical scenario. Here, we propose a series of clinical vignettes that showcase instructive representative situations derived from our routine clinical practice. Using these illustrative clinical cases, we review the diagnostic workup needed for a precise diagnosis and the currently available therapeutic options, discussing their applications in regard to the various PRCA-associated conditions and individual patients' characteristics. Finally, we propose a treatment algorithm that may offer guidance for personalized therapeutic recommendations.
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