Decrease in PNH-type Blood Cells after the Cessation of Thrombopoietin Receptor Agonist in a Case of AA-PNH Syndrome.
Satoshi Ichikawa, Shunsuke Hatta, Noriko Fukuhara et al.
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Case in brief
A 64-year-old man with severe aplastic anemia had a partial response to immunosuppressive therapy, followed by sustained cytopenia recovery with a thrombopoietin receptor agonist (TPO-RA). Over subsequent years, serum lactate dehydrogenase gradually rose, and testing showed marked expansion of paroxysmal nocturnal hemoglobinuria (PNH) clones. After a transient hemolytic episode associated with SARS-CoV-2 infection, TPO-RA was stopped. Follow-up showed fewer PNH clones. Blood counts declined moderately but remained stable without transfusion support.
What made this case unusual
PNH clones decreased after TPO-RA withdrawal, despite the preceding marked expansion during treatment; the patient’s blood counts remained stable without transfusions.
Diagnostic / clinical pearl
In a patient with aplastic anemia receiving TPO-RA, rising lactate dehydrogenase and expanding PNH clones may warrant attention to clonal evolution and hemolysis; this case also documents clone reduction after treatment cessation.