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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells. Misdiagnosed, patients suffer from true errancy of diagnosis mainly because of the large range of bio-clinical presentations. The following case of a 40-year-old male who went from an unexplored anemia to a falsely diagnosed systemic lupus then an Acute Kidney Injury (AKI) that required some dialysis sessions and later a kidney biopsy which the results threw us off track, highlights that exact problematic and showcases the accuracy and the simplicity of the test once the hypothesis strikes mind: The flow cytometry. Paroxysmal nocturnal hemoglobinuria must be evoked when there is a refractory anemia, a Coombs (-) anemia, thrombosis with hemolysis or cytopenia. The flow cytometry is considered the gold standard diagnostic tool.
ARZOUR et al. (Sat,) studied this question.