Rafet EREN, Tayfur TOPTAŞ, İşık Kaygusuz ATAGÜNDÜZ, Tülin Fıratlı TUĞLULAR

Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence

A previously healthy 31-year-old female presenting with dyspnea, iron deficiency anemia, pancytopenia, splenomegaly, and abnormal coagulation tests was admitted to the hospital. Hematology consultation additionally revealed that 71% of cells were indicative of acute promyelocytic leukemia (APL) and the patient tested positive for a t(15;17) translocation, confirming APL. All-trans retinoic acid (ATRA) therapy was initiated immediately, but the patient exhibited severe dyspnea. This subsequently resulted in circulatory and respiratory arrest, followed by death. Just after death, fluorescein-labelled proaerolysin (FLAER) revealed a paroxysmal nocturnal hemoglobinuria (PNH) monocyteclone of 82%, confirming the diagnosis of PNH. Leukemia can be derived from non-PNH clones in PNH patients. Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal

Anahtar Kelimeler:

Lösemi, Promyelositik, Akut, Hemoglobinuri, Proksismal

Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence

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