Yellow Nail Syndrome (YNS) is a rare entity characterised by the triad of nail discolouration, lung manifestations/sinusitis and lymphoedema. With ongoing debate, the exact aetiological mechanisms of YNS remain unknown. YNS is associated with various conditions such as malignancies, autoimmune and immuno-deficiency diseases. Some authors consider YNS as a paraneoplastic phenomenon due to its association with malignancies. Here we report the first patient presenting with the typical triad of YNS and a consecutive diagnosis of acute myeloid leukaemia with recurrent genetic abnormalities (KMT2A-PTD). Nail symptoms showed partial recovery after initiation of chemotherapy. Currently, the patient is off therapy and remains in first complete remission. More research to identify the exact pathophysiological mechanism and clinical significance of YNS is needed.

(BELG J HEMATOL 2022;13(6):249–52)