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In hopes of better understanding the distribution, importance, and clinical significance of these potential markers, a UPLC\u2010MS\/MS method was developed and validated to quantify lyso\u2010Gb<jats:sub>1<\/jats:sub> and six related analogs (with the following modifications on the sphingosine moiety: \u2010C<jats:sub>2<\/jats:sub>H<jats:sub>4<\/jats:sub> (\u201028 Da), \u2010C<jats:sub>2<\/jats:sub>H<jats:sub>4<\/jats:sub>+O (\u201012 Da), \u2010H<jats:sub>2<\/jats:sub> (\u20102 Da), \u2010H<jats:sub>2<\/jats:sub>+O (+14 Da), +O (+16 Da), and +H<jats:sub>2<\/jats:sub>O (+18 Da)), sphingosylphosphorylcholine, and <jats:italic>N\u2010<\/jats:italic>palmitoyl\u2010O\u2010phosphocholineserine in plasma specimens of treated and untreated patients. This 12\u2010min UPLC\u2010MS\/MS method involves a purification step via solid\u2010phase extraction followed by evaporation under nitrogen flow and resuspension in an organic mix compatible with HILIC chromatography. This method is currently used for research purposes and might be used for monitoring, prognostics, and follow\u2010up. \u00a9 2023 The Authors. 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