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The decision to perform splenectomy or hematopoietic stem cell transplantation is nuanced and depends on disease burden and long-term outlook given that targeted therapeutics are in development. In recognition of the complicated nature of the disease and its management and the limitations of the PKD literature, an international working group of 10 PKD experts convened to better define the burden and manifestations of disease. This manuscript summarizes the conclusions of this working group and is a guide for clinicians