Autosomal dominant polycystic kidney disease (
ADPKD) is the most common hereditary
kidney disease, responsible for 10% of patients on
renal replacement therapy. The disease is well known to be associated with many extrarenal manifestations.
Leukopenia may also be present, even if it is not commonly identified as a typical extrarenal manifestation. Herein we describe two case reports of
ADPKD patients with
leukopenia. The first case is about a 47-year-old patient affected by
ADPKD, regularly treated with
peritoneal dialysis, who showed a progressive reduction of white blood cell count, mostly of lymphocytes. Lymphocytic
leukopenia was so severe that, when he was called for
transplantation from a deceased donor, he was considered temporarily not eligible. We then describe a second
ADPKD patient regularly treated with
peritoneal dialysis, who had stable
lymphopenia for years. Six years after starting PD, it was necessary to perform bone marrow aspirate to investigate the simultaneous presence of
hypogammaglobulinemia together with M-
protein and to exclude
monoclonal gammopathy. All the exams performed did not show any significant results, the patients were re-included in the waiting list and one of them was transplanted. Given our experience and what is reported in the literature, there seems to be enough evidence to consider
leukopenia as an extrarenal manifestation of
ADPKD. However, the clinical significance of
leukopenia in
ADPKD patients is not known. It could be interesting to investigate the leucocytes' function and if
ADPKD patients with
leukopenia are more susceptible to
infection, or not. Moreover, it would be very useful to analyze the relationship between such manifestation and genotype/phenotype.