The Course of an Autosomal Dominant Polycystic Kidney Disease: Rare Association with Waldenström Macroglobulinemia and other Co-occurrences

Resumo

We present a case of a 50-year-old female, known to have a history of autosomal dominant polycystic kidney disease (ADPKD) and controlled hypertension, who was referred for polycystic liver. Her family history is remarkable for her father, who received a renal transplant in the setting of ADPKD. The patient remained under regular follow-up with normal renal and hepatic function. She developed recurrent episodes of pain in the upper abdomen, malnutrition and cyst infection. At this point, she had signs of hepatic venous outflow obstruction and imaging showed massive liver enlargement. The patient was proposed for a liver transplant. A decade after ADPKD was identified, she had been diagnosed with Waldenström macroglobulinemia (WM). Hematological malignancies have rarely been described in coexistence with ADPKD. There is one such reported case with multiple myeloma. However, in our search, we have not found any case of ADPKD-associated WM. This association should prompt thorough and frequent monitoring.