sirolimus and Hydronephrosis

Erdheim-Chester disease is a rare non-Langerhans form of systemic histiocytosis of unknown origin. We describe a 45-year-old man presenting with bilateral hydronephrosis suggestive of extrinsic urinary tract obstruction. Computed tomography revealed extensive hypodense soft tissue infiltration in the retroperitoneum surrounding the kidneys. Needle biopsy of the retroperitoneal soft tissue revealed aggregates of lipid-laden histiocytes expressing CD68 but negative for CD1a and S100 protein. The diagnosis of Erdheim-Chester disease was supported by typical radionuclide bone scinitigraphic findings. Treatment with prednisolone, sirolimus, and regular ureteric stent revision was initiated to achieve adequate urinary tract drainage. To our knowledge, this is the second patient with Erdheim-Chester disease reported in Hong Kong. A high index of suspicion is required to avoid delay in the diagnosis of this rare disease.

Topics: Biopsy, Needle; Erdheim-Chester Disease; Hong Kong; Humans; Hydronephrosis; Male; Middle Aged; Prednisolone; Retroperitoneal Space; Sirolimus; Stents; Tomography, X-Ray Computed; Ureteral Obstruction

Anemia is common in patients with chronic kidney disease (CKD) and those who have received a kidney allograft. Anemia is most prevalent in kidney transplant recipients before and immediately after transplantation, but also can occur months after transplantation if the donor kidney begins to fail. Replacement therapy for CKD-related and posttransplantation anemia is effective through the administration of exogenous erythropoiesis-stimulating proteins. Darbepoetin alfa (Aranesp; Amgen Inc, Thousand Oaks, CA) is a unique erythropoiesis-stimulating protein that can be administered at an extended dosing interval relative to recombinant human erythropoietin because of its approximately 3-fold longer serum half-life. Although darbepoetin alfa has been shown to be an effective treatment for patients with anemia of CKD and anemia after kidney transplantation, limited data have been published showing efficacy in treating women with anemia of these conditions during pregnancy. We report a case of successful darbepoetin alfa treatment for severe anemia in a pregnant transplant recipient.

Topics: Adult; Anemia; Cesarean Section; Contraindications; Cyclosporine; Darbepoetin alfa; Erythropoietin; Female; Ferrous Compounds; Humans; Hydronephrosis; Immunosuppressive Agents; Kidney Transplantation; Nephrostomy, Percutaneous; Postoperative Complications; Pre-Eclampsia; Pregnancy; Pregnancy Complications, Hematologic; Pregnancy, High-Risk; Puerperal Disorders; Seizures; Sirolimus; Stents; Treatment Refusal