Abstract: SA-PO235
A Case of Waldenström Macroglobulinemia Recovering from Dialysis-Requiring AKI by Steroid Monotherapy
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Kutsuzawa, Naokata, Tokai University School of Medicine, Isehara, Japan
- Nakagawa, Yosuke, Tokai University School of Medicine, Isehara, Japan
- Hamano, Naoto, Tokai University School of Medicine, Isehara, Japan
- Koizumi, Masahiro, Tokai University School of Medicine, Isehara, Japan
- Wada, Takehiko, Tokai University School of Medicine, Isehara, Japan
- Fukagawa, Masafumi, Tokai University School of Medicine, Isehara, Japan
Introduction
Lymphoplasmacytic lymphoma (LPL) is an uncommon mature B cell lymphoma, whereas Waldenström macroglobulinemia (WM) is a subset of LPL involving the bone marrow with an IgM monoclonal gammopathy in blood. WM mainly presents with symptoms associated with anemia and hyperviscosity syndrome. WM-associated nephropathy is rare and its prognosis is unknown. Here, we report a case of LPL/WM recovering from dialysis-requiring acute kidney injury by steroid monotherapy.
Case Description
A 76-year-old Japanese woman who had been previously healthy admitted to the hospital with complaint of abdominal pain. She became anuric soon after admission, followed by progressive dyspnea due to pulmonary edema. She was referred to our hospital and hemodialysis was started for volume control. She presented with no neurological symptoms but funduscopy showed dilated and tortuous retinal veins with hemorrhagic exudates, which suggested hyperviscosity syndrome. She had severe anemia with rouleaux formation, high level of serum IgM (3776 mg/dl) and monoclonal IgM with lambda light chain on serum immunoelectrophoresis. Bone marrow biopsy demonstrated infiltration by small lymphocytes that exhibit plasma cell differentiation and she was given a diagnosis of LPL/WM. Renal biopsy demonstrated acute tubular necrosis with cast nephropathy and neoplastic lymphocytic infiltration in the interstitium. There were IgM deposits in the glomerular basement membrane and widespread thrombi in the capillaries. She underwent the treatment with high-dose dexamethasone, which steadily made her renal insufficiency ameliorated. Finally, hemodialysis therapy was discontinued at the 31st hospital day.
Discussion
Prognosis of WM-associated nephropathy depends on the type of renal pathology. This case suggested that acute kidney injury mainly caused by neoplastic lymphocytic infiltration might be reversible by chemotherapy.