Distal renal tubular acidosis in lymphoplasmacytic lymphoma Waldenström's macroglobulinemia: A case report

Department of Medical Education, Internal and Preventive Medicine, Griffin Hospital and Yale University School of Medicine USA.
Cases Journal 07/2009; 2(7):7198. DOI: 10.4076/1757-1626-2-7198
Source: PubMed


We present a case of distal renal tubular acidosis and acute renal insufficiency in a patient with Waldenström's macroglobulinemia. Distal renal tubular acidosis has been described in hypergammaglobulinemia, but not in patients with Waldenström's macroglobulinemia. To our knowledge, this is the first report to describe a possible association between distal renal tubular acidosis and Waldenström's macroglobulinemia. Our case also emphasizes the importance of prompt recognition of distal renal tubular acidosis in patients with Waldenström's macroglobulinemia because of its metabolic disturbances and potentially life threatening complications.

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    ABSTRACT: Nephrogenic diabetes insipidus (DI) is characterized by inability of the kidney to concentrate urine due to diminished renal response to vasopressin. We report a patient with diabetes insipidus and hy-perviscosity as presenting manifestations of Waldenstrom's mac-roglobulinemia. After two treatments with plasmapheresis, serum viscosity normalized and the ability to concentrate urine, polyuria, and polydipsia improved. With ongoing chemoimmunotherapy, he has not had a recurrence of DI. Although rare, nephrogenic DI is a complication of hematologic disorders such as Waldenstrom's mac-roglobulinemia, multiple myeloma and sickle cell anemia.
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    ABSTRACT: Therapy of Waldenström´s macroglobulinaemia (WM) is indicated in patients with clinically relevant symptoms. Therapeutic plasmapheresis should be performed in cases with hyperviscosity. The intensity of chemotherapy should be adjusted to the degree of cytopenia. Monotherapy with rituximab is recommended in cases with severe cytopenia, also combination of rituximab with dexamethasone should be possible. Patients with symptomatic WM without severe cytopenia should received a rituximab-containing regimens, optimal variant is combination of rituximab + dexamethasone + alkylation drug (such as cyclophosphamide or bendamustine). Possible treatment combinations are for instance R-CHOP (rituximab, cyclophosphamide, vincristine, and prednisone), R-COP (rituximab, cyclophosphamide, and prednisone), or RCD (rituximab, cyclophosphamide, and dexamethasone). The choice of regimen in individual patients will take into consideration performance status, clinical features including renal function, comorbidities and potential candidacy for stem cell transplantation.Chlorambucil and rituximab is possible treatment options for older patients. The choice of treatment of WM relapse depends on the time of treatment response. Retreatment with primary therapy may be appropriate in patients with duration of treatment response at least 2 years. Other treatment possibilities for WM relapse are regimens containing fludarabine, cladribine or bortezomib. Autologous transplantation of peripheral blood stem cells is feasible therapeutic option for relapsed WM in younger, fitter patients with aggressive chemosensitive disease. Key words: bendamustin - bortezomib - rituximab - hyperviscosity - cryoglobulinemia - cold agglutinin disease - monoclonal imunoglobulin related disorders - Waldenström´s macroglobulinaemia.
    Vnitr̆ní lékar̆ství 02/2014; 60(2):139-57.


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