Open AccessCombined renal proximal tubulopathy and crystal storing histiocytosis in a patient with κ light chain multiple myeloma
Author(s) -
Marco Ungari,
Paolo Ghiringhelli,
Gianluca Marchi,
Simona Fisogni,
Antonio LlombartBosch,
Alfredo Molteni,
Fabio Malberti,
Ramona Bertoni,
Monica Trombatore,
Giuseppina Ferrero,
Marino Daniel Gusolfino,
Elena Varotti,
Giulia Tanzi,
Laura Manotti
Publication year - 2021
Publication title -
pathologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.243
H-Index - 18
eISSN - 1591-951X
pISSN - 0031-2983
DOI - 10.32074/1591-951x-154
Subject(s) - multiple myeloma , medicine , pathology , immunofixation , bone marrow , immunoglobulin light chain , plasma cell dyscrasia , renal biopsy , glomerulopathy , kidney , glomerulonephritis , monoclonal , biopsy , immunology , monoclonal antibody , antibody
Multiple myeloma accounts for 10-15% of all hematologic malignancies, and 20% of deaths related to cancers of the blood and bone marrow. Diagnosis is defined by the presence of a serum monoclonal spike (M-spike) of more than 3 g/dL or more than 10% clonal plasma cells in the bone marrow and at least one myeloma-defining event, such as hypercalcemia, anemia, bone lesions, or renal impairment. The kidney is a major target organ, and renal impairment is frequently the first manifestation of the disease. Renal damage occurs in up to 40% of patients and 10-20% will require dialysis. Monoclonal immunoglobulin light chains are the major causes of renal complications in multiple myeloma. Glomerular disease, with the deposition of monoclonal immunoglobulins or their components, includes monoclonal immunoglobulin deposition disease, AL or AH amyloidosis, type I cryoglobulinemia, proliferative glomerulonephritis with monoclonal IgG deposits, immunotactoid glomerulopathy, and fibrillary glomerulonephritis. In addition, tubulointerstitial diseases with the deposition of monoclonal immunoglobulins or their components, are constituted by light chain cast nephropathy, light chain proximal tubulopathy, and crystal-storing histiocytosis.