- Email: [email protected]
Crystal-storing histiocytosis
nephrology image
www.kidney-international.org
Crystal-storing histiocytosis Shivani Shah1, Sanjeev Sethi2, Lois Arend1 and Duvuru Geetha1 1
Johns Hopkins University, Baltimore, Maryland, USA; and 2Mayo Clinic, Rochester, Minnesota, USA
Correspondence: Shivani Shah, Johns Hopkins Bayview Medical Center, Division of Nephrology, Department of Medicine, 301 Lord Mason Drive, Suite 2500, Baltimore, Maryland 21224, USA. E-mail: [email protected] Kidney International (2016) 89, 507; http://dx.doi.org/10.1016/j.kint.2015.12.012
ª 2016 International Society of Nephrology
Figure 1 | Crystal-storing histiocytosis. Top panel (a–c) light microscopy showing numerous crystals in the glomerular capillary lumen and along the capillary walls (a: hematoxylin and eosin; b: silver methenamine; c: Masson trichome; original magnification all 40, arrows point to crystals). Middle panel (d–f) immunohistochemistry shows that crystals stain for kappa light chains are negative for lambda light chains and are mostly present in macrophages/histiocytes that stain for CD68. Immunofluorescence demonstrated granular staining of kappa light chains in the capillary lumens corresponding to histiocytes (not shown) (original magnification all 40). Bottom panel (g–i) electron microscopy showing crystals within macrophages as recognized by numerous cytoplasmic vacuoles (foam cells, black arrow points to macrophages containing crystals and vacuoles). Note that adjacent proximal tubule does not contain the crystals (white arrow) (g and i: low power; h: high power).
A
67-year-old Caucasian man with diabetes mellitus, hypertension, and antineutrophil cytoplasmic autoantibody– negative pauci-immune vasculitis presented with a rise in creatinine to 1.8 mg/dl from a baseline of 1.3 mg/dl, proteinuria, hematuria, new rash, ascites, and leukopenia. Serum protein electrophoresis revealed an IgG kappa monoclonal gammopathy, and cryoglobulin screen was trace positive. A renal biopsy was performed. The findings were consistent with a membranoproliferative glomerulonephritis with a crystal-storing histiocytosis secondary to an IgG kappa paraproteinemia (Figure 1). Crystal-storing histiocytosis is seen in patients with multiple myeloma and lymphoproliferative disorders; it is Kidney International (2016) 89, 507
characterized by an accumulation of light chain crystals in histiocytes that congregate in the bone marrow or other extramedullary location such as the cornea or kidney. When the kidneys are implicated, the histiocytes are often found in the interstitium. This is the first case describing localization of histiocytes in the capillary loops. In the absence of bone marrow involvement, this patient had a monoclonal gammopathy of renal significance, which may have heralded a diagnosis of multiple myeloma. He developed progressive hepatic and renal failure, myocardial infarction, and ileus. Ultimately, he opted for home hospice. 507
www.kidney-international.org
Crystal-storing histiocytosis Shivani Shah1, Sanjeev Sethi2, Lois Arend1 and Duvuru Geetha1 1
Johns Hopkins University, Baltimore, Maryland, USA; and 2Mayo Clinic, Rochester, Minnesota, USA
Correspondence: Shivani Shah, Johns Hopkins Bayview Medical Center, Division of Nephrology, Department of Medicine, 301 Lord Mason Drive, Suite 2500, Baltimore, Maryland 21224, USA. E-mail: [email protected] Kidney International (2016) 89, 507; http://dx.doi.org/10.1016/j.kint.2015.12.012
ª 2016 International Society of Nephrology
Figure 1 | Crystal-storing histiocytosis. Top panel (a–c) light microscopy showing numerous crystals in the glomerular capillary lumen and along the capillary walls (a: hematoxylin and eosin; b: silver methenamine; c: Masson trichome; original magnification all 40, arrows point to crystals). Middle panel (d–f) immunohistochemistry shows that crystals stain for kappa light chains are negative for lambda light chains and are mostly present in macrophages/histiocytes that stain for CD68. Immunofluorescence demonstrated granular staining of kappa light chains in the capillary lumens corresponding to histiocytes (not shown) (original magnification all 40). Bottom panel (g–i) electron microscopy showing crystals within macrophages as recognized by numerous cytoplasmic vacuoles (foam cells, black arrow points to macrophages containing crystals and vacuoles). Note that adjacent proximal tubule does not contain the crystals (white arrow) (g and i: low power; h: high power).
A
67-year-old Caucasian man with diabetes mellitus, hypertension, and antineutrophil cytoplasmic autoantibody– negative pauci-immune vasculitis presented with a rise in creatinine to 1.8 mg/dl from a baseline of 1.3 mg/dl, proteinuria, hematuria, new rash, ascites, and leukopenia. Serum protein electrophoresis revealed an IgG kappa monoclonal gammopathy, and cryoglobulin screen was trace positive. A renal biopsy was performed. The findings were consistent with a membranoproliferative glomerulonephritis with a crystal-storing histiocytosis secondary to an IgG kappa paraproteinemia (Figure 1). Crystal-storing histiocytosis is seen in patients with multiple myeloma and lymphoproliferative disorders; it is Kidney International (2016) 89, 507
characterized by an accumulation of light chain crystals in histiocytes that congregate in the bone marrow or other extramedullary location such as the cornea or kidney. When the kidneys are implicated, the histiocytes are often found in the interstitium. This is the first case describing localization of histiocytes in the capillary loops. In the absence of bone marrow involvement, this patient had a monoclonal gammopathy of renal significance, which may have heralded a diagnosis of multiple myeloma. He developed progressive hepatic and renal failure, myocardial infarction, and ileus. Ultimately, he opted for home hospice. 507