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[A case of Crow-Fukase syndrome associated with membranoproliferative glomerulonephritis]
S Takeshita1, M Yamakado, M Nagano
1Department of Internal Medicine, Mitsui Memorial Hospital, Tokyo, Japan.
Abstract:
Crow-Fukase syndrome is a rare multiorgan disorder. Although renal disorders, such as proteinuria, and renal impairment, have been observed in half the cases of this syndrome, there have been few reports describing the renal lesions. We report here a case of this syndrome associated with membranoproliferative glomerulonephritis. A 43-year-old woman was referred to our hospital because of hyperglycemia. She had also been suffering from hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy and endocrine dysfunction, including diabetes mellitus and amenorrhea. Serum electrophoresis showed M protein and immunoelectrophoresis revealed IgA (lambda). Bone marrow aspiration showed a slight increase in the number of plasma cells. Urine protein was 30 mg/dl, BUN was 17 mg/dl and creatinine 0.8 mg/dl. Light microscopic examinations showed enlargement of glomeruli with proliferation of mesangial cells and matrix, a lobular pattern of the glomeruli and thickening of the glomerular basement membrane and associated double contour. Electron microscopic examinations showed thickened capillary walls, associated mesangial interposition and subendothelial dense deposits. Moreover, fine granular deposits of IgM, C3, and fibrinogen along the basement membrane were observed on immunofluorescent studies.
Insights
Crow-Fukase syndrome, a rare disorder, can cause kidney problems. This case highlights membranoproliferative glomerulonephritis as a specific renal lesion in this condition.
Area of Science:
- Nephrology
- Hematology
- Endocrinology
Background:
- Crow-Fukase syndrome (POEMS syndrome) is a rare multisystem disorder with variable clinical manifestations.
- Renal involvement, including proteinuria and impairment, occurs in approximately 50% of patients, but detailed renal pathology is infrequently reported.
Observation:
- A 43-year-old woman presented with hyperglycemia, hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy, and endocrine dysfunction (diabetes mellitus, amenorrhea).
- Laboratory findings included M protein (IgA lambda) on serum electrophoresis and increased plasma cells in bone marrow.
- Initial renal function tests showed mild proteinuria (30 mg/dl), BUN 17 mg/dl, and creatinine 0.8 mg/dl.
Findings:
- Light microscopy revealed enlarged glomeruli with mesangial cell/matrix proliferation, lobular pattern, and thickened glomerular basement membranes with double contours.
- Electron microscopy demonstrated thickened capillary walls, mesangial interposition, and subendothelial electron-dense deposits.
- Immunofluorescence studies showed granular deposits of IgM, C3, and fibrinogen along the glomerular basement membrane.
Implications:
- This case illustrates a specific renal pathology, membranoproliferative glomerulonephritis, associated with Crow-Fukase syndrome.
- Understanding these renal lesions is crucial for comprehensive patient management and prognosis in Crow-Fukase syndrome.
- Further research into the pathogenesis of renal involvement in POEMS syndrome is warranted.