nephropathology slide seminar: case 2 european congress of pathology 30.8.2011 anne...
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Nephropathology Nephropathology Slide Seminar: Case 2Slide Seminar: Case 2European Congress of Pathology European Congress of Pathology
30.8.201130.8.2011
Anne Raisanen-Sokolowski, MD, PhDTransplantation Laboratory
Helsinki University Central HospitalHelsinki, Finland
Patient data 1Patient data 1
• 53 years old, previously healthy male• One year ago vision began to worsen,
diagnosed and treated as iritis
• Thereafter fever (ad 39°C) and malaise for 6 months.
• He suffered malfunction of intestine, edema in the lower extremities and muscle weakness, loss of appetite, weight loss
Patient data 2Patient data 2
• The patient presented with: tingling in finger tips and polyneuropathy by
ENMG hepatosplenomegalia
• Ultrasound: several focal defects in liver, suspicion of metastasis
• Biopsy: necrosis -> suspicion of Tbc -> treatment started -> laboratory findings negative
• Ascites
Hypogonadism (low testosterone)
Patient data 3Patient data 3
• The patient presented with: paraproteinemia in plasma (kappa light
chain, 3-10 g/l)• crista biopsy 10% plasma cells
hematuria (>20 erytrocytes/hpf) proteinuria (dU-Prot 0,32-0,42 g/day,
P-Alb 27 g/l, Crea 103 mmol/l) Cachexia
• Kidney biopsy taken
Immunofluoresence studyImmunofluoresence study
• Performed in frozen sections• IgG, IgA, IgM, C3, C1q, fibrinogen,
kappa and lambda were negative
Biopsy findingsBiopsy findings
• Glomeruli lobular and mesangial proliferation GBM duplication, mesangial interposition no immune deposit (IF and EM negative)
• Tubuli and vessels unremarkable
DiagnosisDiagnosis
• Membranoproliferative glomerulonephritis associated with POEMS syndrome Polyneuropathy Organomegaly (hepatosplenomegaly) Endocrinopathy (hypogonadism) M-component (kappa light chain) in plasma Skin lesions (none)
Nakamoto et al: A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome. NDT 1999: 14:2370-2378
POEMS syndrome 1POEMS syndrome 1
• POEMS syndrome is a rare paraneoplastic syndrome secondary to plasma cell dyscrasia Usually lambda light chain (95%)
• Incidence peaks in 5th and 6th decade of life, unlike multiple myeloma (7-8th)
Dispenzieri: POEMS syndrome. Blood Reviews 2007: 21, 285-299
POEMS syndrome 2POEMS syndrome 2
• Complex pathogenesis: elevation of proangiogenic and proinflammatory cytokines are hallmarks of this disorder
• Patients with POEMS have elevated VEGF levels in plasma, serum, ascites and cerebrospinal fluid
Dispenzieri: POEMS syndrome. Blood Reviews 2007: 21, 285-299
POEMS syndrome 3POEMS syndrome 3
• VEGF is the dominant driving cytokine Targets endothelial cells and induces an
increase in vascular permeability Important in angiogenesis and
osteogenesis VEGF is expressed by osteoblasts, in bone
tissue, macrophages, tumor cells (including plasma cells), megakaryocytes and platelets
IL-1 and IL-6 stimulate VEGF productionDispenzieri: POEMS syndrome. Blood Reviews 2007: 21, 285-299
Pathological features of Pathological features of POEMS nephropathyPOEMS nephropathy
• Glomerular enlargement• Cell proliferation and swelling• Mesangial loosening and mesangiolysis• Microaneurysms• Nodular-like lesions• Infiltration of plasma cells and mononuclear cells• Tubular atrophy and interstitial fibrosis• Acute tubular necrosis• No immune deposits
Nakamoto et al: A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome. NDT 1999: 14:2370-2378
Treatment
• No randomized, controlled trials in POEMS• Radiation therapy, chemotherapy,
corticosteroids, anti-VEGF mAb (Bevacizumab, Avestin) and stem cell transplantation
• Case Patient received cyclic chemotherapy-corticosteroids-Bevacizumab treatment for 11 months, remission -> autologous stem cell transplantation 3/2011 ->5/2011 in remission
Dispenzieri: POEMS syndrome. Blood Reviews 2007: 21, 285-299
Midnight Sun in Pyhatunturi, Lapland, July 14, 2011 at 23.28
If we knew what we were doing it would not be called research A. Einstein
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