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Journal of Peking University (Health Sciences) ›› 2020, Vol. 52 ›› Issue (6): 1162-1165. doi: 10.19723/j.issn.1671-167X.2020.06.031

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  • Received:2019-09-24 Online:2020-12-18 Published:2020-12-13
  • Supported by:
    National Natural Science Foundation of China(81470956)

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  • R597+.2

Table 1

Demographics and clinical characteristics of patients with renal AH/AHL amyloidosis"

Items Case 1 Case 2 Case 3 Case 4
Gender Male Male Male Female
Age/years 43 55 57 54
Blood pressure/mmHg 130/100 110/65 145/80 160/100
Peripheral edema + + + +
Weight - ↓ ↓ ↓
Urine protein/(g/24 h) 14.5 8.3 4.6 12
blood albumin/(g/L) 21 22.3 23.9 16.9
Creatinine/(μmol/L) 165 77.4 197 159.5
eGFR/[mL/(min·1.73 m2)] 41.9 96.1 32.5 31.2
CKD stage (3b) 1 3b 3b
Mirror hematuria 10-20 15-20 50-60 20
Wollen lymphabdomen superfi-
cial		 - - - + - + - -
Other organs
Heart - - - -
Liver - - - +
Spleen - - + +
Tongue - - - -
Nervous system - - - -
Ventricular septum - - - -

Table 2

Hemotological characteristics and diagnosis of patients with renal AH/AHL amyloidosis"

Case IFE κ/λ Bone marrow
plasmacyte/%		 Hematological
diagnosis		 Amyloidosis
Blood Urinary
1 IgGκ IgGκ 44 26 MM AH
2 IgMλ λ 0.21 5.5 WM AHL
3 IgGκ IgGκ 6.8 2.5 MGRS AHL
4 IgGκ κ 13.4 2 MGRS AHL

Figure 1

Fluorescence image of renal AH/AHL amyloidosis (Case 3, immunofluorescence ×200) Granular and smudgy deposits were showed in mesangial area, capillary loop, renal interstitium and arteriolar wall. A, IgG(+++); B, IgG1(+); C, κ light chain (++); D, λ light chain (-); E, C3 (++); F, C1q (+++); G, IgM (+)."

Table 3

Fluorescence results in renal pathology of patients with renal AH/AHL amyloidosis case immunofluorescence"

Case Immunofluorescence
1 IgG++ IgG2++ IgM+ C3+
2 IgM+++ λ++
3 IgG+++ IgG1+ κ++ IgM+ C3++ C1q+++ FRA+
4 IgG+ IgG1+ κ++ IgM+ C3+

Figure 2

Light and electron microscopy of patients with renal AH/AHL amyloidosis A, Congo red staining of renal interstitium and renal vessels was positive (Congo red staining × 200); B, amyloid in figure 2A showed apple-green birefringence under polarized light (Case 4); C, The glomerular mesangial area was obviously widened and nodular sclerosis occurred (Case 3, PASM+masson ×200); D, the glomerular mesangial area was obviously widened and nodular sclerosis occurred (Case 1, PAS ×200); E, segmental glomerular basement membrane showed feathery spikes (Case 2, PASM+masson ×400), F, fine fibrils (8-12 nm) with disordered arrangement and no branches were found in glomerular basement membrane and under podocytes.(Case 2, electron microscope ×40 000); G, the glomerular mesangial area was obviously widened and nodular sclerosis occurred (Case 3, HE ×400)."

Table 4

Pathological score of renal biopsy in patients with renal AH/AHL amyloidosis"

Case Deposition of amyloi Total score Glomerulus sclerosis Tubule atroph Mesenchyma fibrosis Total score
Glomerulus Interstic Vessel
1 3 3 2 8 0/31 2 2 4
2 1 0 1 2 0/18 1 1 2
3 3 1 3 7 9/56 2 2 5
4 3 3 3 9 0/17 2 2 4
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