Ewha Med J.  2013 Dec;36(Suppl):S25-S29. 10.12771/emj.2013.36.S.S25.

Clinical Remission of Renal Amyloidosis after Autologous Peripheral Blood Stem Cell Transplantation

• An SY
• Kim YH
• Kwon YE
• Kim YL
• Nam KH
• Choi H
• Kim YJ
• Park KS
• Jeong HJ
• Oh HJ
• Park JT
• Han SH
• Kang SW
• Yoo TH

• KMID: 1726731
• DOI: http://doi.org/10.12771/emj.2013.36.S.S25

Abstract

Primary amyloidosis has unfavorable prognosis, particularly with organ involvement. Here, we report a case of clinical remission of renal amyloidosis after autologous hematopoietic cell transplantation. A 51-year-old female patient visited our hospital due to generalized edema. Initial evaluation showed hyperlipidemia, hypoalbuminemia, and heavy proteinuria, which were consistent with nephrotic syndrome. However, IgM lamda type monoclonal gammopathy was detected in serum and urine electrophoresis studies. Renal biopsy showed Congo red-positive amyloid deposition in mesangial area, glomerular capillary walls, and arterioles and amyloid fibers were confirmed by electron microscopy. Immunohistochemial study of the biopsy tissue demonstrated systemic light-chain amyloidosis (AL amyloidosis). Multiple myeloma was not evident on bone marrow examination. She received autologous hematopoietic cell transplantation after high dose melphalan treatment. Complete remissions were achieved after the treatment, respectively. Our findings suggest the potential role of autologous peripheral blood stem cell transplantation in treatment of AL amyloidosis.

Keyword

AL amyloidosis; Hematopoietic cell transplantation; Melphalan

MeSH Terms

Amyloid
Amyloidosis*
Arterioles
Biopsy
Bone Marrow Examination
Capillaries
Cell Transplantation
Congo
Edema
Electrophoresis
Female
Humans
Hyperlipidemias
Hypoalbuminemia
Immunoglobulin M
Melphalan
Microscopy, Electron
Middle Aged
Multiple Myeloma
Nephrotic Syndrome
Paraproteinemias
Peripheral Blood Stem Cell Transplantation*
Plaque, Amyloid
Prognosis
Proteinuria
Transplants
Amyloid
Immunoglobulin M
Melphalan

Figure

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  Fig. 1 Initial renal biopsy findings. (A) Renal biopsy specimen shows normal glomeruli on light microscopy (H&E, ×400). (B) These materials show an apple green birefringence by Congo red staining under polarized light. (C) Immunofluorescence examinations demonstrate kappa and lambda-chain deposits in glomeruli and arterioles. (D) Electron microscopic findings reveal diffuse fusion of the foot processes, and nonbranched fibers in both subepithelial and subendothelial spaces of the glomerulus. (E) Electron microscopic findings reveal that randomly arranged fibrils are presented.

• 

  Fig. 2 A second renal biopsy 25 month after high-dose melphalan and hematopoietic cell transplantation. (A) Renal biopsy specimen shows normal glomeruli on light microscopy (H&E, ×200). (B) These materials show an apple green birefringence by Congo red staining under polarized light. (C) Immunofluorescence examinations demonstrate weakly kappa and lambda-chain deposits in glomeruli and arterioles than initial renal biopsy.

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