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Gamma 1-heavy chain deposition disease accompanied by IgG kappa in serum, urine, and bone marrow

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Abstract

A 32-year-old Japanese woman presented with hypertension, nephrotic syndrome, microhematuria, and severe hypocomplementemia. Her serum creatinine concentration increased from 1.46 mg/dL (129.0 μmol/L) to 3.46 mg/dL (305.8 μmol/L) over 1 month. Renal biopsy revealed Congo red-negative nodular glomerulosclerosis accompanied by mesangial proliferation. There was extensive staining of immunoglobulin (Ig) G in the glomerular and tubular basement membranes and expanded mesangial regions. Staining was negative for IgA, IgM, and kappa and lambda light chains and positive for the gamma 1 IgG subclass. Staining for constant domains of the gamma heavy chains showed a deletion of the first constant domain (CH1). Electron microscopy revealed electron-dense deposits in the glomerular and tubular basement membranes and mesangium. These findings indicated gamma 1-heavy chain deposition disease (HCDD). Serum and urine immunoelectrophoresis revealed an IgG kappa monoclonal band, whereas bone marrow biopsy revealed monoclonal plasmacytosis with positive staining for kappa chains. HCDD associated with kappa light chain is extremely rare. We report the first case of HCDD with IgG kappa detected in the serum, urine, and bone marrow.

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Conflict of interest

All the authors have declared no competing interest.

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Authors and Affiliations

  1. Department of Nephrology, National Hospital Organization Kyoto Medical Center, 1-1 Fukakusa Mukaihata-cho, Fushimi-ku, Kyoto, 612-8555, Japan

    Keiichi Kaneko, Koichi Seta, Mitsuteru Koizumi, Yuko Kikuchi & Kensei Yahata

  2. Department of Nephrology, Iwate Prefectural Central Hospital, 1-4-1 Ueda, Morioka-shi, Iwate, 020-0066, Japan

    Jun Soma

  3. Department of Nephrology, Saiseikai Ibaraki Hospital, 2-1-45 Mitsukeyama, Ibaraki-shi, Osaka, 567-0035, Japan

    Takashi Kuwahara

  4. Department of Nephrology, Osaka Red Cross Hospital, 5-30 Hudegasaki, Ten-noji, Osaka, 543-8555, Japan

    Akira Sugawara

Authors
  1. Keiichi Kaneko
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  2. Koichi Seta
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  3. Jun Soma
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  4. Takashi Kuwahara
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  5. Mitsuteru Koizumi
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  6. Yuko Kikuchi
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  7. Akira Sugawara
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  8. Kensei Yahata
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Corresponding author

Correspondence to Koichi Seta.

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Kaneko, K., Seta, K., Soma, J. et al. Gamma 1-heavy chain deposition disease accompanied by IgG kappa in serum, urine, and bone marrow. CEN Case Rep 3, 44–48 (2014). https://doi.org/10.1007/s13730-013-0083-0

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  • DOI: https://doi.org/10.1007/s13730-013-0083-0

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