A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
  • Luís Landeiro
    Internal Medicine Department, Hospital da Luz, Lisboa, Portugal
  • Ana Carolina Freitas
    Hematology Department, Instituto Português de Oncologia Francisco Gentil, Lisboa, Portugal
  • Margarida Proença
    Internal Medicine Department, Hospital da Luz, Lisboa, Portugal
  • José Cabeçadas
    Anatomic Pathology Department, Instituto Português de Oncologia Francisco Gentil, Lisboa, Portugal
  • Albertina Nunes
    Hematology Department, Instituto Português de Oncologia Francisco Gentil, Lisboa, Portugal
  • Alexandra Bayão Horta
    Internal Medicine Department, Hospital da Luz, Lisboa, Portugal

Keywords

Multicentric Castleman, nephrotic syndrome, polyneuropathy

Abstract

Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients its etiology is still unknown, hence the designation of idiopathic multicentric Castleman disease (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnosis excluded. Peripheral neuropathy and nephropathy are relatively common findings in cases associated with POEMS syndrome, but very rarely reported in iMCD.

We present the case of a 64-year-old man with iMCD (HIV and HHV-8 negative) with nephrotic syndrome and severe motor polyneuropathy. Alternative diagnoses were excluded. The patient was treated with intravenous glucocorticoid followed by rituximab. Complete clinical and laboratorial remission was achieved and maintained at 2-year follow-up.

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References

  • Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Haematol 2016;3(4):e163–e175.
  • Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 2017;129:1646–1657.
  • Naddaf E, DIspenzieri A, Mandrekar J, Mauermann ML. Clinical spectrum of Castleman disease-associated neuropathy. Neurology 2016;87(23):2457–2462.
  • El Karoui K, Vuiblet V, Dion D, Izzedine H, Guitard J, Frimat L, et al. Renal involvement in Castleman disease. Nephrol Dial Transplant 2011;26(2):599–609.
  • Chan KL, Lade S, Prince HM, Harrison SJ. Update and new approaches in the treatment of Castleman disease. J Blood Med 2016;7:145–158.
  • Dispenzieri A. POEMS syndrome: 2017 update on diagnosis, risk stratification, and management. Am J Hematol 2017;92(8):814–829.
  • Sugimoto T, Ito J, Takeda N, Gasyu I, Okazaki T, Sakaguchi M, et al. A case of Castleman’s disease complicated with nephrotic syndrome due to glomerulopathy mimicking membranoproliferative glomerulonephritis. Am J Med Sci 2008;335(6):495–498.
  • Tazi I, Rachid M, Quessar A, Benchekroun S. A rare association of Castleman’s disease and nephrotic syndrome. Saudi J Kidney Dis Transpl 2011;22(1):116–119.
  • Mauermann ML. The peripheral neuropathies of POEMS syndrome and Castleman disease. Hematol Oncol Clin North Am 2018;32(1):153–163.
  • Ide M, Kawachi Y, Izumi Y, Kasagi K, Ogino T. Long-term remission in HIV-negative patients with multicentric Castleman’s disease using rituximab. Eur J Haematol 2006;76:119–123.

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    Published: 2021-10-20
    Issue: 2021: Vol 8 No 10 (view)

    How to cite:
    1.
    Landeiro L, Freitas AC, Proença M, Cabeçadas J, Nunes A, Bayão Horta A. A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient. EJCRIM 2021;8 doi:10.12890/2021_002831.

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