Massive generalized crystal-storing histiocytosis associated with extracellular crystalline nephropathy: clinical, immunohistochemical, and ultrastructural studies of a unique disorder and review of the literature

  • Fransico Galeano-ValleEmail author
  • F. J. Díaz-Crespo
  • R. Melero-Martín
  • J. E. Apaza-Chávez
  • J. Del-Toro-Cervera
  • P. Demelo-Rodríguez
Case Report


Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of nonneoplastic histiocytes containing intracytoplasmic crystallized immunoglobulins. In most cases, there is an associated underlying lymphoplasmacytic neoplasm expressing Ig kappa light chain. About 131 cases of CSH have been identified. There is a localized and a generalized form of CSH and it can involve several sites including bone marrow, lungs, lymph nodes, liver, spleen, gastrointestinal tract, and kidney. Generalized CSH is less frequent and involves multiple organs and tends to have a worst prognosis than localized CSH. Around 20 cases of renal involvement in CSH have been reported so far. Paraprotein-induced crystalline nephropathy can be divided into two categories based on whether the crystals in the kidney are intracellular (including light chain proximal tubulopathy with crystals and CSH) or extracellular (including the crystalline variant of myeloma cast nephropathy and crystalglobulin-induced nephropathy). The former tends to present with slowly worsening kidney dysfunction and generally has a good prognosis, whereas the latter usually presents with rapidly progressive renal failure and is associated with poor renal outcome. We present a case of generalized CSH associated with extracellular crystalline nephropathy with a fulminant and fatal clinical course. Kappa light-chain crystals were found exclusively extracellularly within the tubular lumen, not within the tubular epithelial cells nor the histiocytes, and the massive presence of those precipitates led to the acute renal failure. Consequently, we review the renal involvement in CSH in the literature and discuss the unique mechanism of renal injury in this case.


Crystal-storing histiocytosis Crystalline nephropathy Histiocytes Kappa light chains Kidney 



We are grateful to the patient and his family because they allowed us to perform the post-mortem study.

Compliance with ethical standards

Conflict of interest

The authors have declared that no conflict of interest exists.

Informed consent

Written informed consent was obtained from the patient’s family for publication of this study.

Ethical standards

This article does not contain any studies with human participants or animals performed by any of the authors.


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Copyright information

© Japanese Society of Nephrology 2019

Authors and Affiliations

  1. 1.Deparment of Internal MedicineHospital General Universitario Gregorio MarañónMadridSpain
  2. 2.Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM)MadridSpain
  3. 3.Department of PathologyHospital General Universitario Gregorio MarañónMadridSpain
  4. 4.Department of NephrologyHospital General Universitario Gregorio MarañónMadridSpain

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