Pseudo-Peritoneal Carcinomatosis Presentation of a Crystal-Storing Histiocytosis With an Unmutated Monoclonal κ Light Chain. - Archive ouverte HAL Accéder directement au contenu
Article Dans Une Revue Medicine Année : 2015

Pseudo-Peritoneal Carcinomatosis Presentation of a Crystal-Storing Histiocytosis With an Unmutated Monoclonal κ Light Chain.

A Aline-Fardin
  • Fonction : Auteur
B Fabiani
  • Fonction : Auteur
D Buob
  • Fonction : Auteur
S Brahimi
  • Fonction : Auteur
Mc Verpont
  • Fonction : Auteur
M Mothy
  • Fonction : Auteur
P Ronco
  • Fonction : Auteur
Jj Boffa
  • Fonction : Auteur
P Aucouturier
  • Fonction : Auteur
L. Garderet
  • Fonction : Auteur

Résumé

Crystal-storing histiocytosis (CSH) is a rare complication of monoclonal gammopathies caused by accumulation of crystalline material inside macrophages, and it may result in a variety of clinical manifestations depending on the involved organs. Although immunoglobulin κ light chains (LCs) seem to be the most frequent pathogenic component, very few molecular data are currently available.A 69-year-old man presented with a very poor performance status. Remarkable features were mesenteric lymph node enlargement and proteinuria, including a monoclonal κ LC. Light and electron microscopy studies revealed the presence of crystals within macrophages in the lymph nodes, bone marrow, and kidney, leading to the diagnosis of CSH. The pathogenic κ LC variable domain sequence was identical to the germline Vk3-2001/Jk201 gene segments, without any somatic mutation, suggesting an extra-follicular B cell proliferation.The patient was successfully treated with 4 cycles of bortezomib and dexamethasone. After a 12-month follow-up, he remains in hematological and renal remission.CSH may present as pseudo-peritoneal carcinomatosis and relate to a monoclonal κ LC encoded by an unmutated gene. Bortezomib-based therapy proved efficacious in this case.

Domaines

Immunologie
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Dates et versions

hal-01212537 , version 1 (06-10-2015)

Identifiants

  • HAL Id : hal-01212537 , version 1
  • PUBMED : 26266355

Citer

A Aline-Fardin, Sébastien Bender, B Fabiani, D Buob, S Brahimi, et al.. Pseudo-Peritoneal Carcinomatosis Presentation of a Crystal-Storing Histiocytosis With an Unmutated Monoclonal κ Light Chain.. Medicine, 2015. ⟨hal-01212537⟩
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