Abnormal regulation of soluble and anchored IL-6 receptor in monocytes from patients with essential thrombocythemia

Exp Hematol. 2010 Oct;38(10):868-876.e1. doi: 10.1016/j.exphem.2010.06.007. Epub 2010 Jun 23.

Abstract

Objective: In a previous study, we found increased plasma soluble receptor for interleukin-6 (sIL-6R) levels in patients with essential thrombocythemia (ET) that could promote megakaryopoiesis through IL-6 binding and further interaction with the signal transducer gp130. Here we have searched for the cell source of sIL-6R within mononuclear cells in these patients and the underlying abnormalities involved in its overproduction.

Materials and methods: Thirty patients with the diagnosis of ET were studied. sIL-6R levels were measured by enzyme-linked immunosorbent assay technique in the supernatants of peripheral monocyte and lymphocyte cultures. Expression of membrane-anchored IL-6R was determined by flow cytometry. In order to study the mechanism of sIL-6R production, tumor necrosis factor-α protease inhibitor was added to specifically block IL-6R shedding. Gene expression of sIL-6R levels were evaluated by reverse transcription polymerase chain reaction.

Results: Monocytes were the main source of sIL-6R. Besides, in ET patients, monocyte sIL-6R release was higher than that of controls (p = 0.0014). Lymphocytes enhanced monocyte sIL-6R production by cell-mediated contact in normal controls, but this cooperation could not be seen in patients. Membrane expression of IL-6R was increased after monocyte adhesion in ET. sIL-6R synthesis was upregulated in most patients, while messenger RNA was normal.

Conclusions: Our results indicate that ET monocytes are responsible for sIL-6R overproduction within mononuclear cells through synthesis upregulation. In addition, the lack of cooperation of lymphocytes in monocyte sIL-6R production in ET could be due to a monocyte abnormality. The agonistic effect of sIL-6R on IL-6 action could contribute to the exacerbated megakaryocytic growth in ET.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cell Membrane / metabolism
  • Cells, Cultured
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Flow Cytometry
  • Gene Expression Regulation
  • Humans
  • Janus Kinase 2 / genetics
  • Lymphocytes / metabolism*
  • Male
  • Middle Aged
  • Monocytes / metabolism*
  • Mutation
  • Receptors, Interleukin-6 / blood*
  • Receptors, Interleukin-6 / genetics
  • Receptors, Interleukin-6 / metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Solubility
  • Thrombocythemia, Essential / blood*
  • Thrombocythemia, Essential / genetics
  • Thrombocythemia, Essential / metabolism
  • Young Adult

Substances

  • IL6R protein, human
  • Receptors, Interleukin-6
  • JAK2 protein, human
  • Janus Kinase 2