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Castleman's Disease

Castleman's disease (CD), also known as angiofollicular lymph node hyperplasia, was first reported in 1954, which described a 40-year-old male with a mediastinal mass characterized histologically by lymph node hyperplasia and follicles with small, hyalinized foci. The clinical features of CD are heterogeneous, ranging from asymptomatic discrete lymphadenopathy to recurrent episodes of diffuse lymphadenopathy with severe systemic symptoms. CD was delineated as a heterogeneous cluster of disorders, with distinct unicentric CD (UCD) and multicentric CD (MCD) subtypes, and identified the essential roles of interleukin-6 (IL-6) and human herpesvirus-8 (HHV-8) in a significant proportion of cases.

So far, CD exhibits several different histological patterns. The feature of plasma cell variant (PCV) is hyperplastic germinal centers and a massive accumulation of polyclonal plasma cells in the interfollicular region. The hyaline vascular variant (HVV) is characterized by abnormal follicles with regressed germinal centers surrounded by widened mantle zones composed of small lymphocytes in an onion ring-like arrangement. Mixed forms show the presence of both hyaline vascular and plasma cell elements. The plasmablastic variant is less common and is associated with human immunodeficiency virus (HIV) infection. A subvariant of the plasma cell form, it is characterized by large plasmablasts harboring (HHV)-8 and can progress to frank plasmablastic monoclonal lymphoma.

The CD has a prevalent inflammatory background, reflected in high levels of c-Myc-dependent transcription factor, vascular endothelial growth factor (VEGF) and IL-6. This cytokine works both as a differentiation factor for macrophages as well as a hepatocyte and B cell stimulating factor. It also has a role in the modulation of immune and central nervous systems. Besides, a viral IL-6 encoded by HHV-8 activates the Janus kinase/signal transducer and activator of transcription (JAK-STAT) signaling pathway, which stimulates more cell types and potentiates the effect of human IL-6. This is translated into the increased angiogenesis and proliferation of polyclonal IgM-lambda plasma cells in the follicles of affected nodes. Autoantibodies produced by plasma cells were regarded as the effectors of the endocrinopathy; nevertheless, no circulating forms against the hormones or its receptors have been found. VEGF affects the endocrine axes by inducing an imbalance in angiogenic factors which regulate hormonal secretion.

These researches have paved the way for the introduction of novel targeted therapies into the CD armamentarium, especially monoclonal antibodies directed against IL-6 and its receptor. Creative Biogene, with an advanced national experimental platform, can offer various Castleman's disease pathway-related products including stable cell lines, viral particles and clones for your pathogenesis study and drug discovery projects.

References:
  1. El-Osta H E, Kurzrock R. Castleman\"s Disease: From Basic Mechanisms to Molecular Therapeutics. The Oncologist, 2011, 16(4):497-511.
  2. Cervantes C E, Correa R. Castleman Disease: A Rare Condition with Endocrine Manifestations. Cureus, 2015.
  3. Miles P H, et al. Update and new approaches in the treatment of Castleman disease. Journal of Blood Medicine, 2016, Volume 7:145-158.

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