Angiofollicular hyperplasia

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The original description of angiofollicular hyperplasia was of a case of localised mediastinal lymph node hyperplasia in 1954 and later described in more detail in 1956 resulting in the alternative names Castleman disease and Castleman's disease.[1] Now taken to refer, more broadly, to a more heterogenous group of lymph node hyperplasia. Also known as giant lymph node hyperplasia.

It is regarded as a lymphoproliferative disorder, although infective agents such as HHV-8 may be implicated in some cases where production of a viral homologue of interleukin 6 is thought to drive the disease.[2][3][4][5]

The disorder can be unicentric or multicentric, the latter having a stronger association with HHV-8. However, the histological classification appears to correlate better with systems and is divided into the hyaline vascular variant (90% unicentric) and the plasmacytic variant (more likely to be multicentric),[6] though a mixed cellularity variant is also described. TAFRO syndrome a form of multicentric angiofollicular hyperplasia has been recently described[7].

Treatment consists of excision in the unicentric type, but various drugs have been tried including steroids and various cytokine inhibitors, in particular IL-6 inhibitors, e.g. tocilizumab or siltuximab.[8] Rituximab and other chemotherapeutic drugs have also used.


  1. CASTLEMAN B, IVERSON L, MENENDEZ VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956 Jul-Aug; 9(4):822-30.
  2. Miles SA, Rezai AR, Salazar-González JF, Vander Meyden M, Stevens RH, Logan DM, Mitsuyasu RT, Taga T, Hirano T, Kishimoto T. AIDS Kaposi sarcoma-derived cells produce and respond to interleukin 6. Proceedings of the National Academy of Sciences of the United States of America. 1990 Jun; 87(11):4068-72.
  3. Moore PS, Boshoff C, Weiss RA, Chang Y. Molecular mimicry of human cytokine and cytokine response pathway genes by KSHV. Science (New York, N.Y.). 1996 Dec 6; 274(5293):1739-44.
  4. Nicholas J, Ruvolo VR, Burns WH, Sandford G, Wan X, Ciufo D, Hendrickson SB, Guo HG, Hayward GS, Reitz MS. Kaposi's sarcoma-associated human herpesvirus-8 encodes homologues of macrophage inflammatory protein-1 and interleukin-6. Nature medicine. 1997 Mar; 3(3):287-92.
  5. Suthaus J, Adam N, Grötzinger J, Scheller J, Rose-John S. Viral Interleukin-6: Structure, pathophysiology and strategies of neutralization. European journal of cell biology. 2011 Jun-Jul; 90(6-7):495-504.(Link to article – subscription may be required.)
  6. Menke DM, Camoriano JK, Banks PM. Angiofollicular lymph node hyperplasia: a comparison of unicentric, multicentric, hyaline vascular, and plasma cell types of disease by morphometric and clinical analysis. Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 1992 Sep; 5(5):525-30.
  7. Nagano M, Matsumoto J. A case of TAFRO syndrome with a large mediastinal mass treated with debulking surgery. Surgical case reports. 2016 Dec; 2(1):61.(Print-Electronic) (Link to article – subscription may be required.)
  8. van Rhee F, Wong RS, Munshi N, Rossi JF, Ke XY, Fosså A, Simpson D, Capra M, Liu T, Hsieh RK, Goh YT, Zhu J, Cho SG, Ren H, Cavet J, Bandekar R, Rothman M, Puchalski TA, Reddy M, van de Velde H, Vermeulen J, Casper C. Siltuximab for multicentric Castleman's disease: a randomised, double-blind, placebo-controlled trial. The lancet oncology. 2014 Aug; 15(9):966-74.(Link to article – subscription may be required.)