loading page

Multicentric Castleman Disease in a DOCK8 Deficient Patient with Orf Virus Infection
  • +8
  • Elif Tugce Aydin Goker,
  • Deniz Cagdas Ayvaz,
  • Inci Bajin,
  • Musa Kukul,
  • Elif Soyak Aytekin,
  • Diclehan Orhan,
  • Alpaslan Alp,
  • Serdar Uzar,
  • Fahriye Sarac,
  • Ates Kara,
  • Tezer Kutluk
Elif Tugce Aydin Goker
Hacettepe University
Author Profile
Deniz Cagdas Ayvaz
Hacettepe University
Author Profile
Inci Bajin
Hacettepe University
Author Profile
Musa Kukul
Hacettepe University
Author Profile
Elif Soyak Aytekin
Hacettepe University
Author Profile
Diclehan Orhan
Hacettepe University
Author Profile
Alpaslan Alp
Hacettepe University, Faculty of Medicine
Author Profile
Serdar Uzar
Istanbul Pendik Veterinary Control Institute
Author Profile
Fahriye Sarac
Istanbul Pendik Veterinary Control Institute
Author Profile
Ates Kara
Hacettepe University
Author Profile
Tezer Kutluk
Hacettepe University
Author Profile

Abstract

Castleman disease is a rare, heterogeneous disorder that driven by proinflammatory responses. Human herpes virus-8 has a major role in pathogenesis of multicentric Castleman disease. There is also a subgroup of cases, human herpes virus-8 negative, idiopathic multicentric Castleman disease. The role of primary immunodeficiencies in idiopathic Castleman disease are poorly described. DOCK8 deficiency is a combined primary immunodeficiency. It has a broad clinic spectrum including atopy, autoimmunity and cancer. We present a 10-year-old, DOCK8 deficient patient. He had giant lobular capillary hemangiomas on his neck, iliac and gluteal regions and multiple lymphadenopathies. Abdominal lymph node pathology revealed hyaline vascular type Castleman disease and human herpes virus-8 staining was negative. His lesions were shown to be infected with orf virus. Our case is the first case to relate idiopathic multicentric Castleman disease and DOCK8 deficiency; also, very unusual presentation of orf virus infection in humans.

Peer review status:ACCEPTED

21 Jun 2021Reviewer(s) Assigned
02 Aug 2021Review(s) Completed, Editorial Evaluation Pending
04 Aug 2021Editorial Decision: Revise Minor
30 Aug 20211st Revision Received
31 Aug 2021Review(s) Completed, Editorial Evaluation Pending
03 Sep 2021Editorial Decision: Revise Minor
05 Sep 20212nd Revision Received
06 Sep 2021Review(s) Completed, Editorial Evaluation Pending
09 Sep 2021Editorial Decision: Accept