The clinical spectrum of Castleman's disease

Abstract

Castleman's disease (CD) is a rare, poorly understood lymphoproliferative disease. The spectrum of symptoms and course of disease are broad, but there is no large study describing the natural history of this disease. Basic clinic and laboratory data from the records of 113 patients with CD evaluated at the Mayo Clinic and University of Nebraska were abstracted. The impact of these variables on overall survival (OS) from time of diagnosis was evaluated. Sixty patients had multicentric disease. Of the patients with multicentric CD, 32% had criteria sufficient for a diagnosis of POEMS syndrome. For all patients, 2, 5, and 10‐year OS was 92%, 76%, 59%, respectively. Most of the factors identified as risk factors for death on univariate analysis cosegregated with diagnostic criteria for POEMS syndrome, which supported the concept of four categories of CD, which are (along with their 5‐year OS): (1) unicentric CD (91%); (2) multicentric CD associated with the osteosclerotic variant of POEMS syndrome (90%); (3); multicentric CD without POEMS syndrome (65%); and (4) multicentric CD with POEMS syndrome without osteosclerotic lesions (27%). We have demonstrated that CD represents a spectrum of disease that can be differentiated by simple prognostic factors that provide a framework for further study. Am. J. Hematol. 2012. © 2012 Wiley Periodicals, Inc.

Clinical and pathological characteristics of HIV- and HHV-8–negative Castleman disease

“…Previous series have reported 5-year survival rates ranging from 55% to 77% for HIV-negative MCD. [38][39][40] Only 2 of 31 iMCD patients in our cohort died within the median 6.6-year follow-up period. This could be explained by one of the following: (1) iMCD cases that cooccurred with malignancy were excluded; (2) MD Anderson is a referral center, so acute patients that may die on presentation would not be as well represented, as would be the case in a different setting; (3) the 18% of patients with iMCD patients who were asymptomatic would have been excluded from other series; and (4) newer treatment options may be improving outcomes.…”

Single-Region Castleman's Disease: Case report

“…Hyalin vaskü-ler ve plazma hücreli tipler arasındaki histopatolojik farklılıklar nedeniyle hastalığın değişik şekillerinin gelişim mekanizmalarının farklı olabileceğini düşü-nen araştırmacılar vardır (10) . Çok-bölgeli CH'da insan herpes virüsü 8 ve insan immun yetersizlik virüsü pozitif olguların varlığı etyopatogenezde aşırı viral uyarımı akla getirmiş, hayvan modellerinde ve klinik çalışmalarda interlökin-6 artışının CH ile yakın iliş-kisi gösterilmiştir (11,12) . Akut yangısal yanıtın klinik göstergeleriyle birlikte seyrettiği için plazma hücreli tipin gerçekte hastalığın daha erken ve aktif bir döne-mini, hyalin vasküler tipin ise daha geç bir dönemini yansıttığı da öne sürülmektedir (1) .…”

“…Olguların %90'dan fazlasının tam cerrahi çıkarmayla tedavi edildiği, çok-bölgeli CH'lilerde ise kemoterapi ve/veya radyoterapi gerekebileceği bildirilmiştir (12) . Radyoterapi ve kemoterapinin tam iyileşme sağlamasalar da tedaviye yardımcı oldukları kabul edilmektedir (4,6,15) .…”

Multicentric Castleman's Disease with Mycobacterium cosmeticum Infection

“…The clinical spectrum ranges from waxing and waning lymphadenopathy with B-symptoms to more severe cases involving intense inflammation, hepatosplenomegaly, vascular leak syndrome with anasarca, pleural effusions, and ascites, organ failure, and even death [4,5].…”