Castleman Disease

Czech version

Authors: P. Szturz ¹; M. Moulis ²; Z. Adam ¹; R. Šlaisová ³; R. Koukalová ⁴; Z. Řehák ⁴; P. Volfová ¹; R. Hájek ¹; J. Mayer ¹
Authors‘ workplace: Interní hematoonkologická klinika, LF MU a FN Brno ¹; Ústav patologie, LF MU a FN Brno ²; Radiologická klinika, LF MU a FN Brno ³; Oddělení nukleární medicíny, PET centrum Masarykova onkologického ústavu, Brno ⁴
Published in: Klin Onkol 2011; 24(6): 424-434
Category: Reviews

Overview

Backgrounds:
Castleman disease is a rare non-clonal lymphoproliferative disorder with the etiopathogenesis not yet thoroughly clarified. Clinically, either unicentric (localized) or multicentric (generalized) forms are recognized while, histopathologically, hyaline-vascular, plasma-cell and mixed variants of the disease exist. These types vary one from another in their clinical courses and, importantly, in methods of therapeutic management. While the unicentric hyaline-vascular form usually manifests as benign growth of a single lymph node and treatment response to complete surgical excision reaches up to 100%, the multicentric plasmocellular variant is an aggressive disease with generalized symptoms, laboratory abnormalities and the need for systemic therapy.

Aim:
The paper provides an overview of information on Castleman disease from its clinical and histopathological signs to diagnostic and therapeutic options. It deals with the role of cytokines and HHV-8 virus infection in the disease pathophysiology and is supplied with ample pictorial documentation of radiographic findings including ultrasonography, computed tomography and hybrid imaging by positron emission tomography (PET) in combination with simultaneously taken full-body computed tomography (CT) scans, the so called PET/CT. We also present photographs of histological specimens taken from an HIV and HHV-8 negative patient with the plasmocellular multicentric form.

Conclusions:
Consequent to its low incidence, Castleman disease is often misdiagnosed or diagnosed with a delay. Therefore, it is always necessary to include this rare condition in differential diagnostics of lymphadenopathy, microcytic anemia as well as B-symptoms (night sweats, fevers and weight loss). In conclusion, we also stress the significance of full-body PET/CT scanning during staging and treatment response evaluation.

Key words:
Castleman disease – lymphadenopathy – tocilizumab – interleukin – HHV-8 – ultrasonography – computed tomography – PET scan

This publication was prepared as part of the Internal Grant Agency of the Czech Republic grants NT 12215-4, the Ministry of Education, Youth and Sports of the CR grants MSM0021622434, LC06027 and the Ministry of Health of the Czech Republic’s Internal Grant Agency grants IGA MZd NT11154, NT12130, NT12215 and NS10408.

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Submitted:
4. 5. 2011

Accepted:
27. 5. 2011

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