Choroba Castlemana – opis przypadku

Abstract

The Castleman disease was described for the first time in 1956 by Castleman. It is a rare disease of the lymphatic tissue, manifesting in excessive proliferation of lymphocites B and plasmatic cells in lymph nodes. The etiopathogenesis if the disease still is unknown. The paper presents a case of the Castlemana disease (hyaline vascular type) in 20-year-old man with unilateral enlargement of neck lymph nodes. The case exemplifies the difficulties met in diagnostics and treatment of the Castleman disease.

Castleman’s disease – a case report

“…Only in the case reported by Kycler et al, but referring to the generalized form of Castleman's disease, were there described anaemia, lymphopenia and coagulation disorders, such as elevated concentration of D-dimers and fibrinogen, being the results of systemic disorders accompanying the disease [22]. Moreover, anaemia is mentioned as a frequent disorder in plasma-cell type of Castleman's disease, which was also confirmed in the case of our patient [11].…”

“…There are two clinical forms, multicentric Castleman's disease (MCD) and localized unicentric Castleman's disease (UCD). Localized Castleman's disease includes plasmacell type (20%), occurring mostly in children and young people in the 2nd-3rd decade of life, often accompanied by changes in blood morphology (anaemia), and hyaline-vascular type (80%), usually benign, typical for patients in the fourth decade of life [11]. Localized form usually concerns one lymph node or a group of nodes with similar localization forming a so-called Castleman's tumour, most often in the mediastinum, retroperitoneal space or axilla.…”

“…Multicentric form includes hyaline-vascular type occurring in older patients in the 6th-7th decade of life, usually accompanied by disseminated lymphadenopathy, hepatosplenomegaly, thrombocytopenia, anaemia, hypoalbuminaemia, hypergammaglobulinaemia, fever, night sweat, weakness, weight loss, increased level of transaminases and inflammation markers, ascites or pleural exudate [9][10][11]. Multicentric hyaline-vascular and transitional types are very infrequent.…”