Morbus Castleman – eine harmlose Differenzialdiagnose einer Lymphadenitis colli? – Eine Fallserie

Abstract

Castleman's disease is a very rare and potentially severe lymphoproliferative disorder. First sign may be cervical lymphadenitis, requiring sufficient support in diagnosis and therapy by an ENT specialist. Based on a case series the current manuscript gives an overview of the symptoms, the course of disease and the therapy options. Patients with the first diagnosis of a Castleman's disease at the ENT clinic of Ulm University during the years 2011-2015 were included. The duration of symptoms, the applied diagnostic and therapeutic algorithms were evaluated. The duration of the rather weak symptoms was inhomogenous and lasted from 14 days to 14 years. After diagnostic exstirpation a hyaline-vascular type of Castelman's disease was confirmed in all cases. One of the 5 cases proved a multicentric type with an additional axillary manifestation, the others were monocentric. In all patients the diagnostic exstirpation was sufficient for therapy without need for adjuvant medication. At the time of publication all patients are in remission for at least 18 resp. 61 months. The ENT specialist encounters Castleman's disease mostly as a long-lasting swelling of cervical lymph nodes refractory to therapy and without severe concomittant symptoms. Due to potentially unfavorable outcomes a timely diagnostic lymph node exstirpation under general anesthesia is indicated. In most cases this surgical intervention represents the sufficient therapy.

Siltuximab-Related Favorable Clinical Outcome for a Patient Suffering from Idiopathic Multicentric Castleman Disease

“…After ruling out lymphoma as an initial tentative diagnosis, we examined the possibility for CD. Because of the wide range of potential clinical outcomes, a correct diagnosis of not only CD but also the subtype is essential for treatment strategy [ 10 ]. Further, thorough differential diagnostic examination following the scheme of Fajgenbaum et al [ 4 ] could reveal the MCD-subtype HHV-8-negative iMCD-NOS in our patient and rule out lymphoma as the initial tentative diagnosis.…”