Zusammenfassung
Die intravaskuläre Variante des diffusen großzelligen B-Zell Lymphoms (IVBL) ist eine seltene Form des Non-Hodgkin-Lymphoms, die oft erst bei der Obduktion diagnostiziert wird, da sie nur mit unspezifischen und verwirrenden Symptomen einhergeht. Wir berichten über eine 63-jährige Frau mit einer IVBL, bei der ursprünglich ein myelodysplastisches Syndrom bestand. Die Symptomatik war Fieber unklarer Ätiologie, beidseitige Vergrößerung der Nebennieren und schließlich Entwicklung eines Panhypopituitarismus. Differentialdiagnostisch bestand der Verdacht auf eine lymphomatöse Infiltration oder eine Osteomyelitis der Sella. Trotz Therapie mit Antibiotika und Cortison verstarb die Patientin innerhalb von 3 Monaten. Bei der Autopsie wurde eine IVBL mit intravaskulären Ansammlungen von lymphomatösen Zellen, die vor allem in den Nebennieren und in der Hypophyse lokalisiert waren, gefunden. Die Kombination eines Panhypopituitarismus mit bilateraler Vergrößerung der Nebennieren kommt in der endokrinologischen Routine nur selten vor. Das Vorkommen eines Befalls mehrerer endokriner Organe durch eine IVBL wurde in der Literatur bisher noch nicht beschrieben.
Summary
The intravascular variant of diffuse large B-cell lymphoma (IVBL) is a rare form of non-Hodgkin lymphoma that is frequently diagnosed at autopsy because the symptoms are nonspecific or confusing. We report a case of IVBL in a woman with pre-existing myelodysplastic syndrome manifested as a fever of unknown origin, bilateral adrenal enlargement and subsequent development of panhypopituitarism. Lymphomatous infiltration or osteomyelitis of the sella was supposed. Despite antibiotic and corticosteroid therapy the patient died within three months. An intravascular variant of B-cell lymphoma with intravascular collections of lymphomatous cells predominantly localized in the adrenal and pituitary glands was found at autopsy. The association of panhypopituitarism with bilateral adrenal enlargement is uncommon in endocrinological praxis and the occurrence of combined endocrine involvement in a patient with IVBL has not been described in previous literature.
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Švajdler, M., Lazúrová, I., Bohuš, P. et al. Intravascular variant of diffuse large B-cell lymphoma with combined endocrine involvement. Wien Klin Wochenschr 118, 422–425 (2006). https://doi.org/10.1007/s00508-006-0617-y
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DOI: https://doi.org/10.1007/s00508-006-0617-y