[Lymphomatoid granulomatosis--a short description of an unusual case of the disease].
Lymphomatoid granulomatosis (LYG) is a rare disease with an unknown pathogenesis. It is considered as a B-cell disorder with an uncertain malignant potential. The disease is classified as an angiocentric and angiodestructive lymphoproliferative disorder. EBV-association (LMP1) and the detection of the surface antigens CD20 and CD30 are the characteristic pathological findings. The lung, the nervous system, the kidneys and the liver are affected most frequently. In the present report the case of a 79-year-old man is described, who showed a cavity in the upper right lobe which was opened out into the trachea. The initial CXR and CT scan demonstrated a large nodular air space opacity (reversed halo sign) on the upper right area and a smaller one on the left side. In addition to CT and bronchoscopy, a surgical biopsy was performed to confirm the diagnosis of lymphomatoid granulomatosis. Treatment consisted in chemotherapy with rituximab, cyclophosphamide, vincristine and prednisolone (R-CVP). Three months after confirmation of diagnosis the patient died of an abscess-forming pneumonia.
Bräunlich
J
J
Universität Leipzig, Abteilung Pneumologie.
Seyfarth
H-J
HJ
Gessner
C
C
Gradistanac
T
T
Wirtz
H
H
D000368:Aged; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis* / Q000628:therapy*; D008230:Lymphomatoid Granulomatosis / Q000175:diagnosis* / Q000628:therapy*; D008297:Male; D035583:Rare Diseases / Q000175:diagnosis / Q000628:therapy