[Primary pseudotumoral pulmonary amyloidosis with Pierre-Marie syndrome].
We report a case of pseudotumoral, nodular, parenchymatous, primary pulmonary amyloidosis in a male patient under 50 years of age. The amyloidosis spread step by step, very progressively, over 12 years before Marie's disease developed. The pre-operative diagnosis was made by electron microscopy of an intrabronchial growth biopsy. Right pleuro-pneumonectomy was performed, resulting in regression of Marie's disease within 3 months. There was partial factor X deficiency. Four years after surgery, nodules have appeared in the left upper lobe, suggesting a recurrence.
Natali
F
F
Service de Pneumologie, Hôpital d'instruction des Armées Percy, Clamart.
Levagueresse
R
R
Herning
R
R
Giudice
A
A
L'Her
P
P
Allard
P
P
D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D003937:Diagnosis, Differential; D005171:Factor X Deficiency / Q000150:complications; D005500:Follow-Up Studies; D006801:Humans; D008171:Lung Diseases / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010005:Osteoarthropathy, Secondary Hypertrophic / Q000000981:diagnostic imaging / Q000209:etiology*; D011013:Pneumonectomy; D013577:Syndrome; D014057:Tomography, X-Ray Computed
Amyloïdose pulmonaire primitive, pseudo-tumorale, avec syndrome de Pierre-Marie.