[Plasmocyte dyscrasia with polyneuropathy, polyendocrinopathy and polyadenopathy].
A 72 years old, male complains difficulty in walking, a peripheral neuropathy of four limbs is found. Clinical examination shows a peripheral lymphadenopathy predominating in left inguinal nodes. A malignant IgA k plasmocytoma is seen on lymph node biopsy. There is no pathological plasmocytosis on bone trephine biopsy. Bone radiographies are normal. Immunoelectrophoreses finds a monoclonal IgA lambda protein of little abundance without lowering of other immunoglobulins. Insufficiency of adenohypophysis clinically suspected, is confirmed by static and dynamic tests as well as anomaly of glucose tolerance. Biopsies of skin, muscle and nerve show neither plasma cell infiltrate nor amyloid deposit. Evolution is stabilized during 12 months by an hematosarcoma type treatment which must be stopped because of bad hematologic tolerance. This interruption is followed by a fast severe evolution that leads to death in few weeks. This case as comparable with those reported by japanese authors in Far East and of which rare cases have been reported in non japanese people. It seems that the association of a non myelomatous plasma cell dyscrasia of uncertain malignancy with peripheral neuropathy, endocrinic, cutaneous and various visceral signs must be individualized. It could be considered as a particular paraneoplastic syndrome more especially as in many case the cure of the plasma cell proliferation has been followed by disparition of visceral signs.
D000368:Aged; D004700:Endocrine System Diseases / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D008206:Lymphatic Diseases / Q000209:etiology* / Q000473:pathology; D008297:Male; D010265:Paraproteinemias / Q000150:complications* / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology
D007070:Immunoglobulin A
Dyscrasie plasmocytaire avec polyneuropathie, polyendocrinopathie et polyadénopathie.