[Crow-Fukase syndrome (POEMS syndrome) and osseous mastocytosis secondary to Castleman's angiofollicular lymphoid hyperplasia].
In a case of Crow-Fukase (POEMS) syndrome there was a chronic, progressive, and eventually lethal polyradiculoneuropathy. In addition, adenomegaly, oedema and pleural effusions, gonadic atrophy, serum monoclonal IgA, and skin pigmentation were present. Plain x-rays and CT scan of the pelvis and lower vertebrae showed multiple poorly defined lesions. At postmortem there was no myeloma and a bone mastocytosis was found. In addition, next to T11, there was an abdominal nodule, 2 cm in diameter, with histological characteristics of Castleman's angiofollicular lymphoid hyperplasia. Immunohistochemical studies showed that plasmocytes of this lesion secreted polyclonal immunoglobulins with a high prevalence of IgA. Thus, the primary interest of this case lies in the association of bone mastocytosis with a POEMS syndrome. Also, one single localisation of Castleman angiofollicular lymphoid hyperplasia was found, assumed to be in this case the cause of the POEMS syndrome. Therefore, a minute and benign hyperplasic lesion, which was only discovered at autopsy, secreted the protein responsible for the symptoms and signs, and eventually the patient's death.
D000368:Aged; D001847:Bone Diseases / Q000150:complications* / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D005871:Castleman Disease / Q000150:complications* / Q000473:pathology; D004700:Endocrine System Diseases / Q000150:complications* / Q000473:pathology; D006801:Humans; D007136:Immunoglobulins / Q000378:metabolism*; D008297:Male; D008415:Mastocytosis / Q000150:complications*; D010525:Peripheral Nerves / Q000473:pathology; D011129:Polyradiculoneuropathy / Q000150:complications*; D013577:Syndrome
D007136:Immunoglobulins
Syndrome de Crow-Fukase ("P.O.E.M.S. syndrome") et mastocytose osseuse secondaires à une hyperplasie lymphoïde angiofolliculaire de Castleman.