Assuntos
Granuloma/etiologia , Hepatopatias/etiologia , Linfoma não Hodgkin/prevenção & controle , Síndrome POEMS/diagnóstico , Doenças Peritoneais/etiologia , Corticosteroides/uso terapêutico , Idade de Início , Alquilantes/uso terapêutico , Proteínas do Líquido Cefalorraquidiano/análise , Diagnóstico Diferencial , Humanos , Hipoparatireoidismo/etiologia , Cadeias lambda de Imunoglobulina/análise , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Osteosclerose/diagnóstico por imagem , Osteosclerose/etiologia , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/complicações , Síndrome POEMS/tratamento farmacológico , Dor/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Sarcoidose/diagnóstico , Esplenomegalia/etiologia , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
We report a case of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) with unusual clinical features. A 62-year-old woman presented a severe polyneuropathy with dysphonia and vegetative symptoms, including bradycardia and sphincterial disorders. The clinical examination showed facial hyperpigmentation, cachexia, anasarca and splenomegaly. She also presented restrictive cardiomyopathy and endocrine disturbances. Nerve conduction studies revealed a severe demyelinating sensorimotor neuropathy. Cerebrospinal fluid analysis showed an elevated protein level. We detected a biclonal gammapathy (Ig G and Ig A with lambda light chain) and lytic pelvic bone lesions. Later, she developed a severe ventilatory failure due to a bilateral phrenic nerve paralysis leading to a mechanical ventilation. Steroids followed by localized radiotherapy partially improved the respiratory status and stabilized the neuropathy. Phrenic nerve paralysis, restrictive cardiomyopathy, vegetative symptoms and cranial nerve palsy are exceptional in POEMS syndrome. Moreover, this case emphasizes the importance of radiological investigations since the discover of plasmocytoma may improve the prognosis of POEMS syndrome.
Assuntos
Cardiomiopatias/etiologia , Síndrome POEMS/patologia , Paralisia/etiologia , Nervo Frênico/patologia , Anti-Inflamatórios/uso terapêutico , Neoplasias Ósseas/patologia , Proteínas do Líquido Cefalorraquidiano/líquido cefalorraquidiano , Feminino , Humanos , Imunoglobulina A/metabolismo , Imunoglobulina M/metabolismo , Cadeias lambda de Imunoglobulina/metabolismo , Pessoa de Meia-Idade , Condução Nervosa/fisiologia , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/complicações , Ossos Pélvicos/patologia , Plasmocitoma/patologia , Respiração Artificial , Insuficiência Respiratória/etiologia , Esteroides , Tomografia Computadorizada por Raios XAssuntos
Anticorpos Monoclonais/líquido cefalorraquidiano , Proteínas do Líquido Cefalorraquidiano/análise , Imunoglobulina G/líquido cefalorraquidiano , Síndrome POEMS/diagnóstico , Doenças do Sistema Nervoso Periférico/etiologia , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Neurônios Motores/fisiologia , Neurônios Aferentes/fisiologia , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/fisiopatologia , Doenças do Sistema Nervoso Periférico/líquido cefalorraquidiano , Doenças do Sistema Nervoso Periférico/patologia , Medula Espinal/patologiaRESUMO
We report the case of a 33-year-old Japanese male who presented with thrombocytosis, lower limb edema, severe polyneuropathy with elevated cerebrospinal fluid (CSF) protein level and serum IgA lambda monoclonal component, fulfilling the manifestations of Crow-Fukase syndrome. A high level of soluble interleukin-6 receptor in the CSF was also found, which fluctuated in parallel with the clinical course. Initial treatment with double-filtration plasmapheresis (DFPP) reduced the serum IgA paraprotein level with improvement of the sensory component of the polyneuropathy and decrease of soluble interleukin-6 receptor in the CSF. The remaining clinical features waned off after steroid treatment. The possible role of interleukin-6 in the pathogenesis of the Crow-Fukase syndrome and the utility of DFPP treatment are discussed.
Assuntos
Interleucina-6/líquido cefalorraquidiano , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/terapia , Receptores de Interleucina/metabolismo , Adulto , Terapia Combinada , Glucocorticoides/uso terapêutico , Humanos , Interleucina-6/metabolismo , Masculino , Síndrome POEMS/metabolismo , PlasmafereseRESUMO
We report six patients affected by POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), a peculiar multiorgan disease frequently associated with osteosclerotic myeloma or other plasma cell disorders. Sensorimotor polyneuropathy was associated with multisystem involvement in all of the patients, with osteosclerotic myeloma in 2 cases, monoclonal gammopathy of undetermined significance in 2 cases and Castleman's disease in the final two. In all of the patients, sural nerve biopsy findings were consistent with a mixed, axonal and demyelinating neuropathy. Increased levels of Interleukin-6 were found in two cases, but the pathogenesis of the disease is far from established.
Assuntos
Síndrome POEMS/fisiopatologia , Idoso , Medula Óssea/patologia , Hiperplasia do Linfonodo Gigante/líquido cefalorraquidiano , Hiperplasia do Linfonodo Gigante/patologia , Eletrofisiologia , Hormônios/sangue , Humanos , Interleucina-6/líquido cefalorraquidiano , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Neurônios Motores/fisiologia , Condução Nervosa/fisiologia , Osteosclerose/patologia , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/patologia , Paraproteinemias/patologia , Prognóstico , Nervo Sural/patologia , Fator de Necrose Tumoral alfa/líquido cefalorraquidianoRESUMO
The pathogenesis of Crow-Fukase (POEMS) syndrome is not well known, and in some cases, a definite diagnosis is difficult to establish. Nerve fibers have been studied in about 120 peripheral nerve biopsies (PNBs), and a mixture of axonal and demyelinating lesions were found in most of them. We report five new cases of Crow-Fukase (POEMS) syndrome with ultrastructural examination of their PNBs. In every case, there were features of axonal degeneration and primary demyelination. Interestingly, uncompacted myelin lamellae (UMLs) were present in every case at a percentage of 1-7. The association of UML and Crow-Fukase (POEMS) syndrome was described 20 years ago but was only reported in a few studies and found in 31 of 41 cases. In fact, this association is very significant because apart from Crow-Fukase (POEMS) syndrome, UMLs can only be found with such a frequency in rare cases of Charcot-Marie-Tooth disease type 1B. UML was also reported in acute and chronic inflammatory demyelinating polyneuropathies but at a much lower percentage. Moreover, in our five cases, UML was frequently associated with a decrease in the number of intra-axonal filaments, and this finding raises the problem of relationships between myelin formation and neurofilaments. So far, glomeruloid hemangiomas present in the dermis of some patients are considered as the only specific criteria of Crow-Fukase (POEMS) syndrome, but we think UML can also be regarded as highly suggestive of this entity on condition that a thorough ultrastructural examination of a PNB is performed.
Assuntos
Síndrome POEMS/patologia , Nervos Periféricos/ultraestrutura , Idoso , Antígenos CD/metabolismo , Antígenos CD20/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Axônios/ultraestrutura , Biópsia/métodos , Doenças Desmielinizantes/metabolismo , Doenças Desmielinizantes/patologia , Feminino , Humanos , Imuno-Histoquímica/métodos , Técnicas In Vitro , Antígenos Comuns de Leucócito/metabolismo , Linfócitos/metabolismo , Masculino , Microscopia Eletrônica/instrumentação , Microscopia Eletrônica/métodos , Pessoa de Meia-Idade , Bainha de Mielina/metabolismo , Bainha de Mielina/ultraestrutura , Síndrome POEMS/líquido cefalorraquidiano , Síndrome POEMS/imunologia , Nervos Periféricos/imunologiaRESUMO
POEMS syndrome is a rare multisystem affliction known for its signs, from which it also takes its acronym name "peripheral neuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, and skin lesions." Our study chronicles the course of this syndrome in a young woman with Castleman's disease (angiofollicular lymph node hyperplasia). Cerebrospinal fluid (CSF) and serum interleukin-6 (IL-6) levels were abnormally elevated at various times during a 9-month period. The authors conclude that the plasma cell dyscrasia associated with the POEMS syndrome of this patient was Castleman's disease. Elevation of serum IL-6 levels might contribute to the pathogenesis of the POEMS syndrome.