POEMS 증후군

소개글

plasma cell 증식성 질환입니다.

목차

Definition
Etiology
Diagnosis
Treatment
Prognosis
References

본문내용

POEMS syndrome
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes)
rare, atypical, plasma cell proliferative disorder.
POEMS syndrome been variously referred to in the literature as osteosclerotic myeloma, Crow-Fukase syndrome, PEP (plasma cell dyscrasia, endocrinopathy, polyneuropathy) syndrome, and Takatsuki syndrome. In almost all cases, the immunoglobulin light-chain type is lambda.
Definition
poorly understood
The associated plasma cell dyscrasia seems to play a crucial role, as clinical improvement follows the disappearance of the monoclonal proteins.
Elevated levels of proinflammatory cytokines, such as TNF-α, interleukins-1, 6, and vascular endothelial growth factor (VEGF) have been implicated in the multisystem manifestations.
The markedly increased serum VEGF level in POEMS is not observed in other demyelinating neuropathies. The high serum level is usually decreased dramatically following successful treatment
Etiology




Treatment
The median age at presentation is 51 years
and the overall median survival duration in the Mayo Clinic series was 165 months.
The number of features involved does not predict for survival, but the presence of fingernail clubbing or extravascular volume overload does.
Survival was adversely affected by the presence of clubbing and extravascular fluid overload.
Responding patients had superior survival times.
Infections and cardiorespiratory failure were the most common causes of death.
no patient died of progression to frank myeloma.
Prognosis

참고 자료

Hoffman: Hematology: Basic Principles and Practice, 5th ed. Chapter 87. multiple myeloma differential diagnosis.
Bradley: Neurology in Clinical Practice, 5th ed. CHAPTER 80 . Disorders of Peripheral Nerves PERIPHERAL NEUROPATHIES ASSOCIATED WITH MONOCLONAL PROTEINS.
Harrison`s Principles of Internal Medicine 17th,