POEMS syndrome pathophysiology: Difference between revisions
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While the main features of the disease have been described, the exact mechanism of the disease has remained elusive. The paraprotein alone is insufficient to explain the multi-organ features, and various [[cytokine]]s produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically [[IL1B|interleukin 1β]], [[interleukin 6]] and [[TNFα]]. There also seems to be a role of [[vascular endothelial growth factor]], given the [[angiogenesis|angiogenetic]] changes in many tissues. | While the main features of the disease have been described, the exact mechanism of the disease has remained elusive. The paraprotein alone is insufficient to explain the multi-organ features, and various [[cytokine]]s produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically [[IL1B|interleukin 1β]], [[interleukin 6]] and [[TNFα]]. There also seems to be a role of [[vascular endothelial growth factor]], given the [[angiogenesis|angiogenetic]] changes in many tissues. | ||
The | The polyneuropathy is usually symmetrical and distal. There is a combination of [[demyelination]] and [[axonal]] damage, and [[sensory neuron|sensory]], [[motor neuron|motor]] and [[autonomic nervous system|autonomic]] neurones are all affected. The typical symptoms are therefore of numbness, tingling and weakness in the feet, later affecting the legs and hands. Pain is unusual, but the weakness may eventually become severe and disabling. The autonomic neuropathy may cause excessive sweating and [[erectile dysfunction]]; hormonal changes may also contribute to the latter. It is usually the symptoms of neuropathy which prompt a person with POEMS syndrome to seek medical attention. | ||
[[Myeloma]] is the most common | [[Myeloma]] is the most common plasma-cell proliferative disorder associated with POEMS syndrome, present in more than half of all cases. Most myelomas associated with POEMS syndrome are [[osteosclerotic]], in contrast to the [[osteolytic]] bone lesions in most other myelomas. Other blood disorders occur in a significant minority of cases, including [[Castleman's disease]], [[plasmacytoma]] and [[MGUS]]. | ||
===Associated Disorders=== | ===Associated Disorders=== | ||
Revision as of 20:42, 19 September 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
While the main features of the disease have been described, the exact mechanism of the disease has remained elusive. The paraprotein alone is insufficient to explain the multi-organ features, and various cytokines produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically interleukin 1β, interleukin 6 and TNFα. There also seems to be a role of vascular endothelial growth factor, given the angiogenetic changes in many tissues.
The polyneuropathy is usually symmetrical and distal. There is a combination of demyelination and axonal damage, and sensory, motor and autonomic neurones are all affected. The typical symptoms are therefore of numbness, tingling and weakness in the feet, later affecting the legs and hands. Pain is unusual, but the weakness may eventually become severe and disabling. The autonomic neuropathy may cause excessive sweating and erectile dysfunction; hormonal changes may also contribute to the latter. It is usually the symptoms of neuropathy which prompt a person with POEMS syndrome to seek medical attention.
Myeloma is the most common plasma-cell proliferative disorder associated with POEMS syndrome, present in more than half of all cases. Most myelomas associated with POEMS syndrome are osteosclerotic, in contrast to the osteolytic bone lesions in most other myelomas. Other blood disorders occur in a significant minority of cases, including Castleman's disease, plasmacytoma and MGUS.
Associated Disorders
Possible associations included pulmonary hypertension, restrictive lung disease, a thrombotic diathesis, arthralgia, cardiomyopathy (systolic dysfunction), fever, low vitamin B12 levels and diarrhea.