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POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell neoplasm. Hypogonadism is the most common endocrine abnormality in POEMS syndrome. There is no data about improvement of hypogonadism and sexual dysfunction after appropriate treatment of POEMS syndrome so far. In this single-center prospective study, the efficacy of low-dose lenalidomide and dexamethasone combination therapy in the improvement of sexual and gonadal function in POEMS syndrome was evaluated.
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-spike, skin changes) syndrome is a rare paraneoplastic syndrome characterized with multisystem involvement and markedly increased vascular endothelial growth factor (VEGF) levels . VEGF is a growth factor for endothelial cells that induces vascular permeability, which is important in angiogenesis and often decreases with successful treatment .
Lenalidomide is a derivative of thalidomide, with powerful activity against malignant plasma cells as well as acting to decrease the levels of proinflammatory and proangiogenic cytokines. Recently, it has been showed that lenalidomide might play a promising role in the treatment of POEMS syndrome [4, 5]. In our previous work, the efficacy and safety of low dose lenalidomide plus dexamethasone had been demonstrated in patients with relapsed or refractory POEMS syndrome . Here we conducted a pilot study to assess the efficacy of lenalidomide plus dexamethasone in the improvement of gonadal and sexual function of POMES syndrome in our center.
Forty-one consecutive patients with newly diagnosed POEMS syndrome (male 28, female 13) were treated with combination of low-dose lenalidomide and dexamethasone at Peking Union Medical College Hospital from April 2014 to November 2015. All patients met the diagnostic criteria defined by Dispenzieri, with two mandatory criteria (polyneuropathy and monoclonoal plasma cell proliferating disorder), at least one major criterion (sclerotic bone lesion, Castleman disease or VEGF elevation) and one minor criterion (organomegaly, edema, endocrinology, skin change, papillary edema or thrombocytosis) . Excluded from the study were patients with a history of medication use, such as antidepressants, psychotropic drugs, beta-blockers and spirolactone, which can interfere with sexual function. No hormone replacement therapy was employed in patients with hypogonadism.
The present study explored the under-investigated area of sexual quality of life in patients with POEMS syndrome. Our results demonstrated that low-dose lenalidomide plus dexamethasone combination therapy is successful in improving sexual function in patients with POEMS syndrome.
Hypogonadism is the most common endocrine disorders in POEMS syndrome. Because of the low incidence, there were only case reports about hypogonadism in POEMS so far [11, 12]. In the Mayo series, 79 % (26/38) male patients had hypogonadism and 61 % (23/39) reported erectile dysfunction . In our reported series, hypogonadism was seen in 52.2 % (26/46) male patients. Impotence (89 %) and gynecomastia (12 %) were common findings in male patients . Sasano et al. reported the first case that showed an improvement in gonadotropin secretion after corticosteroid therapy . After three months of corticosteroids treatment, the 49-year-old male patient showed an improvement in danadotropin secretion but no considerable change in the secretion of the other hormones. Recently, Chu et al. reported their experience of a patient with POEMS syndrome with long-term therapy with lenalidomide. The total testosterone levels had increased from 55 ng/dl to 624 ng/dl after one year .
The pathogenesis of endocrinopathy is not well understood. Circulating antibodies against hormones or specific hormone receptors have not been found. No specific structural changes had been found in endocrine glands . VEGF is a growth factor for endothelial cells that induces an increase in vascular permeability, is important in angiogenesis, and often decreases with successful therapy. There were accumulating evidences suggesting that the high level of VEGF contributed to some specific features of POEMS syndrome, such as extravascular volume overload, organomegaly, hemangioma, and peripapillary retinal thickness [18, 19]. In this study, after 12-month treatment, the mean VEGF levels decreased significantly in both gender. But no association between VEGF levels and IIEF score or FSFI score changes had been demonstrated. There are several other options for treatment of POEMS syndrome, including melphalan, thalidomide and transplantation. All these regimens are effective in decreasing VEGF levels. Whether the effects on sexual and gonadal function are specific to lenalidomide/dexamethasone or not is another interesting issue to investigate.
The present study had several limitations. The main limitation of this study is the number of participants. Taking into account the fact that POEMS syndrome is a rare disorder, small populations do not guarantee a very strong insight. A larger number of patients will provide more information between sexual function and gonadal hormone level changes especially in female patients. We also did not collect data regarding educational status or social economic status.
POEMS (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, skin changes) syndrome is a rare blood disorder with multi-system involvement. The cause is unknown. It is marked by elevated plasma cells, platelets, & VEGF (vascular endothelial growth factor) levels. 52% of patients develop optic disc edema which may be vision threatening but the exact etiology of optic disc edema is uncertain. We report a rare finding of peripheral retinal leakage in POEMS syndrome.
A 60 year-old female with POEMS syndrome presented with bilateral blurred vision. Fundi showed grade 3 disc edema OU. Lumbar puncture showed normal opening pressure. CSF analysis showed elevated proteins with no cells. MRI brain and MR Venogram head were unremarkable. Wide field fluorescein angiography demonstrated multifocal tiny vascular leakage and significant anterior temporal leakage.
The authors hypothesize the disc edema in POEMS syndrome may be caused by increased vascular permeability at the optic disc secondary to increased VEGF (vascular endothelial growth factor) levels. Though disc leakage is a well-documented finding in fundus fluorescein imaging, peripheral retinal leakage in POEMS syndrome is not reported.
Other reported ocular manifestations are macular edema, serous macular detachment, infiltrative orbitopathy, venous sinus thrombosis, uveitis, neovascularization of the disc and peripapillary choroidal neovascularization [5,6,7]. Elevated intracranial pressure, vasculitis, infiltration of the nerve or increased VEGF levels may contribute to the disc edema. Most patients have normal opening pressure as ICP fluctuations are common in POEMS syndrome, however they can have raised intracranial pressure due to increased CSF proteins. It is believed that the disc edema is not true papilledema and its most related to optic disc vasculitis [2, 5]. Disc edema tends to persist even after the treatment with monoclonal antibodies, however the tendency to develop optic atrophy is rare . There was a statistically significant difference in VEGF levels among patients with and without disc edema. Literature shows no statistical difference in mean lumbar puncture opening pressure . Body mass index had no statistical association with disc edema in POEMS .
Calciphylaxis is a syndrome consisting of vascular calcification, thrombosis, and skin necrosis. The syndrome develops often in chronic hemodialysis patients. However, there have been several case reports on calciphylaxis in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome, a systemic disease associated with plasma cell dyscrasia and upregulation of vascular endothelial growth factor (VEGF).
Calciphylaxis is often present in patients with POEMS syndrome. Upregulation of multiple inflammatory cytokines such as VEGF and interleukin-6 may contribute to the development of calciphylaxis, by entirely different mechanism from that in chronic dialysis. POEMS syndrome should be recognized as a potential cause of calciphylaxis.
A 62-year-old male with a six-year history of POEMS syndrome was admitted to our hospital because of painful skin ulcers on both thighs. The diagnosis of POEMS syndrome had been made based on polyneuropathy, M-proteinemia (IgA λ type), elevated serum VEGF levels (4810 pg/ml; normal
We reviewed the records of 76 patients with POEMS syndrome (52 men), seen at Chiba University Hospital between 1992 and 2013. Patient age ranged from 31 to 77 years (median, 56 years). All patients fulfilled the diagnostic criteria of POEMS syndrome [13, 20]. One patient with POEMS syndrome receiving chronic hemodialysis was excluded, and the data from the remaining 75 patients were analyzed.
We investigated the presence of ulcers and vessel calcification on CT, and vascular risk factors such as diabetes, hypertension, and hyperlipidemia. We then compared clinical and laboratory data and treatment history between POEMS syndrome patients with probable calciphylaxis and those without it. The overall neuropathy limitation scale (ONLS) was used to evaluate the severity of neuropathy. The presence of pleural effusion/ascites on CT was evaluated and history of events suggestive of thrombosis, such as cerebral infarction, myocardial infarction, and pulmonary thromboembolism, was also compared between POEMS patients with and without probable calciphylaxis. Serum concentrations of VEGF, tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), creatinine, calcium, phosphate, and albumin, and values of platelet count, prothrombin time-international normalized ratio (PT-INR), activated partial thromboplastin time (APTT), fibrinogen degradation products (FDP), and D-dimer measured around the time of CT (within 4 weeks) were recorded. Corrected serum concentration of calcium ([Ca]) which was calculated by formula showed below, were used for statistical analysis . 59ce067264