Clinical and biochemical characteristics for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome: a pilot observational study
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Background Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is rare life-threatening condition associated with a clonal plasma cell neoplasm. Objective The aim of this study is to investigate the clinical and biochemical features in patients with POEMS syndrome before and post-therapy. Methods Characteristics of demographic information, underlying diseases, clinical manifestations, laboratory indicators, and imaging examination were retrospectively collected when diagnosed and post-therapy in the patients POEMS syndrome between 2018 and 2024. Results Nineteen newly-diagnosed, treatment-naïve patients with POEMS syndrome were enrolled. The diagnosis of POEMS syndrome was re-analyzed and matched the diagnostic criteria updated in 2023. All patients presented the symptoms of polyneuropathy and positive for M-protein. Most patients suffered with hyperpigmentation ( n = 18), organomegaly ( n = 18), elevated vascular endothelial growth factor (VEGF) ( n = 17), extravascular volume overload ( n = 15), sclerotic bone lesions ( n = 11), and hypothyroidism ( n = 10). Serum alanine aminotransferase, aspartate aminotransferase, total bilirubin, total protein, and albumin levels were down-regulated, while uric acid level was up-regulated in patients with POEMS syndrome. Reduced triiodothyronine, thyroxine, free triiodothyronine levels were negatively correlated with urea nitrogen, creatinine, and uric acid levels in patients with POEMS syndrome. VEGF level, which was negatively correlated with Ca 2+ level ( r = −0.56), was reduced in most patients with POEMS syndrome receiving bortezomib/ixazomib and lenalidomide/thalidomide therapy. Aspartate aminotransferase, total protein, and estimated glomerular filtration rate levels were increased, while creatinine and uric acid levels were reduced post-therapy in patients with POEMS syndrome. Conclusion Patients with POEMS syndrome had impaired liver and renal function, and effective therapy might partly repair the liver and renal dysfunction.Keywords:
POEMS syndrome
Organomegaly
Skin hyperpigmentation
Blood urea nitrogen
Polyneuropathy is an initial presentation and essential feature of POEMS ( polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Neuropathy is typically distal, symmetric and slowly progressive with demyelinating changes. After a gradual proximal spread, it usually results in severe muscle weakness and functional disabilities. In the present report, we describe a 40-year-old diabetic male presented with gradually progressive weakness of both lower limbs for 1 year followed by the involvement of both upper limbs for the last 3 months. On examination hyperpigmentation, lymphadenopathy, gynecomastia, anasarca, hepatomegaly, bilateral optic disc swelling, sensory-motor polyneuropathy was found. Laboratory findings showed IgG lambda monoclonal gammopathy, raised VEGF, sensory-motor demyelinating and axonal polyneuropathy. All findings were consistent with POEMS syndrome. The patient was treated with lenalidomide and dexamethasone cyclical therapy with some clinical improvement.
Bangladesh Journal of Neuroscience 2019; Vol. 35 (1): 51-56
POEMS syndrome
Organomegaly
Proximal muscle weakness
Skin hyperpigmentation
Anasarca
Gynecomastia
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Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare paraneoplastic disease with a high prevalence of skin symptoms. Few studies have focused on skin responses to systemic treatment of this disease.To evaluate skin responses after treatment with low-dose lenalidomide plus dexamethasone and determine their relationship with vascular endothelial growth factor (VEGF) and hematological responses.A total of 41 consecutive Chinese patients who were at least 18 years of age with newly diagnosed POEMS syndrome were enrolled. 36 of them completed 12 cycles of treatment. Skin, serum VEGF, hematological and neurological responses were documented at 1, 2, 3, 6, 9, and 12 months during treatment.Skin manifestations were not associated with serum VEGF levels at baseline. Of the patients with hyperpigmentation, hemangiomas, hypertrichosis, white nails, or acrocyanosis separately, 26/34 (76.5%), 11/30 (36.7%), 14/15 (93.3%), 21/21 (100%), and 4/4 (100%) achieved complete responses at 12 months. Reduction in hyperpigmentation after 12 months reflected a good VEGF response (P = 0.017).Low-dose lenalidomide plus dexamethasone therapy is effective in reversing skin changes in patients with POEMS syndrome.Clinicaltrials.gov, identifier NCT01816620.
POEMS syndrome
Organomegaly
Skin hyperpigmentation
Hypertrichosis
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In 1971, the author reported an autopsy case of a 48-year-old Japanese man with polyneuropathy, skin hyperpigmentation, diabetes mellitus, and monoclonal gammopathy. Previously, a total of 2 cases of solitary myeloma accompanied by polyneuropathy and endocrinological disorders have been reported by Fukase et al in 1968 and by Shimomori and Kusumoto in 1970 in Japan. The author's case is the first reported non-myeloma case where polyneuropathy associated with dermatoendocrionological changes and dysglobulinemia was observed. In 1956, Crow reported the British case of a 54-year-old man with osteosclerotic myeloma, polyneuropathy, edema, skin hyperpigmentation, clubbed fingers, white nails, and lymphadenopathy. The term Crow-Fukase syndrome was first used in 1984 in a study performed by Nakanishi et al that involved 102 Japanese patients. In 1986, Bardwick et al suggested the acronym POEMS-i.e., polyneuropathy (P), organomegaly (O), endocrinopathy (E), M-protein (M), and skin changes (S)-that represents the most constant and important features of this syndrome. In this paper, the author has described 3 cases that have been reported earlier by the author and has reviewed important Japanese and international literature related to this syndrome. Recent studies have revealed that the signs and symptoms of patients with this syndrome dramatically recover on using recently developed therapies for myeloma, such as autologous peripheral blood stem cell transplantation and thalidomide therapy.
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Skin hyperpigmentation
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POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome is a multisystem disorder. Peripheral neuropathy and monoclonal plasma cell disorder are the most common manifestations of POEMS. Although osteosclerotic or mixed sclerotic-lytic lesions are typical, osteolytic lesions are rarely encountered. We present a case of a 39-year-old male with a history of multiple endocrine disorders who presented with paresthesia, edema, and hyperpigmentation and was eventually diagnosed with POEMS syndrome. Patients with unexplained neuropathy should be evaluated for POEMS syndrome, especially when it is associated with other findings like endocrinopathy, organomegaly, skin changes, or edema.
Organomegaly
POEMS syndrome
Peripheral edema
Skin hyperpigmentation
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POEMS syndrome
Organomegaly
Adrenocortical Insufficiency
Skin hyperpigmentation
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POEMS syndrome
Organomegaly
Skin hyperpigmentation
Skin biopsy
Nerve biopsy
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POEMS syndrome is a unique clinical entity, the diagnosis of which is made when polyneuropathy and monoclonal gammopathy occur together, associated with other changes such as organomegaly, endocrinopathy, skin changes and papilledema. Cutaneous manifestations are heterogeneous, with diffuse cutaneous hyperpigmentation, hemangiomas and hypertrichosis occurring more frequently. We report the case of a 65- year-old female patient with this syndrome, diagnosed after 15 years of disabling peripheral neuropathy.
Organomegaly
POEMS syndrome
Hypertrichosis
Monoclonal gammopathy
Papilledema
Skin hyperpigmentation
Genodermatosis
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POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes) refers to a rare paraneoplastic entity linked to a plasma cell disorder, characterized by multiple systemic manifestations that must be studied together to establish a timely diagnosis. We report a case of a 60-year-old female who had been initially classified to have Guillain Barré syndrome for one year and seven months, receiving three cycles of immunoglobulin without a positive response. The clinical picture was characterized by painful paresthesias in four limbs and paraparesis, with the patient also developing distal cutaneous hyperpigmentation and multiple adenopathies. Neuroconduction studies revealed chronic sensorimotor axonal polyneuropathy and albumin-cytological dissociation was evidenced in the study of cerebrospinal fluid (CSF). In the subsequent evaluation, Lambda light chains and a lymph node biopsy compatible with Castleman's disease were found, and hence it was determined that the patient met the criteria for POEMS syndrome. This case report highlights the importance of incorporating other diagnostic perspectives when encountering patients with polyneuropathy of immunological origin who fail to respond to conventional therapies.
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POEMS syndrome
Skin biopsy
Skin hyperpigmentation
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Introduction: POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) syndrome is a rare multisystem disorder. Castleman disease is an atypical lymphoproliferative disorder of unknown cause, which can be associated to POEMS. Clinical Case: This is the case of a 37-year-old patient with 2-year history of numbness and weakness in lower limbs associated to multiple adenopathy, skin hyperpigmentation and hypertrichosis. Electromyography showed active chronic sensory-motor polyneuropathy of axonal type; and serum electrophoresis showed a monoclonal band of A Immunoglobulin lambda type. Submaxillary lymph node biopsy was consistent with Castleman Disease. Conclusion: This report highlights the fact that Castleman Disease is frequent among patients with POEMS syndrome, constituting a significant variant, and should always be ruled out. Furthermore, plasmapheresis could be a useful alternative among patients with severe neurological symptoms.
POEMS syndrome
Organomegaly
Castleman disease
Hypertrichosis
Skin hyperpigmentation
Dyscrasia
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POEMS syndrome is a rare multisystemic syndrome characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. We report a case of POEMS syndrome associated with unclassifiable myeloproliferative disorder presenting with polyneuropathy, hepatosplenomegaly, hypothyroidism, hyperprolactinemia, monoclonal gammopathy, hyperpigmentation, hypertrichosis, ascites, peripheral edema, and pulmonary hypertension.
POEMS syndrome
Organomegaly
Hepatosplenomegaly
Hypertrichosis
Peripheral edema
Monoclonal gammopathy
Skin hyperpigmentation
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