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Internal Medicine (Tokyo, Japan) Feb 2023A 36-year-old man developed polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome after conversion from solitary plasmacytoma of...
A 36-year-old man developed polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome after conversion from solitary plasmacytoma of bone to multiple myeloma. Twenty-four days following the neurological onset, he lost his independent walking ability. The level of serum vascular endothelial growth factor (VEGF) at diagnosis was 5,250 pg/mL. Three months after initiating treatment, he regained his independent walking ability in line with a reduction in the elevated serum VEGF level. Due to their genomic instability gained during conversion, myeloma cells may overproduce humoral factors and cytokines, possibly contributing to the development of neuropathy as well as the production of VEGF.
Topics: Male; Humans; Adult; Plasmacytoma; POEMS Syndrome; Multiple Myeloma; Vascular Endothelial Growth Factor A; Endocrine System Diseases
PubMed: 35831107
DOI: 10.2169/internalmedicine.9101-21 -
JAAD Case Reports Jul 2022
PubMed: 35799683
DOI: 10.1016/j.jdcr.2022.05.019 -
Therapeutic Advances in Neurological... 2022Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a multisystem disease associated with underlying plasma...
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a multisystem disease associated with underlying plasma cell neoplasm. Here, we present two cases of POEMS syndrome that manifested on magnetic resonance neurography as an increasing bone mass with cortical disruption, direct invading nerve roots and lumbar gluteal muscles. These features have not been previously reported. We also report a case with diffuse hypertrophy and enhancement of the brachial and lumbosacral plexus, which mimics the most common chronic inflammatory demyelinating polyradiculoneuropathy. Moreover, we detected perineurium effusions in the plexus, coupled with a variety of myofascitis and atrophy in denervated muscle. The case series is of concern to atypical magnetic resonance imaging findings of POEMS syndrome in the bone and peripheral nerve system as critical attacked target organs, which would be facilitating diagnosis.
PubMed: 35785402
DOI: 10.1177/17562864221106350 -
HemaSphere Jul 2022
First-line Usage of Daratumumab, Lenalidomide, Dexamethasone (DRd) Combination in a Case of Castleman Disease Variant of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome (CD-POEMS).
PubMed: 35747589
DOI: 10.1097/HS9.0000000000000728 -
Cureus May 2022A 76-year-old man presented with a four-month history of progressive bilateral lower limb muscle weakness and dysesthesia. The patient had extravascular volume overload,...
A 76-year-old man presented with a four-month history of progressive bilateral lower limb muscle weakness and dysesthesia. The patient had extravascular volume overload, and laboratory findings confirmed hypothyroidism, renal dysfunction, and chronic inflammation. Serum protein and immunofixation electrophoresis revealed biclonality of immunoglobulin A (IgA)-kappa and IgA-lambda, which was attributed to chronic inflammation. Subsequently, we detected the proliferation of monoclonal plasma cells in the bone marrow, which led to a diagnosis of POEMS syndrome. Despite the initiation of chemotherapy, the patient died of aspiration pneumonia. In this case, biclonal gammopathy in peripheral blood delayed a diagnosis of POEMS syndrome.
PubMed: 35747029
DOI: 10.7759/cureus.25153 -
Cureus May 2022POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome is a multisystem disorder. Peripheral neuropathy and monoclonal...
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.
PubMed: 35719778
DOI: 10.7759/cureus.24980 -
Cureus May 2022Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The...
Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma (PCD), and at least one of the minor criteria. The most frequent liver manifestation is hepatomegaly. Idiopathic portal hypertension (IPH) has been reported rarely in POEMS syndrome. The precise etiopathogenesis of IPH is not fully elucidated. We report a 46-year-old male patient presenting with POEMS syndrome. He presented postprandial vomiting and left tinnitus. Loss of appetite, leanness, and excessive sweat were concomitant symptoms. Abdominal examination revealed splenomegaly and an absence of hepatomegaly. Abdominal ultrasonography was therefore performed, revealing hepatosplenomegaly with dilatation of the splenic vein. An abdominal computed tomography confirmed the presence of an 18.5 cm splenomegaly with dilatation of the splenic and portal vein. Upper endoscopy with biopsy showed minimal, non-atrophic, mildly active chronic, follicular antrofundic gastritis without esophageal varices. Laboratory and radiological examinations could not confirm the etiology of portal hypertension. The liver biopsy suggested hepatoportal sclerosis, compatible with IPH. The patient initially received six courses of the CTD (cyclophosphamide, thalidomide, and dexamethasone) protocol. He subsequently proceeded to an autologous stem cell transplant (ASCT), and the patient achieved a considerable improvement. POEMS syndrome could be complicated with IPH. There are only a few cases of IPH associated with POEMS syndrome in the literature. This case highlights the manifestation of portal hypertension in POEMS syndrome.
PubMed: 35698702
DOI: 10.7759/cureus.24923 -
Journal of Orthopaedic Case Reports Jan 2022Multiple myeloma is a plasma cell neoplasm that is commonly associated with lytic bone lesions; however, osteosclerotic multiple myeloma is a rare entity. Osteosclerotic...
INTRODUCTION
Multiple myeloma is a plasma cell neoplasm that is commonly associated with lytic bone lesions; however, osteosclerotic multiple myeloma is a rare entity. Osteosclerotic multiple myeloma has been reported in association with POEMS syndrome.
CASE PRESENTATION
A 60-year-old female patient presented to us with low back pain for 2 months. The investigations revealed that the patient had multiple osteosclerotic lesions in the axial skeleton with a L4 ivory vertebra. The serum immunoelectrophoresis was negative. Positron emission tomography scan was done which showed multiple skeletal lesions in the right iliac bone, left femoral neck, thoracic vertebrae T1, T6, T12, and lumbar vertebra L4. Biopsy and immunohistochemistry of the lesion showed plasma cell proliferation; producing lambda light chains. However, there were no features of POEMS syndrome including polyneuropathy, organomegaly, endocrinopathy, and skin changes. The patient was started on chemoradiation and achieved clinical remission and was asymptomatic at 12 months follow-up.
CONCLUSION
Osteosclerotic myeloma without the features of POEMS syndrome is an extremely rare entity. This case reports documents a unique clinical scenario of osteosclerotic non-secretory light chain myeloma without POEMS syndrome.
PubMed: 35611283
DOI: 10.13107/jocr.2022.v12.i01.2598 -
Internal Medicine (Tokyo, Japan) Jan 2023TAFRO syndrome was first described in 2010, standing for thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly. Because the lymph node histopathology of...
TAFRO syndrome was first described in 2010, standing for thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly. Because the lymph node histopathology of TAFRO syndrome mimics idiopathic multicentric Castleman disease (iMCD), some researchers consider TAFRO syndrome to be a subtype of iMCD. However, the clinical features of TAFRO syndrome considerably differ from those of iMCD without TAFRO. The clinical features of patients with TAFRO syndrome with or without iMCD-histopathology are similar, and these patients require an accurate diagnosis and urgent treatment. Although a histological diagnosis, including a differential diagnosis, is important, lymph node involvement in patients with TAFRO syndrome is usually modest or sometimes absent. Furthermore, a bleeding tendency due to thrombocytopenia and severe anasarca hampers performing a biopsy. Nonetheless, patients with various other disorders may manifest TAFRO syndrome-like symptoms, making the differential diagnosis in borderline cases difficult. Therefore, the establishment of precise and specific biomarkers is important.
Topics: Humans; Castleman Disease; Lymph Nodes; Thrombocytopenia; Edema
PubMed: 35598998
DOI: 10.2169/internalmedicine.9622-22 -
BMC Rheumatology May 2022POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin...
Raynaud's phenomenon and positive antinuclear antibodies as first manifestation of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes): a case report.
BACKGROUND
POEMS syndrome is a rare paraneoplastic syndrome caused by plasma cell disorder almost always lambda restricted. Secondary Raynaud's phenomenon is an overlooked skin manifestation of the disease even though it is present in twenty percent of patients. On POEMS syndrome have not been described positive antinuclear antibodies (ANA) and this could lead to a misdiagnosis of autoimmune disease, mainly systemic sclerosis.
CASE PRESENTATION
A 47-year-old man presented with color changes on fingertips consistent with biphasic Raynaud's phenomenon; an antinuclear antibody test was positive (at 1:320 titers in a speckled pattern) with normal nailfold capillaroscopy. Clinical features of systemic sclerosis were absent. Twenty-four months later, the patient presented symmetric sensorimotor demyelinating polyneuropathy, and he was diagnosed with Guillain-Barre syndrome; treatment with intravenous gammaglobulin had an incomplete response. Raynaud's phenomenon persisted associated with acrocyanosis, white nails, and positive ANA (1:1280 in a nucleolar pattern). POEMS syndrome was suspected, and serum protein electrophoresis (SPEP) was done. The SPEP revealed polyclonal gammopathy, and serum immunofixation showed monoclonal (M)-protein (IgG lambda). Serum vascular endothelial growth factor concentration showed increased levels. The patient was diagnosed with POEMS syndrome, and treatment with lenalidomide and dexamethasone improved the Raynaud's phenomenon, acrocyanosis, and white nails, but the neurological response was partial.
CONCLUSIONS
POEMS syndrome may mimic clinical manifestations of systemic sclerosis v.g. Raynaud's phenomenon, skin thickening, telangiectasia, and positive ANA. Raynaud's phenomenon may precede other clinical manifestations of POEMS syndrome by several months. It is necessary to have a high index of suspicion for the diagnosis, especially in patients with peripheral polyneuropathy and monoclonal paraprotein. The significance of positive ANA in this condition is unknown and deserves further investigation.
PubMed: 35585632
DOI: 10.1186/s41927-022-00258-y