scholarly journals Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome With IgG Kappa/IgG Lambda Biclonal Gammopathy: A Rare Presentation of a Rare Disease

Cureus ◽  
2020 ◽  
Author(s):  
Leonardo Mejia Buritica ◽  
Vanessa Santiago-Pacheco ◽  
Oliver Perilla ◽  
Dayana Quintero
Keyword(s):  
Rare Disease ◽  
Monoclonal Gammopathy ◽  
Poems Syndrome ◽  
Rare Presentation ◽  
Skin Changes ◽  
Biclonal Gammopathy

scholarly journals POEMS syndrome complicated by ischaemic stroke and cerebral vasculitis

2019 ◽  
Vol 12 (7) ◽  
pp. e229428
Author(s):  
Leonid Vasilevskiy ◽  
Hossein Akhondi ◽  
Ann Wierman
Keyword(s):  
Ischaemic Stroke ◽  
Monoclonal Gammopathy ◽  
Cerebral Vasculitis ◽  
Poems Syndrome ◽  
Skin Changes

We present a case of ischaemic stroke in the context of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome associated with cerebral vasculitis as confirmed by imaging. There is little information on the prevalence of cerebral vasculitis in POEMS patients, and we found only one previous report of such case in the literature.

Successful Autologous Peripheral Blood Stem Cell Transplantation in 3 Women with POEMS Syndrome: A Single Centre Experience

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4504-4504
Author(s):  
Giusy Antolino ◽  
Giovanni Antonini ◽  
Antonella Ferrari ◽  
Giacinto La Verde ◽  
Raffaele Porrini ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Light Chain ◽  
Peripheral Blood ◽  
Monoclonal Gammopathy ◽  
Peripheral Blood Stem Cell ◽  
Poems Syndrome ◽  
Skin Changes ◽  
Clinical Stabilization ◽  
Blood Stem Cell Transplantation

Abstract Abstract 4504 INTRODUCTION POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) is a rare multi-system disease characterized by chronic progressive polyneuropathy and plasma-cell dyscrasia. A major diagnostic criterion for POEMS is elevation of circulating vascular endothelial growth factor (VEGF), which is believed to play a pathogenic role in this disease. We report 3 cases of POEMS syndrome treated with high-dose melphalan and autologous peripheral blood stem cell transplantation (PBSCT). PATIENTS AND METHODS CASE 1 A 32-year-old woman presented with a progressive gait difficulty, weakness in limb muscles, skin hyperpigmentation, axillar and laterocervical lymphoadenopathies, peripheral edema. Neurophysiological testing showed a motor and sensory axonal polyneuropathy. At laboratory testing, thrombocytosis and monoclonal gammopathy IgA-λ were detected. A biopsy of laterocervical lymphonode was positive for Castleman’s disease. Initial treatment with intravenous immunoglobulin (IVIg) failed to result in clinical benefit. After cyclophosphamide 2 g plus G-CSF priming a total of 7.1×106/kg stem cells were collected from peripheral blood and used to perform double autologous transplantation at interval of 5 months following conditioning with melphalan 140 mg/sq/m. CASE 2 A 48-year-old woman presented with numbness and paresthesias in foot soles, weakness in lower limbs and axillar lymphoadenopathy. Nerve conduction studies showed motor and sensor demyelinating polyneuropathy. A monoclonal gammopathy IgG K-λ was present and bone marrow biopsy was unremarkable. She was treated with prednisone and plasma exchange with progressive improvement and clinical stabilization for two years. Unfortunately she presented a relapse of neurological symptoms responding to the same therapeutic strategy. A second relapse occurred after 3 months with appearance of sensory ataxia, xerotic skin, hepatomegaly. Laboratory analysis revealed biclonal gammopathy IgG k-λ and IgA-λ and Bence Jones proteinuria (λ light chain). Radiologic studies showed lytic lesions with sclerotic rim in homerus, VII rib and iliac bone and mediastinal, axillary and iliac lymphadenopathies. The patient received cyclophosphamide 2 g and G-CSF for mobilization. Melphalan 200 mg/sq/m was administered intravenously as conditioning regimen followed by reinjection of the cells collected. CASE 3 A 44-year-old woman presented with asthenia, scleroderma-like skin changes, weight loss, limb paraesthesia and weakness. Neurophysiological testing showed a motor and sensor demyelinating polyneuropathy. She presented thrombocytosis and a monoclonal IgG-λ protein at laboratory tests. Radiologic studies showed laterocervical, axillary, mediastinal lymphoadenopathies, multiple thyroid nodules and a mixed sclerotic and lytic lesion in the pelvis and in the right humeral head. Biopsy of this lesion demonstrated a plasmacytoma with λ light chain restriction. Initial treatment with IVIg was unsuccesful. She received oral therapy with melphalan and prednisone for 3 years obtaining clinical stabilization, then she was treated with monthly zoledronic acid infusion only. After 3 years she presented a relapse of neurological symptoms, appearance of hepatomegaly, axillar and laterocervical lymph nodes enlargement, peripheral edema. She therefore underwent PBSC harvesting following cyclophophamide priming (2 g), than she received melphalan (200 mg/sq/m) with CD34-selected PBSC rescue. RESULTS The post-transplant course was uneventful other than the development of febrile neutropenia in 2 cases. Patients 1 and 2 presented a progressive improvement in strength, physical skills and peripheral neuropathy after few months. They are well with clinical stabilization after 4 and 6 years, respectively, from PBSCT. At 3 months from PBSCT, patient 3 presents progressive neurological improvement and reduction of organomegalies. CONCLUSIONS Although treatment for POEMS is not standardized, disseminated disease require systemic therapy. No randomized controlled trials have been conducted, but patients with POEMS have been successfully treated with PBSCT since 1998. Our cases confirm its usefulness and safety and support the idea that, although delayed intervention maintains its efficacy, PBSCT could be considered early during the disease course to obtain the best response. Disclosures: No relevant conflicts of interest to declare.

scholarly journals POEMS syndrome as an uncommon cause of papilledema

2014 ◽  
Vol 6 (1) ◽  
pp. 105-108
Author(s):  
Carlos Mario Franco ◽  
Kepa Balparda ◽  
Ana María Díaz ◽  
Daniel Jiménez ◽  
Ana Paulina Pamplona
Keyword(s):  
Monoclonal Gammopathy ◽  
Signs And Symptoms ◽  
Poems Syndrome ◽  
Skin Changes ◽  
Ocular Manifestations ◽  
Plasma Cell Disorder ◽  
Uncommon Condition ◽  
Bilateral Papilloedema ◽  
Ocular Signs ◽  
Cell Disorder

Background: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is an uncommon condition related to a paraneoplastic syndrome secondary to an underlying plasma cell disorder. Among the myriad of manifestations of the disease, ocular signs and symptoms are relatively prevalent, affecting about half of all patients with the disease. Objective: To report the ocular manifestations of POEMS syndrome. Case: A 47-year-old lady diagnosed to have POEMS syndrome presented with painless progressive visual diminution. Her color vision was impaired. There was bilateral papilloedema. Conclusion: POEMS syndrome should be considered among the differential diagnoses of all patients with a bilateral papilledema in which no other cause can be readily elucidated. DOI: http://dx.doi.org/10.3126/nepjoph.v6i1.10781 Nepal J Ophthalmol 2014; 6 (2): 105-108

scholarly journals Recurrent Transient Ischemic Attacks in a Patient with POEMS Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
A. Akyol ◽  
B. Nazliel ◽  
H. Z. Batur Caglayan ◽  
Y. Oner ◽  
G. Turkoz Sucak
Keyword(s):  
Monoclonal Gammopathy ◽  
Cerebral Arteries ◽  
Poems Syndrome ◽  
Prior History ◽  
Transient Ischemic Attacks ◽  
Skin Changes ◽  
Pathophysiologic Mechanism ◽  
Middle Cerebral Arteries ◽  
History Of ◽  
Intracranial Vasculature

A 47-year-old female with a prior history of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome was admitted with transient ischemic attacks complicated by dysarthria and right-sided hemiparesis. A blood survey indicated thrombocytosis and hyperfibrinogenemia while imaging of intracranial vasculature showed occlusion of the bilateral middle cerebral arteries. POEMS syndrome, of which arterial thromboses have been mentioned as a manifestation, is rarely accompanied by transient ischemic attacks. The pathophysiologic mechanism is yet unclear and needs further investigation.

scholarly journals Waldenstrom’s Macroglobulinemia and Peripheral Neuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes with a Bleeding Diathesis and Rash

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
S. Haider ◽  
T. Latif ◽  
A. Hochhausler ◽  
F. Lucas ◽  
N. Abdel Karim
Keyword(s):  
Bone Marrow ◽  
Peripheral Neuropathy ◽  
Monoclonal Gammopathy ◽  
Poems Syndrome ◽  
Waldenström’S Macroglobulinemia ◽  
Excessive Bleeding ◽  
Skin Changes ◽  
Waldenstrom's Macroglobulinemia ◽  
Waldenstrom’S Macroglobulinemia ◽  
Waldenström's Macroglobulinemia

We report a case of a 29-year-old male who presented with paraesthesia and skin lesions with excessive bleeding after skin biopsy leading to hematology consultation. He was found to have prolonged partial thromboplastin time (PTT) and monoclonal gammopathy on serum protein electrophoresis (SPEP). He experienced excessive bleeding leading to hospitalization after bone marrow biopsy and required blood transfusion. He was diagnosed with Waldenstrom's Macroglobulinemia (WM), based on the presence of IgM-κtype monoclonal (M) protein and infiltration of lymphoplasmacytic cells identified in bone marrow aspirates. He was noticed to have features of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome). This is a very rare case of WM with POEMS syndrome which responded to chemotherapy using bortezomib, steroids, and rituximab.

scholarly journals Do you know this syndrome?

2013 ◽  
Vol 88 (6) ◽  
pp. 1009-1010 ◽  
Author(s):  
Guida Santos ◽  
Sara Lestre ◽  
Alexandre João
Keyword(s):  
Peripheral Neuropathy ◽  
Female Patient ◽  
Monoclonal Gammopathy ◽  
Poems Syndrome ◽  
Clinical Entity ◽  
Cutaneous Manifestations ◽  
Skin Changes

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.

scholarly journals Skin: mirror of marrow- case of POEMS syndrome

2017 ◽  
Vol 3 (2) ◽  
pp. 286
Author(s):  
R. B. Chavan ◽  
V. A. Belgaumkar ◽  
A. S. Salunke ◽  
S. S. Tharewal ◽  
N. M. Bansal
Keyword(s):  
B Cells ◽  
Plasma Cell ◽  
Monoclonal Gammopathy ◽  
Poems Syndrome ◽  
Skin Disorders ◽  
Monoclonal Immunoglobulin ◽  
Skin Changes ◽  
Monoclonal Gammopathies ◽  
Clonal Proliferation ◽  
Plasma Cell Dyscrasias

<p class="abstract"><span lang="EN-IN">Monoclonal gammopathy is clonal proliferation and accumulation of immunoglobulin producing B-cells. A variety of skin disorders are associated with an increased level of monoclonal immunoglobulin proteins. Synonyms such as monoclonal gammopathies, paraproteinemias, plasma cell dyscrasias and dysproteinemias are used to designate gammaglobinopathies. Here in we report a case of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome presenting with sclerodermoid features.</span></p>

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