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poems_syndrome

Poems Syndrome

Related Terms: Lymphedema, Peripheral edema, Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes, lenalidomide, neurological, recovery, Shimpo syndrome, Crow-Fukase Syndrome, PEP Syndrome, Takatsuki syndrome, vascular endothelial growth factor, osteosclerotic myetoma, variable gene, Ischemic stroke, Secondary prophylaxis, Polyneuropathy, lymphoplasmacytic cell, Waldenström's macroglobulinemia

Discussion

POEMS is an acronym for the syndrome's most common signs and symptoms. And the term POEMS syndrome was coined in 1980 by Bardwick:

•Polyneuropathy: Nerve damage causing numbness, tingling and weakness of the hands and feet.

•Organomegaly: Organ enlargement of liver, lymph nodes or spleen.

•Endocrinopathy: Abnormal hormone levels.

•Monoclonal protein: A collection of abnormal blood proteins (immunoglobulins) made from bone marrow cells called plasma cells; also called monoclonal immunoglobulin.

•Skin changes: Increased skin pigment, increased body hair, thickening of the skin and whitening of the nails.(1)

This is another syndrome affecting the lymph nodes (enlargement) and is sometimes referred to as Castleman's Disease. It is defined as the combination of a plasma-cell proliferative disorder (typically myeloma), polyneuropathy, and effects on many other organ systems. (2)

The liver, the lymph nodes, and the spleen are the organs most frequently involved. Enlargement of the lymph nodes and spleen is secondary to changes consistent with Castleman disease (giant angiofollicular hyperplasia, multicentric plasma cell variant) in most patients. Approximately 15% of patients with POEMS syndrome have concomitant evidence of Castleman disease. (3)

Multiple endocrinopathies have been associated with POEMS syndrome, and most patients have more than 1 endocrine abnormality. Many of the abnormalities noted can be explained by elevations in estrogen levels. Impotence and gynecomastia are common among men. Amenorrhea is common among women. Diabetes mellitus and glucose intolerance are also noted in many patients. Other associated endocrinopathies include hypothyroidism, hyperprolactinemia, and hypoparathyroidism. (1)

Symptoms

Most experts agree that patients with Poems Syndrome should have 3 or more of the five main (major criteria) features.

While others believe that the presence of 2 major criteria, including a monoclonal plasma-proliferative disorder and polyneuropathy, in addition to the existence of 1 minor criterion, is sufficient for diagnosis.

Signs and symptoms of Poems syndrome include:

Papilledema, anasarca, pleural effusions, ascites, fever, thrombosis, renal insufficiency, and diarrhea.

In addition dermatologic changes include: hyperpigmentation, skin thickening, sclerodermoid changes, and hypertrichosis. Other skin changes, including whitening of the proximal nail (Terry nails), peripheral edema, hyperhidrosis, clubbing of the fingers, Raynaud phenomenon, and angiomas, have been observed. (3)

In addition, other presenting symptoms may be: Impotence, Gynecomastia, Amenorrhea, Shortness of breath, Hypertrichosis, Hyperpigmentation, Hyperhidrosis, Raynaud phenomenon, Loss of function because of skin tightening, Edema, Diarrhea, Rarely, bone pain and fractures

Additional possible presenting symptoms can be seen at: Poems Syndrome Clinical Presentation

Diagnosis

Diagnoses involves a physical exam, review of your medical history and the use of imaging tests such as x-rays, computerized tomography (CT scan) and/or Positron emission tomography (PET) scan.

Lab tests may include:

Blood tests to asses your blood cells, protein and hormone levels. Biopsy of a small bit of tissue, bone marrow or bone growths. Bone marrow tests to look for the features of the condition including also special stains (immunohistochemistry) to se if there are monoclonal plasma cells in the bone marrow.

Other tests may include a lung (pulmonary) function test and a urine protein test.

Treatment

There are a number of treatments that may be used in Poems syndrome, depending on whether or not the condition is localized or has become widespread.

These treatments include:

•Chemotherapy. When abnormal plasma cells are widespread, your doctor may recommendchemotherapy. •Peripheral blood stem cell transplant. Mayo Clinic doctors have special expertise in performing a peripheral blood stem cell transplant, using stem cells collected directly from blood, to treat POEMS syndrome. For some people, high-dose chemotherapy is given to destroy abnormal cells followed by peripheral blood stem cell transplantation. •Corticosteroids. These powerful anti-inflammatory drugs help subdue your body's immune response and produce extra bone marrow cells. •Radiation therapy. At Mayo Clinic, you have access to the most advanced radiation therapytreatment. (1) In addition, there may need to be intensive supportive care to help manage and lieve the symtoms, physical therapy with the possible use of walking aids or braces and hormona replacement.

If untreated, the condition is progressive and often fatal with a median survival rate of only five years.

Mortality/Morbidity

Morbidity is swpwnsa on the symptoms involved and ranges from skin pigment changes to debilitating weakness and loss o ffunction. The median survival rate for Poems syndrome is 8 years.

With multiple complications, there is shorter survival rate. The complications include xtravascular volume overload (eg, effusions, edema, ascites) and fingernail clubbing. Cardiorespiratory failure, renal failure, infection, and progressive inanition.

(1) MayoClinic

(2) Wikipedia

(3) eMedicine

Studies and Abstracts

Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. May 2012

Mauermann ML, Sorenson EJ, Dispenzieri A, Mandrekar J, Suarez GA, Dyck PJ, Dyck PJ. Source Department of Neurology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA; mauermann.michelle@mayo.edu. Abstract Objective POEMS syndrome (the acronym reflects the common features: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a paraneoplastic disorder with a 'demyelinating' peripheral neuropathy that is often mistaken for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The nerve conduction study (NCS) and electromyography (EMG) attributes that might differentiate POEMS from CIDP and lead to earlier therapeutic intervention were explored. Methods NCS/EMG of POEMS patients identified through retrospective review from 1960 to 2007 were compared with matched CIDP controls. Results 138 POEMS patients and 69 matched CIDP controls were compared. POEMS patients demonstrated length dependent reduction in compound muscle action potentials, low conduction velocities, prolonged distal latencies and prolonged F wave latencies. Compared with CIDP controls, POEMS patients demonstrated: (1) greater reduction of motor amplitudes, (2) greater slowing of motor and sensory conduction velocities, (3) less prolonged motor distal latencies, (4) less frequent temporal dispersion and conduction block, (5) no sural sparing, (6) greater number of fibrillation potentials in a length dependent pattern and (7) higher terminal latency indices (TLI). TLI ≥0.38 in the median nerve demonstrated a sensitivity of 70% and specificity of 77% in discriminating POEMS from CIDP. Conclusions NCS/EMG of POEMS syndrome suggests both axonal loss and demyelination. Compared with CIDP, there is greater axonal loss (reduction of motor amplitudes and increased fibrillation potentials), greater slowing of the intermediate nerve segments, less common temporal dispersion and conduction block, and absent sural sparing. These findings imply that the pathology of POEMS syndrome is diffusely distributed (uniform demyelination) along the nerve where the pathology of CIDP is probably predominantly proximal and distal. Median motor TLI may be useful in clinically distinguishing these disorders.

British Medical Journal

Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy.

Feb 2012

Nasu S, Misawa S, Sekiguchi Y, Shibuya K, Kanai K, Fujimaki Y, Ohmori S, Mitsuma S, Koga S, Kuwabara S. Source Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan. Abstract

BackgroundPOEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome, a rare cause of demyelinating neuropathy associated with multiorgan involvement, has been increasingly recognised. Polyneuropathy is often an initial manifestation and therefore the disorder can be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP).ObjectiveTo elucidate whether POEMS syndrome and CIDP are differentiated based on profiles of neuropathy.MethodsClinical and electrophysiological data were reviewed in consecutive POEMS syndrome (n=51) and typical CIDP (n=46) patients in a single Japanese hospital between 2000 and 2010.ResultsBoth POEMS and CIDP patients showed symmetric polyneuropathy, physiological evidence of demyelination (70% of POEMS patients fulfilled the electrodiagnostic criteria for definite CIDP) and albuminocytological dissociation; 49% of the POEMS syndrome patients had neuropathy onset and 60% of them were initially diagnosed as having CIDP by neurologists. Clinically, POEMSneuropathy more frequently showed severe leg pain (76% vs 7%; p<0.001), muscle atrophy (52% vs 24%; p=0.005) and distal dominant muscle weakness. Electrophysiologically, POEMS syndrome was characterised by less prolonged distal motor latency (mean 5.6 ms vs 8.1 ms; p<0.001) and higher terminal latency index (0.42 vs 0.33; p=0.006) in the median nerves, and unrecordable tibial and sural responses (p<0.001), suggesting demyelination predominant in the nerve trunk rather than in the distal nerve terminals, and axonal loss in the lower limb nerves.ConclusionsBefore development of typical systemic manifestations, POEMS neuropathy can be distinguished from CIDP by the clinical profile and patterns of nerve conduction abnormalities. Recognition of these features leads to early diagnosis and proper treatment for POEMS syndrome.

Journal of Neurology, Neurosurgery & Psychiatry

Ultrasound evaluation of peripheral neuropathy in POEMS syndrome.

Dec 2011

Lucchetta M, Pazzaglia C, Granata G, Briani C, Padua L. Source Department of Neuroscience, University of Padova, Padova, Italy.

Abstract

Keywords: CIDP; neurophysiology; peripheral neuropathy; POEMS syndrome; ultrasound

INTRODUCTION: Polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia whose main neurological feature is a demyelinating polyneuropathy. The aim of our study was to assess the pattern of ultrasound (US) nerve abnormalities in POEMSsyndrome patients.

METHODS: Eight POEMS syndrome patients underwent neurological examination and US evaluation of the median, ulnar, fibular, tibial, and sural nerves. Nerve cross-sectional area and echogenicity abnormalities were analyzed.

RESULTS: US abnormalities were mostly localized at entrapment sites. Enlargements outside the entrapment sites were uncommon. No correlation was found between muscle weakness and focal US findings.

CONCLUSIONS: No specific pattern of US abnormalities was identified in this cohort of patients with POEMS syndrome. The lack of correlation between US and clinical findings may be secondary to the chronic nerve damage that is common inPOEMS syndrome, where the diagnosis is often delayed.

Uterine Glomeruloid Hemangioma in a Patient Without POEMS Syndrome.

Dec. 2011

Giner F, Compañ A, Monteagudo C. Source University Clinic Hospital, University of Valencia, Valencia, Spain.

Abstract

Cutaneous glomeruloid hemangioma is a hallmark of POEMS syndrome. These patients have elevated serum levels of vascular endothelial growth factor. The authors report an extracutaneous uterine glomeruloid hemangioma in an 82-year-old woman with a history of breast and endometrial carcinomas. Within the lumen of myometrial vessels, a lobular, glomeruloid proliferation of capillary-like CD31 and vascular endothelial growth factor receptor-1-positive endothelial cells was found. The capillary loops were lined by endothelial cells, most of them containing PAS-positive and immunoglobulin-positive eosinophilic hyaline globules (thanatosomes). This vascular proliferation was consistent with a glomeruloid hemangioma. Although an extracutaneous glomeruloid vascular proliferation has been found in the retroperitoneal adipose tissue in a patient with POEMS syndrome, this study reports what seems to be the first case of visceral glomeruloid hemangioma in a patient without POEMS syndrome. The authors hypothesize that the glomeruloid endothelial cell proliferation with vascular endothelial growth factor receptor-1 expression may be a paraneoplastic phenomenon.

Surgical Pathology

POEMS syndrome with Guillan-Barre syndrome-like acute onset: a case report and review of neurological progression in 30 cases.

June 2011

Isose S, Misawa S, Kanai K, Shibuya K, Sekiguchi Y, Nasu S, Fujimaki Y, Noto Y, Nakaseko C, Kuwabara S. Source Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan.

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare cause of demyelinating neuropathy with monoclonal plasma cell proliferation, and POEMS neuropathy is usually chronically progressive. Herein, the authors report a 34-year-old woman with POEMS syndrome presenting as acute polyneuropathy. Within 2 weeks of disease onset, she became unable to walk with electrodiagnostic features of demyelination and was initially diagnosed as having Guillan-Barré syndrome. Other systemic features (oedema and skin changes) developed later, and an elevated serum level of vascular endothelial growth factor led to the diagnosis of POEMS syndrome. She received high-dose chemotherapy with autologous peripheral blood stem cell transplantation, resulting in good recovery. The authors also reviewed patterns and speed of progression of neuropathy in the 30 patients with POEMS syndrome; 22 (73%) of them were unable to walk independently with the median period of 9.5 months from POEMS onset (range 0.5-51 months). Whereas the speed of neuropathy progression varies considerably among patients, some POEMS patients can show acute or subacute polyneuropathy. The early diagnosis and treatment could result in rapid improvement as shown in the present patient.

Journal of Neurology, Neurosurgery & Psychiatry

Crow-Fukase (POEMS) syndrome

Apr 2010

[Article in Japanese] Kuwabara S. Source Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.

Abstract

Key Words: Crow-Fukase syndrome, POEMS syndrome, plasma cell dyscrasia, autologous peripheral blood stem cell transplantation, thalidomide

Crow-Fukase syndrome is also called POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome and is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement. The pathogenesis of Crow-Fukase syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably mediated by monoclonal proliferation of plasma cells, is likely to be responsible for most of the characteristic symptoms. However, other cytokines are also upregulated and could contribute to the pathophysiology of thissyndrome. The etiopathophysiology of peripheral neuropathy is unclear, but VEGF may affect the blood-nerve barrier and allow some neurotoxic substances in the serum to access the nerve parenchyma, resulting in nerve demyelination. Moreover, microangiopathy due to proliferative endothelial cells and hypercoagulability may contribute to the development of neuropathy. A recent molecular biological study has shown oligoclonal usage of V(lambda) subfamily in light chain of the M-protein, suggesting that particular patterns of V(lambda) gene are associated with the development of Crow-Fukasesyndrome. There is no established treatment regimen for this syndrome. In appropriate candidates, high-dose chemotherapies with autologous peripheral blood stem cell transplantation is highly recommended, because this treatment can cause obvious improvement in neuropathy as well as other symptoms, with a significant decrease in serum VEGF levels. The indication for this treatment has not yet been established, and the long-term prognosis is unclear. Potential future therapies include the administration of thalidomide or lenalidomide, and anti-VEGF monoclonal antibody (bevacizumab).

Tgaku-Shoin Library

Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients.

April 2008

Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A. Source Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.

Abstract

BACKGROUND:

POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Rare reports of pulmonary manifestations of POEMS syndrome such as pulmonary hypertension exist; however, a comprehensive review of the pulmonary manifestations ofPOEMS syndrome is lacking.

METHODS:

Retrospective review of patients with a diagnosis of POEMS syndrome at our institution between 1975 and 2003. Demographics, signs and symptoms, test findings (ie, radiographs, pulmonary function tests, and echocardiography), and survival data were extracted. Kaplan-Meier survival analysis was performed. In addition, categoric variables were compared using the Pearson chi(2) test or Fisher exact test, where appropriate.

RESULTS:

The study comprised 137 patients (66% male) with a mean age of 51.6 years. Respiratory symptoms were common within 2 years of diagnosis (28%). The median overall survival time was 147 months. Pulmonary manifestations ofPOEMS syndrome included pulmonary hypertension, restrictive lung disease, respiratory muscle weakness, and an isolated diminished diffusing capacity. Significant radiographic findings such as pleural effusions, diaphragm elevation, and increased cardiac silhouette were seen in 23% of patients. When separated by the presence or absence of respiratory muscle weakness, the median survival time was 87 vs 139 months, respectively (p < 0.05). The presence of cough was associated with reduced survival time.

CONCLUSIONS:

Pulmonary manifestations of POEMS are common, and both symptomatic and asymptomatic respiratory involvements are frequent on presentation in patients with POEMS syndrome. Respiratory muscle weakness and cough portend a poorer prognosis. These results suggest the need for increased awareness of the association between POEMS syndrome and pulmonary disease to guide appropriate screening and supportive therapy.

Chest Journal

POEMS syndrome: definitions and long-term outcome.

April 2003

Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA,Fonseca R, Lust JA, Gertz MA. Source Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA. dispenzieri.angela@mayo.edu

Abstract

The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes. The median age at presentation was 51 years; 63% were men. Median survival was 165 months. With the exception of fingernail clubbing (P =.03) and extravascular volume overload (P =.04), no presenting feature, including the number of presenting features, was predictive of survival. Response to therapy (P <.001) was predictive of survival. Pulmonary hypertension, renal failure, thrombotic events, and congestive heart failure were observed and appear to be part of thesyndrome. In 18 patients (18%), new disease manifestations developed over time. More than 50% of patients had a response to radiation, and 22% to 50% had responses to prednisone and a combination of melphalan and prednisone, respectively. We conclude that the median survival of patients with POEMS syndrome is 165 months, independent of the number of syndrome features, bone lesions, or plasma cells at diagnosis. Additional features of the syndrome often develop, but the complications of classic multiple myeloma rarely develop.

Blood Journal

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Classification

DiseasesDB 29226

eMedicine derm/771

Mesh D016878

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Lymphedema People

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