POEMS Syndrome Symptoms and Treatment

POEMS Syndrome Symptoms and Treatment POEMS Syndrome: Paraproteinemic neuropathies, Organomegaly, Endocrinopathy, M-protein and Skin changes Abstract The POEMS syndrome, also known as Crow-Fukase syndrome, is a rare multi organ disorder characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein spike and skin changes. Other associated features, such as sclerotic bone lesions, edema, ascites, hematological disorÂÂ ­ders and Castleman disease can also be present. We report a case of POEMS syndrome who presented with insidious onset, progressive sensorimotor polyneuropathy, pedal edema, ascites, hepatomegaly along with skin changes. X-ray pelvis showed osteosclerotic lesions. Thyroid function tests showed hypothyroidism. M-protein (IgG) monoclonal band was seen on immunoelectrophoresis. The patient was started on melphalan and corticosteroid combination therapy. We emphasis on the importance of recognizing a challenging diagnosis of a rare disease, which is shown to be treatment responsive. Introduction POEMS syndrome is a rare paraneoplastic disorder of plasma cell dyscrasias, which was first described in 1956 by Crow and then in 1968 by Fukase [1]. The name POEMS was given to it by Bardwick and co-workers in 1980 based on five salient features: polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes [1]. It is more prevalent in men, with male to female ratio of 2.5:1. It usually manifests in 5th and 6th decades of life. Its inheritance is uncertain and its pathophysiology is still not well understood. Case Presentation A 40-year-old male presented with progressive weakness, tingling and numbness sensation in both lower limbs for two years. He had swelling of lower limbs, abdominal distention and dermatologic changes in form of discoloration and thickening of skin over the cheeks, nose, hands and feet for one year. He had also gave history of erectile dysfunction and loss of libido for six months. There was no history of syncope, bony pain or drug abuse. He had no previous history of hypertension, diabetes or tuberculosis. On physical examination, the patient had bilateral pedal edema [Figure 1.c] and abdominal distention [Figure 2.a]. Skin was thickened and hyperpigmented over the face, fingers of the hands and shin (Figure.1a,b,c). Bilateral gynecomastia and testicular atrophy were present. Abdominal examination showed hepatomegaly and ascites. Higher mental functions and speech were normal. Fundus examination showed papilledema on both side and rest of the cranial nerves examination were normal. Motor power in upper limbs was normal and in lower limbs showed predominant distal weakness [Medical Research Council (MRC) 4/5 at hip joint and 4-/5 at ankle joint). Deep tendon reflexes in upper limb were diminished (+1) including biceps, triceps and supinator and absent in lower limbs. There was 30% loss in pain, touch and temperature sense in both lower limbs below knees. Posterior column sensations (joint position and vibration sense) were also impaired in lower limbs below the anterior superior iliac s pine. Romberg sign was positive. Hemogram, liver and renal function tests, muscle enzymes (creatine phosphokinase), serum ferritin and vitamin B12 level were normal. Serum total protein was 7.1 gm/dL, albumin 3 gm/dL, globulin 4.1 gm/dl, and A:G ratio 1:1.3. Fasting and postprandial blood sugar level were normal. Thyroid function test showed raised TSH level (16.62 ÃŽ ¼/ml). Luteinizing hormone (LH) and testosterone levels were 15 IU/L (1.8-8.6 IU/L) and 111 ng/L (300-1,000 ng/dL), respectively. Antinuclear antibody (ANA), Rheumatoid factor (RF), serum human immunodeficiency virus (HIV) ELISA test, hepatitis B and C markers were negative. Ultrasonography of abdomen showed hepatomegaly (16 cm), moderate ascites and enlargement of multiple lymph nodes along the iliac vessels. Fine needle aspiration cytology of mesenteric lymph node was inconclusive. Ascitic fluid examination revealed exudative nature (SAAG Discussion POEMS syndrome is a rare, multiple system disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal or M-protein band and skin changes. Any three of the five features may be present to establish diagnosis [2]. However, some authors have proposed clinical criteria for diagnosis in which includes two major criteria, which can be either presence of polyneuropathy or plasma cell proliferative disorder. Minor criteria include sclerotic bone lesions, organomegaly, edema, endocrinopathy, papilledema or skin changes [2]. Polyneuropathy is a predominant feature of POEMS syndrome and is found in >90% of the cases. It is usually a sensorimotor, axonal and demyelinating type polyneuropathy [3]. As in our patient, both axonal and demyelinating polyneuropathy are seen on electrodiagnostic studies. The mechanism of neuropathy is not known but the recent evidence of the presence of anti-neural antibodies points to an immunological mechanism [4]. Endocrinopathies occur with a frequency of 60%-80% and the most common are gonadal failure (70%) and glucose intolerance/diabetes mellitus (50%). Hypo or hyperthyroidism, hyperprolactinemia and adrenal insufficiency have also been reported. The mechanism of endocrinopathy is also not obvious; however, involvement of direct acting antibodies against hypothalamo-hypophyseal-axis and endocrine end organs has been hypothesized [5]. In our patient, impotence, loss of libido and testicular atrophy and hypothyroidism on ancillary laboratory investigation were present. Increased levels of vascular endothelial growth factor (VEGF) are found in POEMS syndrome. VEGF increases microvascular permeability, thereby inducing edema, ascites and pleural effusions as were present in our patient [6,7]. However, measurement of VEGF level in ascites was not available in our patient. Papilledema may be seen in approximately 37% of patients and is not associated with the increase in intracranial pressure. The real cause of papilledema is not still known. Hepatomegaly may be seen in up to 50% of patients with splenomegaly and lymphadenopathy occurring less often. The hyperpigmentation over the face, legs and hands was also observed in our case. The skin changes usually observed in POEMS syndrome are hyperpigmentation, lichenification, hypertrichosis, sclerodermoid changes and glomeruloid hemangiomas. Skin biopsy may show inflammation, fibrosis, or nonspecific changes. Monoclonal protein is detected in more than 90% of patients and may become positive in the follow- up of patients who have no monoclonal gammopathy initially [8]. Nearly all cases reported in the literature show lambda positivity as in our patient. It may be rarely found in urine and cerebrospinal fluid (CSF). The natural course of POEMS syndrome is chronic, with a reported median survival for a decade (8-13.8ys). The morbidity depends on the extent and number of systems involved. The cause of POEMS syndrome is still unknown. It is tempting to incriminate the presence of lambda light chains in the pathogenesis because of their unexpected frequency (more than 95% of patients), but histopathologic review of affected organs and nerves does not support that it is a form of deposition disorder. Increased levels of cytokines IL-1ß, TNF-Ã ¡ and IL-6, more specifically VEGF, appear to play a pathogenic role in the disorder [9,10]. In view of the constellation of a mixed polyneuropathy, monoclonal gammopathy, osteosclerotic myeloma, extravascular volume overload, bilateral papilledema, skin changes and endocrinopathies, our patient was diagnosed as POEMS syndrome. However, other close differential diagnosis like tuberculosis and hemochromatosis were ruled out with appropriate investigations. Patient was treated with combination of alkylating agent melphalan and corticosteroid. To conclude, when a patient present with unexplained sensorimotor polyneuropathy, signs of extravascular volume overload and evidence of other system involvement, a high index of suspicion should be kept for a diagnosis of POEMS syndrome, to avoid missing this rare syndrome, which is amenable to treatment. Figure Legends Figure 1. Photographs of patient showing skin hyperpigmentation over the face, hand and limbs (thin arrows). Thick arrow showing pitting edema over the left leg. Figure 2. Photograph of patient (a) showing abdominal distention (free fluid was confirmed by ultrasonography). X-ray pelvic bone (b) showing multiple sclerotic lesion over right iliac crest (thin arrow) and one large osteosclerotic lesion (thick arrow) over the neck of left femur (b). Figure 3. Nerve conduction study showing axonal and demyelinating neuropathy in right median nerve. Figure 4. Bone marrow smear in centre reveals one large atypical plasma cell, which has prominent nucleoli and abundant cytoplasm. There is loss of normal nuclear configuration with fraying border. References Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M proteins and skin changes: the POEMS syndrome: Report on two cases and a review of the literature. Medicine (Baltimore). 1980;59:311-322. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101(7):2496-2506. Min JH, Hong YH, Lee KW. Electrophysiological features of patients with POEMS syndrome. Clin Neurophysiol. 2005;116(4):965-968. Kelly JJ Jr, Kyle RA, Miles JM, O’Brian PC, Dyck PJ. The spectrum of peripheral neuropathy in myeloma. Neurology. 1981;31:31-34. Reulecke MD, Dumas M, Merrier C. Specific antibody activity against neuroendocrine tissue in a case of POEMS syndrome with IgG gammopathy. Neurology. 1988;38:614-616. DSouza A, Hayman SR, Buadi F, Mauermann M, Lacy MQ, Gertz MA, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood. 2011;118(17):4663-4665. Watanabe O, Maruyawa I, Arimura K, Kitajawa I, Arimura H, Hanatani M, et al. Overproduction of vascular endothelial growth factor vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve. 1998;21(11):1390-1397. Miralles GD, O’Fallen JR, Talley NJ. Plasma cell dyscrasia with polyneuropathy; the spectrum of POEMS syndrome. N Eng J Med. 1992;327:1919-1923. Kanai K, Sawai S, Sogawa K, Mori M, Misawa S, Shibuya K, et al. Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome. Neurology. 2012;79(6):575-582. Soubrier M, Dubost JJ, Serre AF, Ristori JM, Sauvezie B, Cathebras P, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum. 1997;40:786-778.

The Day My Music Died :: Personal Narrative, Autobiographical Essay

Some memories never fade. Tucked away in the accordion file of our minds, some memories live forever. Some memories walk hand in hand with us to our six-foot retirement homes. And though it is true that some are great blessings, some memories scar the hopes, some memories mar our dreams. In my heart I will always have a passion for music. The rhythm of the beat is the blood in my veins. I can close my eyes and hear the notes, tasting the sweet sound they leave on the top of my tongue. The sound of my soul, that reverberates inside the corridors of my heart. I feel empty without its presence, naked without the silken cloth of sound. This is my life; this is my true love. But love does not pay the bills, my father would say to me, loosening his tie and kicking off his leather shoes, those tight leather shoes. I like my sandals, I thought to myself, as I had so many times before. After all, thinking seems to be the only thing to do. I couldn't speak; I couldn't put that replenishing sound into his heart. Hell, thinking was the only cure. It sure beat hearing another lecture on the myopia of my dreams. Really, I was tired of it all. Every time I tried to tell my father about the passion, the sheer energy that music provided for me, my words fell on deaf ears. Listening, he would cross his arms and nod his head in that patronizing way. Soon, I could no longer distinguish the true meaning of any of my father's gestures. In our home, a nod no longer meant yes; a smile no longer meant happiness. As soon as my father realized that I was serious about being a musician, his world and mine collided head on. Father always believed in the importance of politics. He lived his life based on the philosophy that it isn't what you do, but who you know that really matters in the world. I still cringe at the thought of such an idea. I was never the one to go after people. I wasn't the type of person who you would interview when composing a "How to Win Friends and Influence People" book. If for some reason I didn't feel like grinning like a birthday boy upon making the acquaintance of a friend of my fathers, then that was that.

First Draft

Moreover, it saves time and healthcare expenditure. This essay aims to discuss the comparisons between two projects, in Cambodia-partners Telekinetic project and a remote volcanic group of Islands In South Atlantic Ocean-Telemeter comes home. To begin with, recent studies illustrate that there are many benefits of using telekinetic, especially for the least developed environments like Tristan dad Chunk and several distant places in Cambodia. It can be used effectively via 24-hour satellite-internet connection in Tristan De Chunk; first of all, It Is helpful for doctors to monitor patients' conditions and even for surgery.According to a foreman of medication at IBM named Richard Baklava, â€Å"What we are starting to see now is a patient-doctor model. † It is presently ordinary that physicians send patients' information such as X-rays in a computer program version to other experts. For example, If Dry. Van De Mere requires advising specialists, he can function It over a video l ink. As the technology amends, a mechanical-liked operation system called Dad Vinci was used to remove a gall-bladder on a patient in the year of 2001. (The Economist, 2008, p. 3) In Cambodia, telekinetic is typically used In provinces In some resource-poor areas the same as In South Atlantic Ocean, too. The Cambodia villagers, especially those who live In Roving and Ban Lung, have been very grateful for the provision of internet access. To be able to send and obtain data, the local populations need solar and generator- powered satellite dishes, wireless technology and mobile systems to approach. Furthermore, telemeter mostly works on some sicknesses like malaria and diarrhea that are likely to be occurred on kids in the remote Cambodia.On the other hand, without the motormen who take responsibilities on delivering patients' information to physicians, it is almost impossible for the villagers to know what recklessly goes wrong with their health. (Hellmann, 2005, Internet). In other words, with the aids of the internet, telekinetic has cured and saved so many people's lives. Taking the ill patients at Roving in Preach Viewer province as an example, if they want to visit the doctors at Phonon Penn city, they have to spend time and fees on traveling to receive better treatments or Just to have simple health check-ups.As regards to a Cambodia report, It states that almost 80% of the residents derived a great satisfaction from the resistance involved and that they would be willing to foot 1 OFF hey are not required a direct visit to the specialty hospitals. (Heinlein, 200, internet) Similarly, Tristan dad Chunk, does not have an airstrip to provide clinical heath care for urgent situations, therefore, it would be a difficulty to contact other places in the world face to face in approximately one week.

The American Presidency essays

The American Presidency essays The Power Of the American Presidency The office of the American President is unquestionably a democratically ruled position. The checks and balances of the Legislative and Judicial branches, known as Separation of Powers, keep the presidency one of the most important duties in the world, but at the same time, the checks and balances do not let the president have too Separation of power was very effective; The three branches of government (executive, legislative, and judicial) are kept separate, and each has different powers. Congress has legislative, or law making, powers; the President has the power to carry out, or execute, the laws; and the Judicial branch had the judging power, used to interpret the laws. In addition, each branch is able to restrain or balance the powers of the other two branches upon power abuse. If the President is suspected of unlawful acts, he can be impeached, or tried by the House and Senate for misusing his power. If he is found guilty, he can be thrown out of office, unless two thirds of Congress agrees with a treaty he proposes. Furthermore, if the President wants to spend money, his request must pass through Congress, since it has control over spending. Lastly, Congress can re-pass a vetoed bill. Congress also has checks and balances against itself. The president can veto a bill from Congress, and although Congress can override a veto, obtaining a two-thirds vote is very difficult. Public speeches by the President may also concern the public with an issue, putting pressure on Congress to The American President is one of the most important persons on earth. The president, however, cannot pass a single bill without the approval of the House and the Senate. The president can propose bills, and he often deals with many short, yet unthinkably important questions concerning various factors throughout the world. ...

Evaluating You and Your Business Idea a Book by Joseph Covello and Brian Hazelgren

Evaluating You and Your Business Idea a Book by Joseph Covello and Brian Hazelgren The paper is aimed at examining the chapter Evaluating You and Your Business Idea from the textbook written by Joseph Covello and Brian Hazelgren. These authors pay attention to the process of goal setting, and they offer some valuable recommendations to future entrepreneurs.Advertising We will write a custom essay sample on â€Å"Evaluating You and Your Business Idea† a Book by Joseph Covello and Brian Hazelgren specifically for you for only $16.05 $11/page Learn More On the whole, these researchers believe that an individual should identify personal and economic objectives that should be attained (Covello and Hazelgren 13). In other words, it is necessary to examine the financial objectives that should be achieved by the enterprise. Moreover, it is vital to quantify these economic objectives (Covello and Hazelgren 13). Furthermore, a person should explain why he/she wants to work in a specific area of business. Moreover, it is vital to explain how t his particular choice can help a person fulfill his/her talents or ambitions. To some degree, these goals included in this plan are consistent with the recommendations offered by these authors. In particular, this plan includes clear economic objectives that are quantified. However, it is necessary to pay more attention to personal objectives because they are not clearly identified. This is one of the drawbacks that can be singled out. Additionally, it is important to discuss the distinction between short and long-term objectives. This distinction is important for the successful implementation of business plans. In this way, one can better develop the strategies for the development of a business (Covello and Hazelgren 13). Furthermore, this knowledge can help a person see the relations between different steps that will be taken in the future (Covello and Hazelgren 13). The given plan includes some of the goals that will be attained in the near future. Moreover, it is critical to sin gle out the resources that should be accumulated. For instance, the author of this plan discusses the need to accumulate start-up capital. Apart from that, this plan includes some long-term objectives. In particular, the writer mentions the intention to open new shops within the next four years. Furthermore, Joseph Covello and Brian Hazelgren believe that it is necessary to set the timelines for the implementation of the goals (15). However, these researchers also note that in many cases, such timelines cannot be rigidly fixed. Moreover, by focusing only on timelines, a person can make flawed business decisions. This is one of the pitfalls that should be avoided. To a great extent, the plan, which is analyzed, is compatible with the suggestions offered by the authors of this chapter. Nevertheless, there are some limitations of this plan, and they should not be overlooked. For instance, this document does not clearly show how this venture can gain the loyalty of clients. It is not ev ident why they will choose the services of this particular cafà ©. In turn, this issue is examined by Joseph Covello and Brian Hazelgren who believe that entrepreneurs should differentiate the venture among other companies. This is one of the issues that should be taken into account.Advertising Looking for essay on business economics? Let's see if we can help you! Get your first paper with 15% OFF Learn More On the whole, the discussion suggests when starting up a business venture, a person should clearly explain the steps that will be taken to attain business goals. Moreover, one should clearly identify the resources that will be needed. The elements of this plan are compatible with the recommendations offered by scholars. Nevertheless, the author does not show why the future venture will be remain competitive. These are the main arguments that can be advanced. Covello, Joseph, and Brian Hazelgren. â€Å"Evaluating You and Your Business Idea.† Comp lete Book of Business Plans: Simple Steps to Writing Powerful Business Plans. Ed. Joseph Covello and David Carpenter. New York, NY: Sourcebooks, Inc, 2006. Print.

Review of the Literature 3 Essay Example | Topics and Well Written Essays - 1250 words

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Exporting goods via airfreight Essay Example | Topics and Well Written Essays - 500 words

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