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.

Ethics Scholarship Essay Essay

One ethical issue to which I became aware of while in college mainly involved cases illegal drugs, bribery, and cheating. Although there is an existing law against illegal drugs and most schools and universities are very stringent in imposing penalties for illegal activities and other things that are criminal in nature, sadly there are still some students who find ways to break those rules, especially outside the campus. While I am not the one involved in these illegal activities, I felt that I had a moral obligation because two of my classmates were involved. These classmates of mine were actually good people, but unfortunately, they ended up with the wrong company. It took place not very long ago, during a house party held by one of the rich students in the university. This certain student, whom I personally know, constantly held parties in his/her lavish house. My two classmates were not invited because the host of the party didn’t like their introvert nature. They were very shy, had very little friends, and were only interested in playing video games and the like. Nevertheless, as I have mentioned above, they were people who possessed values. The problem is they just lacked social skills. One day in class, I noticed that they were talking to a guy who was a notorious rule breaker in the university and was one of the friend of the rich person mentioned earlier. Later, I found out that they were invited to one of the parties held by the rich person and they were both very excited to go as it was their first time to have the change to mingle with a lot of people. On the night of the party, I was to see them talking to a couple of girls and boys. Although I do not wish to judge them, I was a bit confused because, as mentioned above, they were very shy and were not the type of people who would approach someone for a casual conversation. I felt happy for my two classmates but I noticed that certain things about them started to change in the succeeding months. There were instances that they were absent from class, which was something they never did, and there were also times that they were asleep during a lecture or an exam. After some time, I spoke with one of the two and found out that the reason behind their unusual behavior was because of illegal drugs. This person further revealed that they were invited to the rich student’s party on the condition that they would do the assignments of a group of people who were close to of him/her (rich student). This classmate of mine also told me that they were doing drugs because it was considered â€Å"cool† among their new circle of friends. However, the main problem was that both these classmates of mine were nearly failing a lot of their subjects due to their absences and neglect. Because I was concerned for them, I sought the help of fellow students who were also concerned and together we notified the university officials on condition of anonymity. Although it was the right thing to do, I had an ethical dilemma because as a result of my actions, my two classmates were indefinitely suspended from school and were taken to a rehabilitation facility. I was very bothered at first because in a way, I cost them a lot by revealing their secret illegal activities. But after several months, I realized that it was the only thing to do because I didn’t want their future to be ruined by drugs. Basically, this event has impacted me in such a way that I carefully choose whom I associate with. Although I have a lot of friends, I only trust people whom I am sure would not give in to negative influences and to peer-pressure from the wrong ground. It also made me more vigilant and more careful of people who are trying to be nice to me but actually have ulterior motives. On the business perspective, it made me more cautious in dealing with alleged â€Å"easy money† gimmicks and deals which are perpetrated by people who have questionable backgrounds. In short, it made me wiser and clever in terms of business dealings because now I am more aware of the capabilities of certain people who only claim to offer good deals but in truth, do not offer anything of value at all.

What Is So Fascinating About The Other World Essay Topics?

What Is So Fascinating About The Other World Essay Topics? Inspiration to make your own advertising or media argumentative essay topics isn't tricky to discover. The point is that someone with a wide ranging vocabulary needs to be in a position to chat about any of the typical essay topics easily using words that are particular to that topic. On the opposing side, acquiring a list of good persuasive essay topics is insufficient. It's important to select debatable argumentative essay topics since you need opposing points you may counter to your own points. Comparative essays are a breeze to write. As you have observed, different forms of essay topics have various strategies to approach them, and in addition, they have their own benefits. Before writing the essay, it's important to pick an intriguing topic. Researching the topic will permit you to find out more about what fascinates you, and should you pick something you truly like, writing the essay will be more enjoyable. If you wish to compose the essay yourself, we believe it would be best to select a universal subject or issue. You don't need to find super technical with legal argumentative essays, but make sure you do your homework on what the recent laws about your preferred topic actually say. You should research the selected topic and discover facts to contradict your primary thesis. Therefore, the topic needs to be debatable! A History of the Other World Essay Topics Refuted Mention similarities and the way both ideas differ. So now you are aware of how to select the best compare and contrast topics and the various segments which you need to address when writing. A far better thing to do is to begin a vocabulary notebook. There is an assortment of genres of prompts that will motivate you to finish a great academic paper. The Do's and Don'ts of the Other World Essay Topics You don't necessarily have to analyze the life span of people from precisely the same region or field of activi ty. Well, different individuals have various interpretations of the way the game ought to be played. For example an individual may pick a topic like, life in the shoes of a werewolf. F. Bacon The main goal of the capital isn't to get as much money as possible, yet to make sure that money results in a better life. Your argument needs to be based on your own opinion. A conclusion is, without a doubt, the most essential portion of the argumentative essay since you can either support the great impression or destroy it entirely. It's possible for you to restate your thesis statement and point out a number of the arguments used over the full essay that backs it up. Afterwards, the greatest action to do is to narrow the subject down according to the guidelines supplied by your professor. As an example, in college, you might be requested to compose a paper from the opposing standpoint. It is possible to also order a fully written compare and contrast essay and alleviate the quant ity of work you must do. On the flip side, in case you made a great choice, focus on what influenced you to make that decision and the way it has changed you. On the flip side, it discourages the revolutionary global system. You also know how to discover sources and the ideal kind to utilize in your paper to ensure it is relevant and interesting. Our on-line essay editing service gives a good value for the money since you can receive the best grades without sweating over it. In this kind of situation, it's more convenient to locate ready-made essays and use them as an example. You may continue to keep your argumentative essays for your upcoming job portfolio in case they're highly graded. If you still need more information, you can have a look at the world wide web, but in case you already understand what things to do, you should make sure that you get started writing your essay as early as possible so you have more time to proofread and edit it. If you're confused with a selection of interesting topics to research online, it's much better to choose what interests you the most. You may use a variety of examples on the world wide web free of charge. Decide on a law and explain why it's so important to you. The Argument About the Other World Essay Topics As stated above, one of the means where the examiner judges your vocabulary is the way many topic particular words you use. There are many sources from where it is possible to gather information on your subjects but make certain you always go with facts. In most instances, the topics you pick should be closely related. If you're going to succeed in the IELTS test you must be using and exposing yourself to English everyday. Other World Essay Topics Features The majority of the questions are on current topics, therefore staying current with what's going on in the world is really going to help you think of ideas. You will find that lots of the topics can be adapted to suit almost any type of writing assignment. If you still find it tough to work on your expository essay, get in touch with our on-line writing team made from professional writers with 10 or more decades of experience to acquire your own personal academic life buoy! Bear in mind you can make funny argumentative essays if you do a few things. Introduction with the effective hook and thesis statement stays the same. Naturally, writing is among the major learning programs in any significant school institution. There are two major means by which you can use the most typical IELTS essay topics to your benefit. To compose an outstanding essay, it's critical to use specific academic language.

Does Race / Ethnicity Impact Pay Inequity - 1805 Words

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