Rosai-Dorfman Disease with Cervical Lymphadenopathy

Rosai-Dorfman Disease with Cervical Lymphadenopathy Rosai-Dorfman Disease with Cervical Lymphadenopathy and Orbital Involvement: A Case Report *Sameer Saleem 1, Sundas Younas 2, Kamran Qayyum 3 1 MBBS (2013), Khyber Medical College Peshawar, Pakistan 2 MBBS (2013), Khyber Medical College Peshawar, Pakistan 3 MBBS (2014), Khyber Medical College Peshawar, Pakistan ABSTRACT Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare histiocytic disorder which occurs due to the over-production of non Langerhans sinus histiocytes. It is a nonmalignant disorder that most frequently affects children and young adults and typically presents with fever, night sweats, nonpainful cervical lymphadenopathy, leukocytosis and an elevated ESR. Extranodal involvement may also occur which includes skin and soft tissues, nasal cavity, orbit, bones, central nervous system, salivary glands, kidneys, respiratory tract and liver. The digestive tract, heart and breast can also be affected but very rarely. The disease has an unknown etiology, although some viruses like Human Herpes virus 6 and Epstein Barr virus have been implicated as causative agents. RDD can often be misdiagnosed as lymphoma, leukemia or tuberculosis, so it is imperative to distinguish it from these conditions as well as other causes of histiocytosis becaus e of the different treatment modalities. Diagnosis of Rosai-Dorfman disease is based on biopsy of affected tissue. Biopsy showing the presence of emperipolesis, or the engulfment of lymphocytes and other immune cells by histiocytes that express S-100 antigen is diagnostic of Rosai-Dorfman disease. Once diagnosed, further workup including imaging studies are undertaken in order to determine the extent of the disease. In majority of cases, the disease resolves on its own however, treatments including corticosteroids, chemotherapy, surgical treatment or radiotherapy are carried out in severe or persistent disease or when organ function is at stake (e.g. breathing obstruction, kidney failure, visual problems). The case we report is that of a 16 year old girl who presented with a 6 month history of gradual onset drooping of left upper eyelid with mild proptosis of the left eye alongwith mild drooping of right upper eyelid, low grade fever, night sweats and cervical lymphadenopathy. Blood workup showed increased ESR, CT scan of orbits showed superior orbital masses and diagnostic biopsy revealed Rosai-Dorfman disease. Keywords: Rosai Dorfman disease, cervical lymphadenopathy, emperipolesis, histiocytes, proptosis CASE REPORT A 16 year old girl presented to the outpatient department of Khyber Teaching Hospital, Peshawar, Pakistan in February 2015 with a six month history of gradual onset, painless left upper eyelid drooping alongwith low-grade fever and night sweats plus a 20 day history of gradual onset right upper eyelid drooping. She also noticed a few lumps in her cervical region. She had no significant past medical history of any major illness and no family history of tuberculosis or blood disorders was found. Her vitals were as follows, BP 120/80 mm Hg, pulse 90/min, respiratory rate 15/min and temperature 100.2 ÃÅ'Ã…   F. On examination she had bilateral painless cervical lymphadenopathy and bilateral superior orbital masses on palpation. Her vision was 6/6 in both eyes. Ptosis was seen in both right (3mm) and left (5mm) eyes. Mild left eye proptosis was also seen. Extraocular movements were restricted in upper gaze of both eyes, more so of the left eye. There was no evidence of any visceromegaly and the rest of the general physical and systemic examinations were unremarkable. Lab investigations: Hb 11.1 g/dl, RBC 4.27 million/cmm, Hct 32.4 %, MCV 76 fl, MCH 25.9 pg, MCHC 34.2 g/dl, Platelet count 328000/cmm, TLC 11000/cmm, normocytic normochromic picture with DLC showing 80% neutrophils, 15% lymphocytes and 5% monocytes on peripheral smear, ESR 70 mm/1st hour, negative HbS and HCV screening, negative PPD and sputum AFB, normal Liver function tests and normal Renal function tests. Chest X-ray was normal, U/S and CT scan of the abdomen and pelvis were normal. CT scans of the orbits showed bilateral superior orbital masses and mild proptosis of the left eye. Incisional biopsy of the left Superior orbital mass was performed that revealed the diagnosis of Rosai-Dorfman disease. CT scan orbit (Coronal View): Bilateral Superior Orbital Masses CT scan Orbit (Axial View): Mild Proptosis of the Left Eye Treatment: The patient was counseled about the nature of the disease and administered Inj. Methylprednisolone 1gm x OD for 3 days followed by Tab Prednisolone 1mg/Kg body weight x OD and advised followup after 4 weeks. On followup visit, examination showed that her ptosis and cervical lymphadenopathy had improved. She was also assessed for side effects of steroid therapy. No side effects were noted. She was advised followup after 8 weeks. DISCUSSION Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare histiocytic disorder which occurs due to the over-production of non Langerhans sinus histiocytes. [1, 2] It was first described as a unique clinicopathologic entity by Rosai and Dorfman in 1969. [3] Although lymph nodes are more commonly involved, any organ may be affected. [1] Cardinal features include painless cervical lympahadenopathy, fever and elevated ESR. [4]Extranodal involvement has been reported in diverse anatomic sites, particularly the skin, orbit, and upper respiratory tract. [5, 6] Central nervous system involvement without nodal disease has also been reported. [7] Rosai-Dorfman disease though quite rare, is distributed worldwide with 80% cases occurring in children and young adults with a slight male predominance (58%) and has a general predilection for individuals with African descent. [8] The etiology of RDD is unknown, however certain viruses like Human Her pes virus 6 and Epstein-Bar virus via causing immune dysregulation have been implicated in the pathogenesis of this disease. [9, 10, 11] The diagnosis of Rosai-Dorfman disease is not easy since its presentation can mimic a number of other non-malignant as well as malignant conditions ranging from bacterial or viral infections to malignancies including leukemia and lymphoma. Biopsy of the lymph node or affected tissue is required for the diagnosis of this disease. Proliferating S100 and CD68 antigens positive histiocytes exhibiting emperipolesis i.e. phagocytosis of intact lymphocytes and other immune cells, is the classical histologic finding on biopsy in Rosai Dorfman disease. No specific treatment protocol is established for Rosai Dorfman disease because the disease is rare and its course is mostly self limiting. [14] However, patients with severe, persistent disease or in cases where organ function is compromised steroid therapy, chemotherapy, surgical resection or radiotherapy can be instituted with varying success rates. [12, 14, 15] CONCLUSION Rosai-Dorfman disease shares many of its presenting features with leukemia, lymphoma, tuberculous lymphadenitis and other causes of histiocytosis, so it should be considered in the differentials of patients, especially children and young adults who present with painless cervical lymphadenopathy. It is also important for physicians to recognize that the disease can have a myriad of clinical manifestations depending upon the tissue involved, as was the case in our patient we presented in this case report having cervical lymphadenopathy with orbital involvment in whom lymphoma, leukemic deposits, orbital pseudotumor, Langerhan’s cell histiocytosis and hemangioma were also amongst the list of differentials until biopsy confirmed the diagnosis of RDD. It is essential for pathologists as well to look for the histopathologic features of this disease in biopsy specimens, since if promptly diagnosed and managed, can reduce unnecessary diagnostic workups and mismanagement due to misdiag nosis of this disease. CONSENT Written informed consent was taken from the patient and her parents for the publication of this case report and any accompanying images. ABBREVIATIONS AFB Acid Fast Bacilli BP Blood Pressure CT Computed Tomography DLC Differential Leukocyte Count ESR Erythrocyte Sedimentation Rate Hb Hemoglobin HbS Hepatitis B surface antigen HCV Hepatitis C virus Hct Hematocrit MCH Mean Corpuscular Hemoglobin MCH CMean Corpuscular Hemoglobin concentration MCV Mean Corpuscular Hemoglobin PPD Purified Protein Derivative RBC Red Blood cells RDD Rosai-Dorfman Disease SHML Sinus Histiocytosis with Massive Lymhpadenopathy TLC Total Leukocyte Count References Riyaz N, Khader A, Sarita S. Rosai-Dorfman syndrome.Indian J Dermatol Venereol Leprol.2005;71:342–4. James, William D.; Berger, Timothy G.; et al. (2006).Andrews Diseases of the Skin: clinical Dermatology. Saunders Elsevier.ISBN0-7216-2921-0 Kong Y, Kong J, Shi D, Lu H, Zhu X, Wang J, Chen Z:Cutaneous Rosai–Dorfman Disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol2007,21:341-350. Foucar E, Rosai J, Dorfman R: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a review of the entity. Semin Diagn Pathol 1990; 7:19-73 Puppin D Jr, Chavaz P, Harms M: Histiocytic lymphophagocytic panniculitis (Rosai-Dorfman disease): a case report. Dermatology 1992; 184:317-320 Andriko JW, Morrison A, Colegial CH, et al: Rosai-Dorfman disease isolated to the central nervous system. A report of 11 cases. Mod Pathol 2001; 14:172-178 Woodcock RJ, Mandell JW, Lipper MH: Sinus histiocytosis (Rosai-Dorfman disease) of the suprasellar region: MR imaging findings a case report. Radiology 1999; 213:808-810 Sodhi KS, Suri S, Nijhawan R, Kang M, Gautam V:Rosai–Dorfman disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy. Br J Radiol2005,25:845-847. Ensari S, Selcuk A, Dere H, Perez N, Dizbay Sak S:Rosai–Dorfman disease presenting as laryngeal masses. Kulak Burun Bogaz Ihtis Derg2008,18:110-114. Pinto DCG, Vidigal TA, Castro B, Santos BH, DeSousa NJA:Rosai–Dorfman disease in the differential diagnosis of cervical lymphadenopathy. Bras J Otorrinolaringol2008,74:632-635. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES:Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease). J Infect Dis1992,166:291-295. Yoon A, Parisien M, Feldman F, Young-In Lee F:Extranodal Rosai–Dorfman disease of bone, subcutaneous tissue and paranasal sinus mucosa with a review of its pathogenesis. Skeletal Radiol2005,34:653-657. Montgomery EA, Meis JM:Rosai–Dorfman disease of soft tissue. Am J Surg Pathol1992,16:122-129. Pinto DCG, Vidigal TA, Castro B, Santos BH, DeSousa NJA:Rosai–Dorfman disease in the differential diagnosis of cervical lymphadenopathy. Bras J Otorrinolaringol2008,74:632-635. Moore J, Zhao X, Nelson E:Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) and diffuse large B-cell lymphoma: a case report. J Med Case Reports2008,2:70.

Bosu Balance Trainer Essay

BOSU has used a few types of competitive advantage`s principles in order to defeat copycat products. BOSU created a new product and locked in customers and buyers by establishing alliances with trainers. BOSU`s marketing strategy created market entry barriers. 2. Information systems played a key role in BOSU`s success. Fitness Quest maintains a database of trainer data. It uses that database for email and postal correspondence as well as for other marketing purposes. Fitness Quest was crucial in the process of BOSU developing a successful marketing strategy. By Fitness Quest database of trainer data, BOSU was able to establish alliances and lock in consumers and buyers. 3. There are many ideas that could be used by Fitness Quest in order to develop its information systems. The main idea would be to help to strengthen ties between customers and between costumers and trainers. Trainers could share ideas about fitness classes for example. There could be also an available chat for customers, so BOSU buyers could participate in chat groups about how they like and use their BOSU trainers. 4. Through trainers, BOSU was able to differentiate. BOSU`s focus was on fitness trainers within the industry. This worked because the trainers believed the BOSU was the best product for giving customer value, which built a relationship between each customer and the product. 5. 1.enhance products and services 2.differentiate products and services 3.lock in customers and trainers 4.raise barrier to market 5.entry and establish alliances 6. There are many differences between BOSU and Indo-Row since those are two completely different products with two different goals. BOSU product focused on balance and Indo-row focused on total work out (it competes with other equipment-based forms of group exercise as Spinning). Indo-row is also more expensive than BOSU and there is no direct competitor since the product is new in the market. The main threat Indo-row and BOSU may face is the possibility of competing with a copycat product in future. 7. It would be already expected from Fitness Quest to send the Indo-row information to trainers and clients by database provided through email and mail. It would be also interesting to provide seminars and training for trainers in order to instruct them how to use the product (so they would be able to teach people how to get the best out of Indo-row). Customers and trainers group chats would be also a good idea, so they would be able to interchange opinions about the product.

What Everybody Else Does When It Comes to Customresearchpapers Essay and What You Need to Be Doing Different

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Evaluation Of Air Pollution Of Bangkok, Thailand - 1508 Words

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Bangkok’s transport system and causing pollution problems are unique, such as Bangkok has 4,075 kilometers of roads with a total area of 58.45 km2, but even then, an increasing number of vehicles in Bangkok has not been matched by a proportional increase in road construction. Thus leading to traffic congestion, added more pollution caused by transport delay. The Pollution control Department and Thai Environment Institute (Boontheerawaraet al., 1994) reported that currently issue of air pollution became a major environmental problem of Thailand. Emissions of particulate from traffic, carbon monoxide, sulfur dioxide nitrogen oxides and ozone have been increasing gradually over the past few decades. 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Capital Punishment And The Death Penalty - 967 Words

Capital punishment, death by execution, or the death penalty is a government sanctioned punishment. Crimes that result in the death penalty are known as capital crimes or capital offences. Today the death penalty is viewed quite different throughout different countries and states. But is the death penalty worth it by all means? If a person is already aware of the possible outcomes for committing a crime, is that going to stop them? Usually once a course of action has so much time spent planning it, there is no turning back. If they are willing to take such a high risk for something that seems rewarding to them, these criminals will not care about what comes after because they do not intend on getting caught. First reason why the United States should drop the death penalty is the costs in keeping that prisoner and the case prices. Cases that do not involve the death penalty cost on average seven hundred and fifty thousand. Cases that involve the death penalty cost upwards of one milli on dollars. But the extra costs don’t stop there, each person inducted onto death row costs tax payers an extra ninety thousand dollars a year. Just think, all that extra money that is wasted on the execution could be given to the victim’s family to help pay for the funeral and future expenses. While that money will not bring back their loved one, it can ease some of the problems after. The second reason for dropping the death penalty is crime rates throughout the United States. The thought ofShow MoreRelatedCapital Punishment : The Death Penalty1482 Words   |  6 PagesMrs. McElmoyl 12/12/14 Capital Punishment As stated by former governor of New York, Mario M. Cuomo, Always I have concluded the death penalty is wrong because it lowers us all; it is a surrender to the worst that is in us; it uses a power- the official power to kill by execution- that has never brought back a life, need inspired anything but hate. 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Nabeel Tahir Free Essays

It is indeed with great pleasure that I recommend Mr. Muhammad Shoaib to pursue his Master of Electrical Engineering. I have been associated with the Department of Electrical Engineering for the past 4 years and in that course of time had the opportunity to interact and assess many students who have passed through the portals of this Department. We will write a custom essay sample on Nabeel Tahir or any similar topic only for you Order Now I have been in contact with Mr. Muhammad Shoaib ever since his 3rd year of engineering when he attended the course of Control Systems which I taught. From day one, I have seen many examples of his talent and have long been impressed by his diligence and work ethic. He is highly motivated and his apparent enthusiasm and interest in the subject is supported by his diligent work. He is smart, a high achiever, and eager to learn. He has always shown an extraordinary sense of perception of the subjects, and has made a difference to the classes with his insightful remarks. In all his course activities, his grades have been above average and he stands in the top 3% of her class. Now, being our faculty member he is teaching Control Systems theory course with me to the undergraduates of electrical engineering. This speaks to his overall intellect and ability to learn, attributes that will serve him well in graduate study. Mr. Shoaib actively participated in extra-curricular activities also. He was an active member of the Electronic Club and Social Secretary of IEEE, UET Lahore Section. On inter-personal level too, Mr. Shoaib has endeared his self to his class-mates with his kind and compassionate nature. Inasmuch as these qualities, he is elected as Joint Secretary, Teacher Staff Association at UET Lahore for the year 2010. His combination of intelligence, commitment, perseverance, creativity, and compassionate character will certainly make him a valuable member of any academic program. It is for these reasons that I offer high recommendations for Shoaib without reservations. His drive and abilities will truly be an asset to your institution. I encourage you to look favorably upon his application. If you have any questions regarding this recommendation, please do not hesitate to contact me. How to cite Nabeel Tahir, Essay examples

Internet Company Speed Connect Pty Ltd. Hamid Speaks

Question: Hamid is a refugee from Afghanistan who was recently released from the detention centre in PNG and is currently living in Adelaide. While walking in Rundle Mall one day he was approached by Kathy who was selling internet access contracts for a newly formed internet company Speed Connect Pty Ltd. Hamid speaks very little English and Kathy is aware of this. Kathy explains the terms of the contract which is essentially that Hamid would be bound to a 2 year contract and if he breaks the contract anytime before the 2 years he will incur a $1,200 penalty. The contract comes with a free Samsung tablet. Kathy uses some technical language to explain the terms to Hamid, aware that he doesnt understand the full extend of the contract terms.There is a telephone translation service available but Kathy chooses not to use this as this translation service usually takes twice as long. Kathy works on a commission basis. The more contracts she sells the more she earns. She wants to sign up this contrac t quickly as she is keen to sign up three more contracts before close of business. Hamid has been thinking of getting internet connection as he wishes to Skype his family back home in Afghanistan and also to do a free on-line English course. He is under the impression that he can terminate this contract anytime he wants without any penalty and all he has to do is give Speed Connect one months notice and return the tablet. Three months into the contract Hamid finds that he is unable to cope with the financial demands of this contract as there are many hidden costs which he was unaware. When he contacted Speed Connect to give his termination notice he was shocked to learn that there is a $1500 penalty. Advise Hamid if this contract with Speed Connect is valid? Can he get out of this contract? Answer: The Australian Contract Act. Issue In the given case law Mr. Hamid is from the country Afghantistan who had been detained but now released from PNG and is residing in Australia in the city of Adelaide. He was being approached by Mrs. Kathy for a new internet connection while he was out for the tour at the mall. These contracts were on behalf of the new internet company named as Speed Connect Pty Ltd. The terms of the contract was being demonstrated to humid in English which was a lest known language to him and this was in the knowledge of Kathy and she also added the 2 year block period and the penalty clause in the discussion of offering the internet connection. There was also the use of some of the technical language which was not suited to the Hamid as he was not aware of these terms as well. The process also included the rejection of the professional services when the There is a telephone translation service available but Kathy chooses not to use this service because of its time consuming nature and not necessary nature as told Kathy to Mr. Hand. 1. Need Of Hamid In the Issue The issue is that whether Mr. Hamid is justified in cancelling the contract before the expiry of 2 years of contract without any levy of penalty clause. Hamid as the breach egad done on no misreprenatation is being saved and the whole house to be vest as the complete disclosure of hidden costs were not made known to him. The issue pertaining in this regards is that whether Mr. Hamid can get out of the contract without any penal provision. The main issue that can be discussed is the condition of the breach of contract and thus would help in the understanding of the issues which lead to the breach of contract for a company. There are two types of breach namely the actual breach and the anticipatory breach and thus it would help in the decision of understanding the conditions leading to the breach of contract by the parties. (A)RULES APPLICATION: If Mr. Hamid wants to be justified in rescinding the contract there must me failure of the contract. It can be said that there It may be argued that prima facie there was a repudiation of the contract as Mr. Hamid was acting honestly and did not intend to repudiate his obligations if the seller Ms.Kattyhad applied a correct procedure of applying the telephone translation method. Katy was aware of the fact that Mr. Hamid was not good at English enough to understand the technical terms of the contract. Makati could have followed the afore mentioned method to make Mr. Hamid clear about the terms of the contract but it would have taken twice the time to make him understand. It would be pertinent to note that Makati is a commission based seller which earns on the basis of quantum of selling internet access contracts. This is the case of Miss-representation under the instant circumstances. So we can prove it under the law as under: the statement shout should be a demonstrator of back and profiler attitude. The test to determine whether the statement is of fact is objective. The context in which the representation was made suggests that it is factual. Certainly the relative expertise and differential knowledge of Mr. Hamid is relevant. Misrepresentation does not actually is linked to the fact that someone persuaded a person but it is that the contract was entered because of the persuasion and then only the former person is guilty. Infect in the instant case also Ms. Katty was aware that Mr. Hamid had a very little knowledge of technical terms used by her in explaining the terms of the contract. She had an alternative to this that she should have opted telephone translation method to make him understand the relevant terms of the contract. The test lies in the fact that whether the representation was done on the ordinary course of contract and the terms of breach. Nicolas v Thompson Contract voidable The laws applicable would bring good conclusion for the case and will make the contract voidable from the conduct of one of the parties. The contract however cannot be rescinded as there is no base for the same to be rescinded of the contract in any given case. Accordingly, it can be inferred that Ms. Katty was misrepresented violated the normal provisions of the contract clauses. (B) CONCLUSION: Ms. Kathy has misrepresented the facts to Mr. Hamid as she could have opted the method of telephone translation to explain the terms more clearly to him but as this method takes twice the normal time, she did not opted that method as she had the primary objective of making consideration out of commission by selling more internet access contracts. Besides being aware of the fact that Mr. Hamid do not understand the technical terms, she still used such terms to sell the contract which made Mr. Hamid understand that he could get out of the contract even before 2 years without any penal provision of $1200. But after the expiry of 3 months from the date of entering into the contract, Mr. Hamid realizes that there were certain hidden costs a penalty of $1500 on getting out of the contract. Accordingly, it can be inferred that Ms Kathy was wrong in not opting the telephone translation method for selling the internet access contract. Hence, due to the misrepresentation the contract becomes voidable. The areas that need to be covered by the case law that all is moving right but where is the issue that resulted in the downfall of the company providing internet connection to the people the sale tagline is incorrect. Mr hamid should be told about the product when Miss Kathy approach she k know the angry age issue so she should I have complained The main fact to understand over here is the contractual obligations that would arise as a result of the breach of the contract over the time. The fact of the contract is that Mr Hamid is not thinking of taking steps over the issue such that positive results could be achieved by the parties to the contract. The results of the breach are generally that they is chances to cancel the contract, or compensate the other parties for the losses caused to him. So this is the conclusion for the case in detail. References https://articles.latimes.com/keyword/breach-of-contract https://www.nydailynews.com/entertainment/gossip/nyc-chauffeur-sues-taylor-swift-contract-breach-article-1.1956885 https://www.reuters.com/article/2014/08/23/us-cargill-lawsuit-idUSKBN0GN00V20140823 https://www.reuters.com/article/2014/08/23/us-cargill-lawsuit-idUSKBN0GN00V20140823