Long Term Follow-Up of Pediatric-Onset Evans Syndrome
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Evans Syndrome As Rare Presentation in Systemic Lupus Erythematosus
International Journal of Health Sciences and Research www.ijhsr.org ISSN: 2249-9571 Case Report Evans Syndrome as Rare Presentation in Systemic Lupus Erythematosus Dr Sabarish Mahalingam1, Dr P. Z. Wadia2, Dr Priyanka Lad1 1Resident Doctor, Department of Internal Medicine, Government Medical College, Surat 2Additional Professor, Department of Internal Medicine, Government Medical College, Surat Corresponding Author: Dr Sabarish Mahalingam ABSTRACT Evans syndrome is a rare disorder in which the body’s immune system produces antibodies that mistakenly destroy red blood cells, platelets and sometimes certain white blood cell known as neutrophils. It is one of the rare presenting features of autoimmune disorders, especially systemic lupus erythematosus (SLE), and sometimes may even precede the onset of disease. Primary Evans syndrome with no cause is very rare and is seen in children. Here, we describe a case of secondary Evans syndrome with severe autoimmune hemolytic anemia leading to acute kidney injury. This is one of the rare presentations of SLE and there are only few case reports. Key word: Evans syndrome, systemic lupus erythematosus, autoimmune haemolytic anaemia. INTRODUCTION literature; [9-11] therefore, the characteristics Evans syndrome (ES), which was and outcome of adult's ES are poorly first described in 1951, is an autoimmune known. disorder characterized by the simultaneous or sequential development of autoimmune CASE REPORT hemolytic anemia (AIHA) and immune A 28 aged female came to (ITP) and/or immune neutropenia in the emergency department with the complain of absence of any underlying cause. [1,2] ES has breathlessness for past 5 days. On been since its first description considered or examination, patient was pale and defined as an “idiopathic” condition and tachypneic, systemic examination was thus mainly as a diagnosis of exclusion, ES normal. -
Bacterial Skin Infections an Observational Study
RESEARCH Geoffrey Spurling Deborah Askew David King Geoffrey K Mitchell MBBS, DTM&H, FRACGP, is Senior PhD, is Senior Research Fellow, MBBS, MPH, FRACGP, is Senior Lecturer, MBBS, PhD, FRACGP, FAChPM, Lecturer, Discipline of General Practice, Discipline of General Practice, Discipline of General Practice, University is Associate Professor, Discipline University of Queensland. g.spurling@ University of Queensland. of Queensland. of General Practice, University of uq.edu.au Queensland. Bacterial skin infections An observational study Bacterial skin infections such as impetigo and boils are Background common, contagious, often painful, and have the potential to We aimed to determine the feasibility of measuring resolution rates of recur. They are caused by Staphylococcus aureus and bacterial skin infections in general practice. occasionally by Streptococcus pyogenes, and are transmitted Methods by skin-to-skin contact, fomite contact or contact with nasal Fifteen general practitioners recruited patients from March 2005 to carriers.1 In the United Kingdom, incidence of skin infections October 2007 and collected clinical and sociodemographic data at in children in 2005 was approximately 75 per 100 000.2 Skin baseline. Patients were followed up at 2 and 6 weeks to assess lesion infection rates are likely to be higher in warmer climates. The resolution. only Australian data we found were for one Northern Territory Results Aboriginal Medical Service (Danila Dilba), which recorded 7.5 Of 93 recruited participants, 60 (65%) were followed up at 2 and 6 per 100 consultations for localised skin infections.3 weeks: 50% (30) had boils, 37% (22) had impetigo, 83% (50) were prescribed antibiotics, and active follow up was suggested for 47% Suggested risk factors for impetigo include: household crowding, (28). -
Skin Cancer in the Immunocompromised Patient
Dermatologic Risks and Transplantation Allison Hanlon, MD, PhD Vanderbilt University School of Medicine Department of Medicine Division of Dermatology I have no relevant conflicts of interest to disclose. Dermatologic Risks and Transplantation • Acne • Folliculitis • Sebaceous hyperplasia • Overgrowth of hair • Infections – warts, molluscum contagiosum • Skin thinning and increased bruising • Skin cancer Folliculitis and Acne Folliculitis Sebaceous Hyperplasia Overgrowth of Hair Cyclosporine associated Gingival Hyperplasis Molluscum Contagiosum Verruca Easy Bruising and Skin Thinning Overview of Skin Cancer in SOTR • Clinical appearance of most common skin cancers • Risk factors for developing skin cancer • Skin cancer prevention • Multidisciplinary care Basal Cell Carcinoma Basal Cell Carcinoma Nodular Basal Cell Carcinoma Basal Cell Carcinoma Squamous Cell Carcinoma Squamous Cell Carcinoma Field Cancerization Immunocompromised patients at risk for metastasis Melanoma Nail Unit Melanoma Nodular Melanoma Melanoma Benign Seborrheic Keratosis Skin cancer is the most common malignancy in solid organ transplant recipients • Skin cancer accounts for 40% of malignancies in solid organ transplant recipients (SOTR) • 50% of Caucasian SOTR will develop skin cancer • Non-melanoma skin cancer (NMSC) > melanoma Euvrard S, Kanitakis J, Claudy A. Skin cancers after organ transplantation. N Engl J Med 2003;348:1681 Skin Cancer in SOTR • Squamous cell carcinoma (SCC) is the most common cutaneous malignancy in transplant patient • Basal cell carcinoma (BCC) is second most common skin cancer in transplant patient • Melanoma risk 3.6 times greater likelihood in SOTR Hollenbeak CS et al. Cancer 2005; 104:1962 than the general Euvrard S et.al. N Engl J Med 2003;348:1681 population Lanov E et.al. Int J Cancer. 2010;126:1724 Proposed Mechanisms Of Immunosuppression relationship to Skin Cancer Development • Direct carcinogenic effects of immunosuppression medications • Proliferation of oncogenic viruses • Reduced immune surveillance within transplant skin cancers Carucci et.al.PLoS One. -
Case Report Treatment of Severe Evans Syndrome with an Allogeneic Cord Blood Transplant
Bone Marrow Transplantation, (1997) 20, 427–429 1997 Stockton Press All rights reserved 0268–3369/97 $12.00 Case report Treatment of severe Evans syndrome with an allogeneic cord blood transplant E Raetz1, PG Beatty2 and RH Adams1 Departments of 1Pediatrics and 2Medicine, University of Utah School of Medicine, Salt Lake City, UT, USA Summary: began experiencing increased difficulty with mucosal bleeding, which prompted frequent platelet transfusions. At Immunosuppressive therapy is commonly used in the 4. years, he had a major gastrointestinal bleed, followed management of autoimmune disorders. As marrow- 1 month later by an intracranial hemorrhage. He required derived lymphocytes appear to play a key role in these transient ventilator support, but eventually regained full diseases, lymphoid ablation followed by replacement neurologic function. Direct (DAT) and indirect (IAT) with autologous or allogeneic stem cells may be a thera- Coombs evaluations were always 3+ positive. Due to the peutic option. We report a 5-year-old boy with severe severe and refractory nature of his disease, the option of Evans syndrome which consists of immune thrombocy- novel therapy with bone marrow transplantation was topenia and Coombs-positive hemolytic anemia. He was pursued. rendered into complete remission with marrow ablation HLA typing of the family, and a search of the unrelated followed by rescue with an HLA-identical sibling cord marrow donor registries, did not identify an appropriate blood transplant. He unexpectedly died 9 months donor, but DNA-based typing for HLA-A, -B, -DRB1 of following transplant from acute hepatic failure of the amniotic fluid of a sibling fetus, of 6 months gestational unknown etiology. -
Molluscum Contagiosum
Partners in Pediatrics, PC 7110 Forest Ave Suite 105 Richmond, VA 23226 804-377-7100 Molluscum Contagiosum Although molluscum contagiosum is a common skin rash in kids, many parents have never heard of it. The most important thing to know about it is that, for most children, the rash is no big deal and goes away on its own over time. About Molluscum Contagiosum Molluscum contagiosum is a viral infection that causes a mild skin rash. The rash looks like one or more small growths or wart-like bumps (called mollusca) that are usually pink, white, or skin-colored. The bumps are usually soft and smooth and may have an indented center. Infection is most common among kids between 1 and 12 years old, but also occurs in: teens and adults some athletes, such as wrestlers, swimmers, and gymnasts people whose immune systems have been weakened by HIV, cancer treatment, or long-term steroid use As you might guess by its name, this skin disorder is contagious, and can be passed from one person to another. It is unknown how long the rash and virus may be contagious. Causes Molluscum contagiosum is caused by the molluscum contagiosum virus (MCV), a member of the poxvirus family. This virus thrives in warm, humid climates and in areas where people live very close together. Infection with MCV occurs when the virus enters a small break in the skin's surface. Many people who come in contact with the virus have immunity against it, and do not develop any growths. For those not resistant to it, growths usually appear 2 to 8 weeks after infection. -
Inflammatory Or Infectious Hair Disease? a Case of Scalp Eschar and Neck Lymph Adenopathy After a Tick Bite
Case Report ISSN: 2574 -1241 DOI: 10.26717/BJSTR.2021.35.005688 Adherent Serous Crust of the Scalp: Inflammatory or Infectious Hair Disease? A Case of Scalp Eschar and Neck Lymph Adenopathy after a Tick Bite Starace M1, Vezzoni R*2, Alessandrini A1 and Piraccini BM1 1Dermatology - IRCCS, Policlinico Sant’Orsola, Department of Specialized, Experimental and Diagnostic Medicine, Alma Mater Studiorum, University of Bologna, Italy 2Dermatology Clinic, Maggiore Hospital, University of Trieste, Italy *Corresponding author: Roberta Vezzoni, Dermatology Clinic, Maggiore Hospital, University of Trieste, Italy ARTICLE INFO ABSTRACT Received: Published: April 17, 2021 The appearance of a crust initially suggests inflammatory scalp diseases, although infectious diseases such as impetigo or insect bites should also be considered among April 27, 2021 the differential diagnoses. We report a case of 40-year-old woman presentedB. Burgdorferi to our, Citation: Starace M, Vezzoni R, Hair Disease Outpatient Service with an adherent serous crust on the scalp and lymphadenopathy of the neck. Serological tests confirmed the aetiology of while rickettsia infection was excluded. Lyme borreliosis can mimic rickettsia infection Alessandrini A, Piraccini BM. Adherent and may present as scalp eschar and neck lymphadenopathy after a tick bite (SENLAT). Serous Crust of the Scalp: Inflammatory Appropriate tests should be included in the diagnostic workup of patients with necrotic or Infectious Hair Disease? A Case of Scalp scalpKeywords: eschar in order to promptly set -
Bacterial Infections Diseases Picture Cause Basic Lesion
page: 117 Chapter 6: alphabetical Bacterial infections diseases picture cause basic lesion search contents print last screen viewed back next Bacterial infections diseases Impetigo page: 118 6.1 Impetigo alphabetical Bullous impetigo Bullae with cloudy contents, often surrounded by an erythematous halo. These bullae rupture easily picture and are rapidly replaced by extensive crusty patches. Bullous impetigo is classically caused by Staphylococcus aureus. cause basic lesion Basic Lesions: Bullae; Crusts Causes: Infection search contents print last screen viewed back next Bacterial infections diseases Impetigo page: 119 alphabetical Non-bullous impetigo Erythematous patches covered by a yellowish crust. Lesions are most frequently around the mouth. picture Lesions around the nose are very characteristic and require prolonged treatment. ß-Haemolytic streptococcus is cause most frequently found in this type of impetigo. basic lesion Basic Lesions: Erythematous Macule; Crusts Causes: Infection search contents print last screen viewed back next Bacterial infections diseases Ecthyma page: 120 6.2 Ecthyma alphabetical Slow and gradually deepening ulceration surmounted by a thick crust. The usual site of ecthyma are the legs. After healing there is a permanent scar. The pathogen is picture often a streptococcus. Ecthyma is very common in tropical countries. cause basic lesion Basic Lesions: Crusts; Ulcers Causes: Infection search contents print last screen viewed back next Bacterial infections diseases Folliculitis page: 121 6.3 Folliculitis -
Case Report Evans Syndrome
Bangladesh Med J. 2018 Sept; 47(3) Case Report Evans Syndrome: A Case Report *Biswas SK1, BiswasT2, Khondoker N3, Alam MR4, Rahim MA5, Paul HK6, Shahin MA7, Hasan MN 8, Bhuiyan AKMM9 Abstract: INTRODUCTION Evans syndrome, a combined clinical condition of autoimmune Evans syndrome is an uncommon clinical condition de!ned haemolytic anaemia (AHA) and idiopathic thrombocytopaenic by the combination of autoimmune hemolytic anemia 1 purpura (ITP) and has non-speci!c pathogenesis. "e clinical (AHA) and idiopathic thrombocytopenic purpura (ITP). It cases are extremely rare, since only 4% of AHA or ITP are is a chronic immune- associated disease which has unknown incorporated with Evans. It is distinguished from di#erentials, pathophysiology. "e true Evans syndrome is diagnosed such as lupus, IgA de!ciency, and acquired immunode!ciency, when possibility of other confounding disorders is excluded. by peripheral blood !lm, bone marrow, Coombs test, and In 1951, Dr. Robert Evan discovered the spectrum like coagulation pro!le. A case of adult female from Pabna, relationship between these two combined diseases after 1 Bangladesh is documented in this report. She complained of studying twenty-four cases. Epidemiologically, the high grade intermittent fever, exertional dyspnea, icteric skin condition is extremely rare that only less than 4% of ITP or 2-5 and sclera. Other features included mild splenomegaly, dark AHA are diagnosed as Evans syndrome. "ere is evidence urine, and profuse sweating after fever. Investigation reports of both cellular and humoral immune-abnormalities in 6 were consistent with AHA and ITP, with normal coagulation Evans syndrome. Di#erent scientists provided di#erent and viral pro!le. -
022291S015 Eltrombopag Clinical BPCA
CLINICAL REVIEW Application Type sNDA Application Number(s) 022291, S-015 Priority or Standard Priority Submit Date(s) December 19, 2014 Received Date(s) December 19, 2014 PDUFA Goal Date June 19, 2015 Division / Office Division of Hematology Products/OHOP Reviewer Name(s) Lori A. Ehrlich Review Completion Date May 26, 2015 Established Name Eltrombopag Trade Name Promacta® Therapeutic Class Thrombopoietin Agonist Applicant Glaxo SmithKline Formulation(s) Tablet Dosing Regimen 50 mg once daily Indication(s) Chronic ITP Intended Population(s) Pediatric patients ≥6 years old with chronic ITP Template Version: March 6, 2009 Reference ID: 3765521 Clinical Review Lori A. Ehrlich, MD, PhD NDA 022291, S-015 Promacta® (eltrombopag) tablets Table of Contents 1 RECOMMENDATIONS/RISK BENEFIT ASSESSMENT ......................................... 8 1.1 Recommendation on Regulatory Action ............................................................. 8 1.2 Risk Benefit Assessment.................................................................................... 9 1.3 Recommendations for Postmarket Risk Evaluation and Mitigation Strategies . 11 1.4 Recommendations for Postmarket Requirements and Commitments .............. 11 2 INTRODUCTION AND REGULATORY BACKGROUND ...................................... 11 2.1 Product Information .......................................................................................... 12 2.2 Tables of Currently Available Treatments for Proposed Indications ................. 12 2.3 Availability of Proposed Active Ingredient -
Eyelid Conjunctival Tumors
EYELID &CONJUNCTIVAL TUMORS PHOTOGRAPHIC ATLAS Dr. Olivier Galatoire Dr. Christine Levy-Gabriel Dr. Mathieu Zmuda EYELID & CONJUNCTIVAL TUMORS 4 EYELID & CONJUNCTIVAL TUMORS Dear readers, All rights of translation, adaptation, or reproduction by any means are reserved in all countries. The reproduction or representation, in whole or in part and by any means, of any of the pages published in the present book without the prior written consent of the publisher, is prohibited and illegal and would constitute an infringement. Only reproductions strictly reserved for the private use of the copier and not intended for collective use, and short analyses and quotations justified by the illustrative or scientific nature of the work in which they are incorporated, are authorized (Law of March 11, 1957 art. 40 and 41 and Criminal Code art. 425). EYELID & CONJUNCTIVAL TUMORS EYELID & CONJUNCTIVAL TUMORS 5 6 EYELID & CONJUNCTIVAL TUMORS Foreword Dr. Serge Morax I am honored to introduce this Photographic Atlas of palpebral and conjunctival tumors,which is the culmination of the close collaboration between Drs. Olivier Galatoire and Mathieu Zmuda of the A. de Rothschild Ophthalmological Foundation and Dr. Christine Levy-Gabriel of the Curie Institute. The subject is now of unquestionable importance and evidently of great interest to Ophthalmologists, whether they are orbital- palpebral specialists or not. Indeed, errors or delays in the diagnosis of tumor pathologies are relatively common and the consequences can be serious in the case of malignant tumors, especially carcinomas. Swift diagnosis and anatomopathological confirmation will lead to a treatment, discussed in multidisciplinary team meetings, ranging from surgery to radiotherapy. -
HIV Infection and AIDS
G Maartens 12 HIV infection and AIDS Clinical examination in HIV disease 306 Prevention of opportunistic infections 323 Epidemiology 308 Preventing exposure 323 Global and regional epidemics 308 Chemoprophylaxis 323 Modes of transmission 308 Immunisation 324 Virology and immunology 309 Antiretroviral therapy 324 ART complications 325 Diagnosis and investigations 310 ART in special situations 326 Diagnosing HIV infection 310 Prevention of HIV 327 Viral load and CD4 counts 311 Clinical manifestations of HIV 311 Presenting problems in HIV infection 312 Lymphadenopathy 313 Weight loss 313 Fever 313 Mucocutaneous disease 314 Gastrointestinal disease 316 Hepatobiliary disease 317 Respiratory disease 318 Nervous system and eye disease 319 Rheumatological disease 321 Haematological abnormalities 322 Renal disease 322 Cardiac disease 322 HIV-related cancers 322 306 • HIV INFECTION AND AIDS Clinical examination in HIV disease 2 Oropharynx 34Neck Eyes Mucous membranes Lymph node enlargement Retina Tuberculosis Toxoplasmosis Lymphoma HIV retinopathy Kaposi’s sarcoma Progressive outer retinal Persistent generalised necrosis lymphadenopathy Parotidomegaly Oropharyngeal candidiasis Cytomegalovirus retinitis Cervical lymphadenopathy 3 Oral hairy leucoplakia 5 Central nervous system Herpes simplex Higher mental function Aphthous ulcers 4 HIV dementia Kaposi’s sarcoma Progressive multifocal leucoencephalopathy Teeth Focal signs 5 Toxoplasmosis Primary CNS lymphoma Neck stiffness Cryptococcal meningitis 2 Tuberculous meningitis Pneumococcal meningitis 6 -
Practice Parameter for the Diagnosis and Management of Primary Immunodeficiency
Practice parameter Practice parameter for the diagnosis and management of primary immunodeficiency Francisco A. Bonilla, MD, PhD, David A. Khan, MD, Zuhair K. Ballas, MD, Javier Chinen, MD, PhD, Michael M. Frank, MD, Joyce T. Hsu, MD, Michael Keller, MD, Lisa J. Kobrynski, MD, Hirsh D. Komarow, MD, Bruce Mazer, MD, Robert P. Nelson, Jr, MD, Jordan S. Orange, MD, PhD, John M. Routes, MD, William T. Shearer, MD, PhD, Ricardo U. Sorensen, MD, James W. Verbsky, MD, PhD, David I. Bernstein, MD, Joann Blessing-Moore, MD, David Lang, MD, Richard A. Nicklas, MD, John Oppenheimer, MD, Jay M. Portnoy, MD, Christopher R. Randolph, MD, Diane Schuller, MD, Sheldon L. Spector, MD, Stephen Tilles, MD, Dana Wallace, MD Chief Editor: Francisco A. Bonilla, MD, PhD Co-Editor: David A. Khan, MD Members of the Joint Task Force on Practice Parameters: David I. Bernstein, MD, Joann Blessing-Moore, MD, David Khan, MD, David Lang, MD, Richard A. Nicklas, MD, John Oppenheimer, MD, Jay M. Portnoy, MD, Christopher R. Randolph, MD, Diane Schuller, MD, Sheldon L. Spector, MD, Stephen Tilles, MD, Dana Wallace, MD Primary Immunodeficiency Workgroup: Chairman: Francisco A. Bonilla, MD, PhD Members: Zuhair K. Ballas, MD, Javier Chinen, MD, PhD, Michael M. Frank, MD, Joyce T. Hsu, MD, Michael Keller, MD, Lisa J. Kobrynski, MD, Hirsh D. Komarow, MD, Bruce Mazer, MD, Robert P. Nelson, Jr, MD, Jordan S. Orange, MD, PhD, John M. Routes, MD, William T. Shearer, MD, PhD, Ricardo U. Sorensen, MD, James W. Verbsky, MD, PhD GlaxoSmithKline, Merck, and Aerocrine; has received payment for lectures from Genentech/ These parameters were developed by the Joint Task Force on Practice Parameters, representing Novartis, GlaxoSmithKline, and Merck; and has received research support from Genentech/ the American Academy of Allergy, Asthma & Immunology; the American College of Novartis and Merck.