On Lupus, Vitamin D and Leukopenia

Total Page:16

File Type:pdf, Size:1020Kb

On Lupus, Vitamin D and Leukopenia r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):206–211 REVISTA BRASILEIRA DE REUMATOLOGIA w ww.reumatologia.com.br Original article On lupus, vitamin D and leukopenia ∗ Juliana A. Simioni, Flavia Heimovski, Thelma L. Skare Rheumatology Unit, Hospital Universitário Evangélico de Curitiba, Curitiba, PR, Brazil a r t i c l e i n f o a b s t r a c t Article history: Background: Immune regulation is among the noncalcemic effects of vitamin D. So, this Received 7 October 2014 vitamin may play a role in autoimmune diseases such as systemic lupus erythematosus Accepted 24 June 2015 (SLE). Available online 4 September 2015 Objectives: To study the prevalence of vitamin D deficiency in SLE and its association with clinical, serological and treatment profile as well as with disease activity. Keywords: Methods: Serum OH vitamin D3 levels were measured in 153 SLE patients and 85 controls. Data on clinical, serological and treatment profile of lupus patients were obtained through Systemic lupus erythematosus Vitamin D chart review. Blood cell count and SLEDAI (SLE disease activity index) were measured simul- Leukopenia taneously with vitamin D determination. Granulocytopenia Results: SLE patients have lower levels of vitamin D than controls (p = 0.03). In univariate analysis serum vitamin D was associated with leukopenia (p = 0.02), use of cyclophos- phamide (p = 0.007) and methotrexate (p = 0.03). A negative correlation was verified with prednisone dose (p = 0.003). No association was found with disease activity measured by SLEDAI (p = 0.88). In a multiple regression study only leukopenia remained as an independent association (B = 4.04; p = 0.02). A negative correlation of serum vitamin level with granulocyte (p = 0.01) was also found, but not with lymphocyte count (p = 0.33). Conclusion: SLE patients have more deficiency of vitamin D than controls. This deficiency is not associated with disease activity but with leucopenia (granulocytopenia). © 2015 Elsevier Editora Ltda. All rights reserved. Acerca de lúpus, vitamina D e leucopenia r e s u m o Palavras-chave: Introduc¸ão: A regulac¸ão imune está entre os efeitos não calcêmicos da vitamina D. Assim, Lúpus eritematoso sistêmico essa vitamina pode influenciar em doenc¸as autoimunes, como o lúpus eritematoso Vitamina D sistêmico (LES). Leucopenia Objetivos: Estudar a prevalência da deficiência de vitamina D no LES e sua associac¸ão com Granulocitopenia o perfil clínico, sorológico e de tratamento, bem como com a atividade da doenc¸a. Métodos: Mensuraram-se os níveis séricos de OH-vitamina D3 em 153 pacientes com LES e 85 controles. Os dados sobre o perfil clínico, sorológico e de tratamento de pacientes com ∗ Corresponding author. E-mail: [email protected] (T.L. Skare). http://dx.doi.org/10.1016/j.rbre.2015.08.008 2255-5021/© 2015 Elsevier Editora Ltda. All rights reserved. r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):206–211 207 lúpus foram obtidos por meio da revisão de prontuários. Simultaneamente à determinac¸ão da vitamina D, foi feito um hemograma e foi aplicado o Sledai (SLE disease activity índex [índice de atividade da doenc¸a no LES]). Resultados: Os pacientes com LES tinham níveis mais baixos de vitamina D do que os con- troles (p = 0,03). Na análise univariada, a vitamina D sérica esteve associada à leucopenia (p = 0,02) e ao uso de ciclofosfamida (p = 0,007) e metotrexato (p = 0,03). Foi verificada uma correlac¸ão negativa com a dose de prednisona (p = 0,003). Não foi encontrada associac¸ãocom a atividade da doenc¸a medida pelo Sledai (p = 0,88). Em um estudo de regressão múltipla, somente a leucopenia permaneceu como uma associac¸ão independente (B = 4,04; p = 0,02). Também foi encontrada correlac¸ão negativa do nível sérico de vitamina D com os granulóc- itos (p = 0,01), mas não com a contagem de linfócitos (p = 0,33). Conclusão: Os pacientes com LES têm mais deficiência de vitamina D do que os controles. Essa deficiência não está associada com a atividade da doenc¸a, mas com a leucopenia (granulocitopenia). © 2015 Elsevier Editora Ltda. Todos os direitos reservados. that this medication hinders the production of dihydrox- Introduction yvitamin D as they inhibit the alpha-hydroxylase enzyme 1 responsible for transformation of mono in dihydroxyvitamin Vitamin D has immune modulatory properties. The main 17 D. This action could increase circulating levels of the level source of vitamin for humans is the conversion of 7- of monohidroxy vitamin D3 in detriment of dyhidroxy active dehydrocholesterol in pre vitamin D3 in the skin, which 17 2 form. occurs by exposure to ultraviolet radiation. A small portion In the present study we aimed to determine whether the comes from the diet, mainly seafood 17. Vitamin D3 must levels of vitamin D in a Southern Brazilian population with SLE first undergo one hydroxylation in the liver resulting in 25 OH 2 are associated with disease activity, serological, clinical profile vitamin D3 or calcidiol which is the circulating form of the and with medications used for treatment. vitamin and that it was the form used for serum measure- ment at present. The most active form of vitamin D is 1,25 2 (OH)2 vitamin D synthesized in the kidney. It is known that Methods this vitamin plays an inhibitory role of dendritic cells, CD4, CD8, B lymphocytes and the production of cytokines, such as This study was approved by the local Ethics Committee in IFN ␥, IL-2, IL-6, TNF-␣, and increases the number of T regula- Research and all participants signed an informed consent. tory cells and synthesis of other cytokines such as IL-4, IL-10 One hundred and fifty three patients with at least 4 of 1997 1,3,4 and TGF ␤. Thus it is conceivable that serum vitamin D modified American College of Rheumatology (ACR) classifi- 18 levels exert influence on autoimmune diseases such as SLE. cation criteria for SLE were invited to participate during However existing studies in this area are contradictory. Bir- the period of six months, according to the order of consul- 5 mingham et al. reported that patients with acute disease flare tation in the clinic and willingness to participate in the study. have lower levels of vitamin D but a cause-effect relationship We excluded patients using anticonvulsants with creatinine could not be established. Others noted that deficiency of vita- greater than 1.3 mg/dL and pregnant women. None of the 6–8 min D has been associated with increased disease activity, included patients had made replacement of vitamin D in the 4,9,10 while others denied it. Some clinical manifestations have last year and none made use of more than 600 IU of vita- 4 been associated with vitamin D levels such as leukopenia min D3/day, which is routinely done in the service for all 4 renal involvement, photosensitivity and the presence of anti- glucocorticoids users. Demographic, clinical and serological 7 ds DNA. data were collected through chart review. Clinical data were The vitamin D receptor gene polymorphism has been considered a cumulative way and defined according to the 18 linked with SLE susceptibility in Asian, Polish and Egyp- 1997 ACR classification criteria for SLE. Disease activity was 11–13 19 tian patients. In Brazilians this association could not be calculated by SLEDAI (or SLE Disease activity index). Also, demonstrated although it seemed to influence the appearance data on creatinine levels and blood cell count were collected of cutaneous and articular manifestations and in the presence simultaneously with vitamin D as well as the drugs consid- 14 of anti-ds DNA. ered for study were those used at the moment of vitamin D Some factors contribute to a higher prevalence of vitamin determination. D deficiency in SLE patients than in the general population. As controls, we included 85 self-reported healthy individ- The photoprotection, held as part of the disease treatment, uals from the same geographical area, matched for age and can block the synthesis of cholecalciferol induced by UVB gender. 15 radiation on the skin. Antimalarials, widely used in this dis- The serum vitamin D (25 OH vitamin D3) was analyzed by ease, are described by some authors to be associated with its chemiluminescence by the Liaisom 25OH Vitamin D Assay 16 ≥ deficiency due to its photoprotective effect. Others believe (DiaSorin Inc., Stillwater, MN, USA). Value 30 mg/dL were 208 r e v b r a s r e u m a t o l . 2 0 1 6;5 6(3):206–211 considered normal; between 20 and 29 mg/dL were consid- Table 1 – Clinical, serological and treatment profile of ered as vitamin insufficiency and values below 20 mg/dL as 153 patients with systemic lupus erythematous. deficiency. n Data were collected on the frequency and contin- gency tables. Study of data distribution was performed by Arthritis 86/140 61.4% Serositis 34/105 32.3% the Kolmogorov–Smirnov test and central tendency was Hemolytic anemia 12/139 8.6% expressed as median and interquartile range (IQR) for non- Leukopenia 40/139 28.7% parametric and mean and standard deviation for parametric Thrombocytopenia 35/136 25.7% data. Association studies of vitamin D levels with clinical, Glomerulonephrites 60/140 42.8% serological and demographic variables were made by Fisher’s Discoid lesions 15/136 11.0% and chi-square when data was nominal and for unpaired t Malar rash 72/134 53.7% test when nominal. Correlation studies of SLEDAI values, age, Photossensitivity 103/148 69.5% Oral ulcers 55/138 39.8% prednisone dose, granulocyte and lymphocyte blood count Seizures 17/137 12.4% with values of serum vitamin D were done using Pearson and Psychosis 9/96 9.3% Spearman test.
Recommended publications
  • Agammaglobulinemia Hypogammaglobulinemia Hereditary Disease Immunoglobulins
    Pediat. Res. 2: 72-84 (1968) Agammaglobulinemia hypogammaglobulinemia hereditary disease immunoglobulins Hereditary Alterations in the Immune Response: Coexistence of 'Agammaglobulinemia', Acquired Hypogammaglobulinemia and Selective Immunoglobulin Deficiency in a Sibship REBECCA H. BUCKLEY[75] and J. B. SIDBURY, Jr. Departments of Pediatrics, Microbiology and Immunology, Division of Immunology, Duke University School of Medicine, Durham, North Carolina, USA Extract A longitudinal immunologic study was conducted in a family in which an entire sibship of three males was unduly susceptible to infection. The oldest boy's history of repeated severe infections be- ginning in infancy and his marked deficiencies of all three major immunoglobulins were compatible with a clinical diagnosis of congenital 'agammaglobulinemia' (table I, fig. 1). Recurrent severe in- fections in the second boy did not begin until late childhood, and his serum abnormality involved deficiencies of only two of the major immunoglobulin fractions, IgG and IgM (table I, fig. 1). This phenotype of selective immunoglobulin deficiency is previously unreported. Serum concentrations of the three immunoglobulins in the youngest boy (who also had a late onset of repeated infection) were normal or elevated when he was first studied, but a marked decline in levels of each of these fractions was observed over a four-year period (table I, fig. 1). We could find no previous reports describing apparent congenital and acquired immunologic deficiencies in a sibship. Repeated infections and demonstrated specific immunologic unresponsiveness preceded gross ab- normalities in the total and fractional gamma globulin levels in both of the younger boys (tables II-IV). When the total immunoglobulin level in the second boy was 735 mg/100 ml, he failed to respond with a normal rise in titer after immunization with 'A' and 'B' blood group substances, diphtheria, tetanus, or Types I and II poliovaccines.
    [Show full text]
  • Ataxia Telangiectasia: a Diagnostic Challenge. Case Report
    case reports 2020; 6(2) https://doi.org/10.15446/cr.v6n2.83219 ATAXIA TELANGIECTASIA: A DIAGNOSTIC CHALLENGE. CASE REPORT Keywords: Ataxia Telangiectasia; Neurodegenerative Diseases; Cerebellar Ataxia; Spinocerebellar Degenerations; Telangiectasia. Palabras clave: Ataxia telangiectasia; Enfermedades neurodegenerativas; Ataxia cerebelosa; Degeneraciones espinocerebelosa; Telangiectasia. Natalia Martínez-Córdoba Universidad Militar Nueva Granada - Faculty of Medicine - Pediatric Neurology Research Group - Bogotá D.C. - Colombia. Hospital Militar Central - Pediatric Neurology Service - Bogotá D.C. - Colombia. Eugenia Espinosa-García Universidad Militar Nueva Granada - Faculty of Medicine - Pediatric Neurology Research Group - Bogotá D.C. - Colombia. Hospital Militar Central - Pediatric Neurology Service - Bogotá D.C. - Colombia. Universidad del Rosario - Medical School - Bogotá D.C. - Colombia. Corresponding author Natalia Martínez-Córdoba. Faculty of Medicine, Universidad Militar Nueva Granada. Bogotá D.C. Colombia. Email: [email protected]. Received: 28/10/2019 Accepted: 08/01/2020 case reports Vol. 6 No. 2: 109-17 110 RESUMEN ABSTRACT Introducción. La ataxia-telangiectasia (AT) es Introduction: Ataxia-telangiectasia (AT) is a un síndrome neurodegenerativo con baja inciden- neurodegenerative syndrome with low incidence cia y prevalencia mundial que es causado por una and prevalence worldwide, which is caused by a mutación del gen ATM, es de herencia autosó- mutation of the ATM gene. It is an autosomal re- mica recesiva y se asocia a mecanismos defec- cessive disorder that is associated with defective tuosos en la regeneración y reparación del ADN. cell regeneration and DNA repair mechanisms. It Este síndrome se caracteriza por la presencia de is characterized by progressive cerebellar atax- ataxia cerebelosa progresiva, movimientos ocula- ia, abnormal eye movements, oculocutaneous res anormales, telangiectasias oculocutáneas e telangiectasias and immunodeficiency.
    [Show full text]
  • 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.
    [Show full text]
  • Neonatal Leukopenia and Thrombocytopenia
    Neonatal Leukopenia and Thrombocytopenia Vandy Black, M.D., M.Sc., FAAP March 3, 2016 April 14, 2011 Objecves • Summarize the differenHal diagnosis of leukopenia and/or thrombocytopenia in a neonate • Describe the iniHal steps in the evaluaon of a neonate with leukopenia and/or thrombocytopenia • Review treatment opHons for leukopenia and/ or thrombocytopenia in the NICU Clinical Case 1 • One day old male infant admiUed to the NICU for hypoglycemia and a sepsis rule out • Born at 38 weeks EGA by SVD • Birth weight 4 lbs 13 oz • Exam shows a small cephalohematoma; no dysmorphic features • PLT count 42K with an otherwise normal CBC Definions • Normal WBC count 9-30K at birth – Mean 18K • What is the ANC and ALC – <1000/mm3 is abnormal – 6-8% of infants in the NICU • Normal platelet count: 150-450,000/mm3 – Not age dependent – 22-35% of infants in the NICU have plts<150K Neutropenia Absolute neutrophil count <1500/mm3 Category ANC* InfecHon risk • Mild 1000-1500 None • Moderate 500-1000 Minimal • Severe <500 Moderate to Severe (Highest if <200) • Recurrent bacterial or fungal infecHons are the hallmark of symptomac neutropenia! • *ANC = WBC X % (PMNs + Bands) / 100 DefiniHon of Neutropenia Black and Maheshwari, Neoreviews 2009 How to Approach Cytopenias • Normal vs. abnormal (consider severity) • Malignant vs. non-malignant • Congenital vs. acquired • Is the paent symptomac • Transient, recurrent, cyclic, or persistent How to Approach Cytopenias • Adequate vs. decreased marrow reserve • Decreased producHon vs. increased destrucHon/sequestraon Decreased neutrophil/platelet producon • Primary – Malignancy/leukemia/marrow infiltraon – AplasHc anemia – Genec disorders • Secondary – InfecHous – Drug-induced – NutriHonal • B12, folate, copper Increased destrucHon/sequestraon • Immune-mediated • Drug-induced • Consumpon à Hypersplenism vs.
    [Show full text]
  • Interactions of the Classical and Alternate Complement Pathway with Endotoxin Lipopolysaccharide EFFECT on PLATELETS and BLOOD COAGULATION
    Interactions of the Classical and Alternate Complement Pathway with Endotoxin Lipopolysaccharide EFFECT ON PLATELETS AND BLOOD COAGULATION Michael A. Kane, … , Joseph E. May, Michael M. Frank J Clin Invest. 1973;52(2):370-376. https://doi.org/10.1172/JCI107193. Research Article The contributions of the classical and alternate pathways of complement activation to the biological effects of endotoxin have been examined in the guinea pig, with particular reference to thrombocytopenia, leukopenia, and the development of the hypercoagulable state. Injection of endotoxin into normal guinea pigs led to a 95% fall in the level of circulating platelets within 15 min as well as a fall in circulating granulocytes. C4-deficient guinea pigs, known to have a complete block in the activity of the classical complement pathway, but with the alternate pathway intact, sustained no fall in platelets. The development of granulocytopenia proceeded normally. Endotoxin did activate the alternate complement pathway in C4D guinea pigs, as evidenced by the fall in C3-9 titers. With restoration of serum C4 levels, endotoxin- induced thrombocytopenia was observed in C4D animals. Thus, function of the classical complement pathway was an absolute requirement for the development of thrombocytopenia. Experiments performed in cobra venom factor (CVF)- treated normal guinea pigs, with normal levels of C1, C4, and C2, but with less than 1% of serum C3-9 demonstrated the importance of the late components in the development of thrombocytopenia but not leukopenia. C4-deficient guinea pigs had normal clotting times demonstrating that C4 was not required for normal clotting. In addition, development of the hypercoagulable state, evidenced by a marked shortening of the clotting […] Find the latest version: https://jci.me/107193/pdf Interactions of the Classical and Alternate Complement Pathway with Endotoxin Lipopolysaccharide EFFECT ON PLATELETS AND BLOOD COAGULATION MICHAEL A.
    [Show full text]
  • Blood and Immunity
    Chapter Ten BLOOD AND IMMUNITY Chapter Contents 10 Pretest Clinical Aspects of Immunity Blood Chapter Review Immunity Case Studies Word Parts Pertaining to Blood and Immunity Crossword Puzzle Clinical Aspects of Blood Objectives After study of this chapter you should be able to: 1. Describe the composition of the blood plasma. 7. Identify and use roots pertaining to blood 2. Describe and give the functions of the three types of chemistry. blood cells. 8. List and describe the major disorders of the blood. 3. Label pictures of the blood cells. 9. List and describe the major disorders of the 4. Explain the basis of blood types. immune system. 5. Define immunity and list the possible sources of 10. Describe the major tests used to study blood. immunity. 11. Interpret abbreviations used in blood studies. 6. Identify and use roots and suffixes pertaining to the 12. Analyse several case studies involving the blood. blood and immunity. Pretest 1. The scientific name for red blood cells 5. Substances produced by immune cells that is . counteract microorganisms and other foreign 2. The scientific name for white blood cells materials are called . is . 6. A deficiency of hemoglobin results in the disorder 3. Platelets, or thrombocytes, are involved in called . 7. A neoplasm involving overgrowth of white blood 4. The white blood cells active in adaptive immunity cells is called . are the . 225 226 ♦ PART THREE / Body Systems Other 1% Proteins 8% Plasma 55% Water 91% Whole blood Leukocytes and platelets Formed 0.9% elements 45% Erythrocytes 10 99.1% Figure 10-1 Composition of whole blood.
    [Show full text]
  • Immunodeficiency Disorders Ivan K
    Immunodeficiency Disorders Ivan K. Chinn, MD,*† Jordan S. Orange, MD, PhD‡x *Department of Pediatrics, Section of Immunology, Allergy, and Rheumatology, Baylor College of Medicine, Houston, TX †Center for Human Immunobiology, Texas Children’s Hospital, Houston, TX ‡Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, NY xNew York Presbyterian Morgan Stanley Children’s Hospital, New York, NY Education Gaps Immunodeficiencies are no longer considered rare conditions. Although susceptibility to infections has become well-recognized as a sign of most primary immunodeficiencies, some children will present with noninfectious manifestations that remain underappreciated and warrant evaluation by an immunologic specialist. Providers must also consider common secondary causes of immunodeficiency in children. Objectives After completing this article, readers should be able to: 1. Recognize infectious signs and symptoms of primary immunodeficiency that warrant screening and referral to a specialist. 2. Understand noninfectious signs and symptoms that should raise concern for primary immunodeficiency. 3. Determine appropriate testing for patients for whom immunodeficiency is suspected. AUTHOR DISCLOSURE Dr Chinn has disclosed that he is the recipient of a 4. Discuss the management of patients with primary immunodeficiency. Translational Research Program grant from the Jeffrey Modell Foundation. Dr Orange has 5. Appreciate secondary causes of immunodeficiency. disclosed that he is a consultant on the topic of therapeutic immunoglobulin for Shire, CSL Behring, and Grifols and that he serves on the scientific advisory board for ADMA Biologics. This commentary does contain a discussion of INTRODUCTION an unapproved/investigative use of a commercial product/device. Immunodeficiency disorders represent defects in the immune system that result in weakened or dysregulated immune defense.
    [Show full text]
  • Hyperimmunoglobulin E Syndrome: Genetics, Immunopathogenesis, Clinical Findings, And
    Hyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and TICLE R treatment modalities A Hassan Hashemi1,2, Masoumeh Mohebbi1,2, Shiva Mehravaran1,3, Mehdi Mazloumi2, Hamidreza Jahanbani-Ardakani4,5, Seyed-Hossein Abtahi4,5,6 1Noor Ophthalmology Research Center, Noor Eye Hospital, Tehran, 2Department of Ophthalmology, Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, 4Isfahan Eye Research Center, Feiz Eye Hospital, Isfahan University of Medical Sciences, Isfahan, 5Isfahan Medical Students Research Center (IMSRC), Isfahan University of Medical Sciences, Isfahan, 6Department of Ophthalmology, Feiz Eye Hospital, 3 EVIEW Isfahan University of Medical Sciences, Isfahan, Iran, Department of Ophthalmology, Stein Eye Institute, David Geffen School of Medicine at UCLA, University of California, Los Angeles, California, USA R The hyperimmunoglobulin E syndromes (HIESs) are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levels of immunoglobulin E (IgE), recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. Most cases of HIES are sporadic although can be inherited as autosomal dominant and autosomal recessive traits. A fundamental immunologic defect in HIES is not clearly elucidated but abnormal neutrophil chemotaxis due to decreased production or secretion of interferon γ has main role in the immunopathogenesis of syndrome, also distorted Th1/Th2 cytokine profile toward a Th2 bias contributes to the impaired cellular immunity and a specific pattern of infection susceptibility as well as atopic‑allergic constitution of syndrome. The ophthalmic manifestations of this disorder include conjunctivitis, keratitis, spontaneous corneal perforation, recurrent giant chalazia, extensive xanthelasma, tumors of the eyelid, strabismus, and bilateral keratoconus.
    [Show full text]
  • Practical Guidance for the Evaluation and Management of Drug Hypersensitivity: Specific Drugs
    Specific Drugs Practical Guidance for the Evaluation and Management of Drug Hypersensitivity: Specific Drugs Chief Editors: Ana Dioun Broyles, MD, Aleena Banerji, MD, and Mariana Castells, MD, PhD Ana Dioun Broyles, MDa, Aleena Banerji, MDb, Sara Barmettler, MDc, Catherine M. Biggs, MDd, Kimberly Blumenthal, MDe, Patrick J. Brennan, MD, PhDf, Rebecca G. Breslow, MDg, Knut Brockow, MDh, Kathleen M. Buchheit, MDi, Katherine N. Cahill, MDj, Josefina Cernadas, MD, iPhDk, Anca Mirela Chiriac, MDl, Elena Crestani, MD, MSm, Pascal Demoly, MD, PhDn, Pascale Dewachter, MD, PhDo, Meredith Dilley, MDp, Jocelyn R. Farmer, MD, PhDq, Dinah Foer, MDr, Ari J. Fried, MDs, Sarah L. Garon, MDt, Matthew P. Giannetti, MDu, David L. Hepner, MD, MPHv, David I. Hong, MDw, Joyce T. Hsu, MDx, Parul H. Kothari, MDy, Timothy Kyin, MDz, Timothy Lax, MDaa, Min Jung Lee, MDbb, Kathleen Lee-Sarwar, MD, MScc, Anne Liu, MDdd, Stephanie Logsdon, MDee, Margee Louisias, MD, MPHff, Andrew MacGinnitie, MD, PhDgg, Michelle Maciag, MDhh, Samantha Minnicozzi, MDii, Allison E. Norton, MDjj, Iris M. Otani, MDkk, Miguel Park, MDll, Sarita Patil, MDmm, Elizabeth J. Phillips, MDnn, Matthieu Picard, MDoo, Craig D. Platt, MD, PhDpp, Rima Rachid, MDqq, Tito Rodriguez, MDrr, Antonino Romano, MDss, Cosby A. Stone, Jr., MD, MPHtt, Maria Jose Torres, MD, PhDuu, Miriam Verdú,MDvv, Alberta L. Wang, MDww, Paige Wickner, MDxx, Anna R. Wolfson, MDyy, Johnson T. Wong, MDzz, Christina Yee, MD, PhDaaa, Joseph Zhou, MD, PhDbbb, and Mariana Castells, MD, PhDccc Boston, Mass; Vancouver and Montreal,
    [Show full text]
  • Immunology of the Acquired Immunodeficiency Syndrome
    Henry Ford Hospital Medical Journal Volume 32 Number 2 Article 6 6-1984 Immunology of the Acquired Immunodeficiency Syndrome Patrick W. McLaughlin Carl B. Lauter Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation McLaughlin, Patrick W. and Lauter, Carl B. (1984) "Immunology of the Acquired Immunodeficiency Syndrome," Henry Ford Hospital Medical Journal : Vol. 32 : No. 2 , 107-115. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol32/iss2/6 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hospital Med J Vol. 32, No. 2, 1984 Immunology of the Acquired Immunodeficiency Syndrome Patrick W. McLaughlin, MD* and Carl B. Lauter, MD** The Acquired Immunodeficiency Syndrome (AIDS) first known to cause immunodeficiency. Simian AIDS, an recognized in 1981, has been extensively studied, and animal model of virus-induced immunodeficiency, is the most recent studies have demonstrated that its also discussed. Recommendations are made regarding defects extend well beyond the apparent deficient the interpretation and limitations of immune testing in cell-mediated immunity to include humoral immunity, AIDS, includinga discussion of how apparently healthy autoimmunity, and abnormal immunoregulation. The homosexuals and groups at risk develop an immu­ recent discovery ofa retrovirus, HTLV III, as a possible nologic profile which mimics that of patients with AIDS. etiologic agent is discussed in relation to other viruses J\r\ce late 1981, an unexpected outbreak of oppor­ The immune system has "surveillance" functions to tunistic infections and unusual neoplasms has been protect humans (and other vertebrates) from infections noted, described, and studied (1-4).
    [Show full text]
  • Chronic Lymphocytic Leukemia Early Detection, Diagnosis, and Staging Detection and Diagnosis
    cancer.org | 1.800.227.2345 Chronic Lymphocytic Leukemia Early Detection, Diagnosis, and Staging Detection and Diagnosis Finding cancer early often allows for more treatment options. Some early cancers may have signs and symptoms that can be noticed, but that's not always the case. ● Can Chronic Lymphocytic Leukemia Be Found Early? ● Signs and Symptoms of Chronic Lymphocytic Leukemia ● How Is Chronic Lymphocytic Leukemia Diagnosed? Stages and Outlook (Prognosis) After a cancer diagnosis, staging provides important information about the extent of cancer in the body and anticipated response to treatment. ● How Is Chronic Lymphocytic Leukemia Staged? Questions to Ask About CLL Here are some questions you can ask your cancer care team to help you better understand your CLL diagnosis and treatment options. ● Questions to Ask About Chronic Lymphocytic Leukemia 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 Can Chronic Lymphocytic Leukemia Be Found Early? For certain cancers, the American Cancer Society recommends screening tests1 in people without any symptoms, because they are easier to treat if found early. But for chronic lymphocytic leukemia (CLL) , no screening tests are routinely recommended at this time. Many times, CLL is found when routine blood tests are done for other reasons. For instance, a person's white blood cell count may be very high, even though he or she doesn't have any symptoms. If you notice any symptoms that could be caused by CLL, report them to your doctor right away so the cause can be found and treated, if needed. Hyperlinks 1. www.cancer.org/healthy/find-cancer-early/cancer-screening-guidelines/american- cancer-society-guidelines-for-the-early-detection-of-cancer.html Last Revised: May 10, 2018 Signs and Symptoms of Chronic Lymphocytic Leukemia Many people with chronic lymphocytic leukemia (CLL) do not have any symptoms when it is diagnosed.
    [Show full text]
  • Hematological Profile of HIV Patients in Relation to Immune Status - a Hospital-Based Cohort from Varanasi, North India
    Turk J Hematol 2008; 25:13-19 RESEARCH ARTICLE ©Turkish Society of Hematology Hematological profile of HIV patients in relation to immune status - a hospital-based cohort from Varanasi, North India Suresh Venkata Satya Attili1, V. P. Singh1, Madhukar Rai1, Datla Vivekananda Varma2, A. K. Gulati3, Shyam Sundar1 1Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India ) [email protected] 2Department of Skin and Venereal Disease, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India 3Department of Microbiology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India Received: 11.05.2007 • Accepted: 09.01.2008 ABSTRACT Objectives: To study the spectrum of hematological manifestations and evaluate the relationship between various hematological manifestations and CD4 cell counts in a hospital-based cohort of HIV-infected adults in and around Varanasi, North India. Materials and Methods: The clinical and hematological profiles of the patients attending the Infectious Disea- se Clinic, Varanasi, India were recorded. The relationship between CD4 counts and various hematological ma- nifestations was analyzed. Results: A total of 470 HIV-infected individuals were followed for 830 person years of observation (PYO). Rate of hematological episodes was 1047 episodes per 1000 PYO. CD4 counts were significantly lower in in- dividuals with severe anemia and neutropenia compared to those without. However, no relation could be es- tablished between thrombocytopenia and CD4 counts. In the above- mentioned population, CD4 levels were significantly lower in those with anemia/neutropenia harboring any particular disease compared to those who had the same disease without anemia/ neutropenia. Conclusions: There is a strong negative association between CD4 counts and the severity of anemia and ne- utropenia in this population.
    [Show full text]