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eCommons@AKU LABRAD Publications 3-2020 LABRAD : Vol 46, Issue 1 - March 2020 Aga Khan University Hospital, Karachi Follow this and additional works at: https://ecommons.aku.edu/labrad Part of the Pathology Commons, and the Radiology Commons NEWSLETTER OF THE DEPARTMENTS OF PATHOLOGY & LABORATORY MEDICINE AND RADIOLOGY MARCH 2020 VOL. 46, ISSUE 1 1 MARCH 2020 VOL 46, ISSUE 1 A Publication of the Departments of Pathology & Laboratory Medicine and Radiology March 2020 Role of Immunofluorescence Studies in the Diagnosis of 3 Volume 46, Issue 1 Autoimmune Disorders Cystathionine Beta-Synthase Deficiency 5 Editor Treatment of Factor VIII Inhibitor 6 Dr. Lena Jafri Ergonomics In Laboratory: What You Need To Know About 7 Repetitive Motion Associate Editor Effective Internal Communication in Laboratories 9 Dr. Nasir Ud Din Digital Breast Tomosynthesis 11 Editorial Committee Therapeutic Drug Monitoring of Methotrexate: factors resulting 12 Dr. Najia Ghanchi in delayed clearance Dr. Hafsa Majid Overview of Common Myeloproliferative Neoplasms 13 Dr. Mohammad Zeeshan Dr. Anila Rashid Radiology Pathology Correlation. Bone Pathology 15 Dr Sidra Arshad E-Cigarette, Vaping Associated Lung Injury (EVALI), 17 Histopathological Diagnosis (literature review). Radiology Clinical Significance of free Androgen Index (FAI) 18 Dr. Shayan Anwar Dr. Shaista Afzal Radiology Quiz 19 Triple Negative Myeloproliferative Neoplasms 20 Associate Members Sony Siddiqui The Best of the Past 21 Iffat Arman Histological Evaluation of Common Gastro Intestinal Tract 22 Zeba Anwer Pathogens Ayman Syed Sadat Ali Quiz Answer 25 Labrad Administration Office Retinoblastoma: Updated CAP Guidelines for Staging 25 Farhana Arshad Section Of Histopathology: Newer Horizons 28 Department of Pathology and Laboratory Medicine Aspects of Quality Assurance in Laboratory Medicine: 30 A Road to Quality? Aga Khan University Hospital Stadium Road, P. O. Box 3500 A Webinar Series ‘Rare Links’ 31 Karachi 74800, Pakistan A Look into the Eye can Unleash Vital Diagnostic Pearls for 32 Rare Disorders Tel: 92 21 3486 1551 Lab & Rad Mesh up for the Early Recognition of Rare Disorders 33 Fax: 92 21 3493 4294, 3493 2095 Polaroid 34 hospitals.aku.edu/Karachi/clinical-laboratories 2 MARCH 2020 VOL 46, ISSUE 1 From the Editor’s Desk What distinguishes a remarkable Pathology from Hematology, “Therapeutic Drug Monitoring Department from an average one? Faculty members of Methotrexate: factors resulting in delayed and technologists who are committed to provide clearance” by technologist and faculty from Clinical better patient care with accurate rapid delivery Chemistry, “E-Cigarette, Vaping Associated Lung of laboratory test results, faculty members and Injury, Histopathological Diagnosis” from team of technologists who have advanced subspecialty histopathology and last and the best is the partnership trainings and are dedicated to teach and mentor the of Pathology and Radiology in the article “Radiology leaders of the future. These qualities are exactly what Pathology Correlation. Bone Pathology”. sets the Department of Pathology and Laboratory Medicine at Aga Khan University apart from those at Please welcome our new associate members of other institutions. Labrad who have joined our team from this year. My editorial team has several plans for improvements of First issue of Labrad for the year 2020 is in your Labrad during our tenure; we have already started hands. We always encourage residents, technologists the working. Don’t forget to take a look at two of the and faculty to come together and submit articles. sections Labrad team introduced last year: In this issue we have some good reads contributed • The Best of the Past by small teams. Some such examples in the current • Polaroid issue of Labrad are: “Role of Immunoflorescence Studies in the Diagnosis of Autoimmune Disorders” written by a team of technologists and faculty Happy reading ☺ from Histopathology, similarly “Overview of Dr Lena Jafri Common Myeloproliferative Neoplasms” by team Clinical Chemistry Role of Immunofluorescence Studies in the Diagnosis of Autoimmune Disorders Samar Naz, Miss Iqra Saleem and Dr. Sabeeh Siddique Histopathology Diagnosis of connective tissue and immunofluorescence Assays (IIFA) on Hep-2 rheumatological disorders can be challenging cells is the gold standard for autoimmune body task for the clinicians. The term connective screening in patients with systemic autoimmune tissue disorder encompasses a variety of distinct rheumatic diseases. IIFA has a good sensitivity diseases which have one thing in common; and it detects a wide range (approximately 100 that is the presence of circulating autoantigens to 150) of nuclear and cytoplasmic autoantigens. in the patients’ blood. The detection of these Due to its high sensitivity, it is often employed autoantibodies in the laboratory may help the as the first line diagnostic test that detects even clinicians in confirming the diagnosis. A variety those autoantigens which may be missed by other of laboratory based techniques are available to commercially available multiplex ANA tests. detect the relevant autoantibodies in patients’ Some of the patterns that are detected by IIFA and sera. However according to the American the associated diseases are listed in Table 1. College of Rheumatology (ACR), Indirect 3 MARCH 2020 VOL 46, ISSUE 1 Table 1. ANA Patterns and the Associated Diseases follow-up testing is advisable. If follow-up testing ANA pattern Associated Rheumatic Disease identifies the antigen, the clinical relevance can be Homogeneous l Systemic lupus erythematosus further refined. ICAP advocates that information l Mixed connective tissue disease on Hep-2 IIFA patterns should be reported to the l Drug induced lupus clinicians and should be incorporated in diagnostic l Juvenile idiopathic arthritis and classification criteria instead of simply reporting as ‘ANA Positive/Negative’. Speckled l Systemic lupus erythematosus l SjÖgren’s syndrome l Polymyositis/dermatomyositis Recently our laboratory has acquired a complete l Systemic sclerosis/scleroderma automation equipment (HELIOS®) for processing and reporting of the ANA samples. HELIOS® is the Nucleolar l Diffuse systemic sclerosis/scleroderma l Polymyositis first fully automated IIF processes and it includes an integrated optical system for automatic slide reading Centromere l Limited systemic sclerosis/scleroderma which not only discriminates between positive and negative samples but also detects the patterns and Peripheral (rim) l Systemic lupus erythematosus end point titers. The software provided with this l Systemic sclerosis/scleroderma equipment has an extensive pattern library, which Traditionally Hep-2 IIFA patterns were associated the technologist or the pathologist can access for with diseases. Now it has been realized that many academic purposes as well as for comparing the of these associations are only valid if the antigen results of the test samples. Some of these patterns are specificity has been confirmed by follow-up testing. shown in Figure 2. Therefore The International Consensus on ANA Patterns (ICAP) advocates that disease associations should be replaced by clinical relevance. Pattern assignment in clinical laboratories has been inconsistent. That is why ICAP initiated the consensus on nomenclature & definitions of the Hep-2 patterns. ICAP consensus is based on the clinical relevance of 29 Hep-2 IIFA patterns (Figure 1). The consensus on clinical relevance is defined in the clinical context Figure 2: Various ANA staining patterns seen on samples performed on HELIOS® Figure 1: Taken from: ICAP International Consensus on ANA In contrast to the manual performance, running the Patterns;https://www.anapatterns.org/trees-full.php ANA samples on an automated machine has the following benefits: of the patient, that is, suspected disease and includes l Provides optimum and consistent conditions for recommended follow-up testing (antigen specific processing of samples immunoassay). Cytoplasmic and mitotic patterns may l Minimal human involvement minimizes also have clinical relevance and therefore appropriate chances of technical errors 4 MARCH 2020 VOL 46, ISSUE 1 l Provides automated pattern recognition, end- l A positive ANA result in conjunction with point titer suggestions and reduction of clinical suspicion suggests, but does not interpretation bias necessarily confirm the presence of an l Less intensive training required autoimmune disease l Less labour intensive, medical technologists can l Positive results are not uncommon in healthy be spared do other chores individuals (particularly as they age) and those l Since the machine can run 106 samples in with certain infectious diseases or cancer a single batch the turnaround time is l In cases with strong clinical suspicion, specific significantly reduced. antibody testing may be appropriate even if the ANA IFA result is negative As mentioned previously that IIFA performed on l If the ANA IFA is positive but the tested HEp-2 cells is a sensitive test for detecting the antibodies are negative, the patient may still autoantigens in a patient, however several points are have an autoimmune disease other than those worth mentioning here: typically associated with the antibodies tested IFA ALGORITHIM ACCORDING TO THE INTERNATIONAL CONSENSUS Cystathionine Beta-Synthase Deficiency Saba Abdul Mateen Clinical Chemistry Cystathionine beta Synthase (CBS) deficiency is Homocystinuria, Beta-Synthase Deficiency an inherited metabolic disorder, in which