scholarly journals IgG4-related lymphadenopathy masquerading as Hodgkin lymphoma: lessons from a pathologist’s desk

2019 ◽  
Vol 12 (9) ◽  
pp. e229800 ◽  
Author(s):  
Sree Rekha Jinkala ◽  
Bheemanathi Hanuman Srinivas ◽  
Puthenpurackal Sivanpillai Priyamvada ◽  
Debdatta Basu
Keyword(s):  
Clinical Presentation ◽  
Correct Diagnosis ◽  
Renal Involvement ◽  
Multiple Organ ◽  
Organ System ◽  
Rare Presentation ◽  
Related Disease ◽  
Organ Systems ◽  
Reactive Proliferation ◽  
Treatable Condition

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting multiple organ systems. The awareness of this disease has tremendously increased over the last decade leading to effective treatment and decreased morbidity to the patients. Histopathology plays an important role in the diagnosis of IgG4-RD, and definite histologic criteria are proposed in clinically suspected patients. We report a patient with multiple organ system involvements of the salivary gland, lymph node and kidney. IgG4-related lymphadenopathy (IgG4-RL) in this patient was misdiagnosed as nodular lymphocyte predominant Hodgkin’s lymphoma (NLPHL). Refractoriness to treatment for NLPHL and subsequent manifestations of renal involvement lead us to the correct diagnosis of this potentially treatable condition. IgG4-RL can mimic reactive proliferation as well as lymphomas. We report the clinical presentation and discuss the problems faced by pathologists in diagnosing IgG4-RL. We believe that awareness of this rare presentation will enhance the knowledge in diagnosing IgG4-RD.

Thrombotic and Hemorrhagic Disorders

2011 ◽  
Vol 16 (5) ◽  
pp. 1-4
Author(s):  
Stephen L. Demeter
Keyword(s):  
Daily Living ◽  
Multiple Organ ◽  
Organ System ◽  
Fourth Edition ◽  
Sixth Edition ◽  
Hematological Disorders ◽  
Organ Systems ◽  
Hemorrhagic Disorders ◽  
Platelet Disorders ◽  
Single Organ

Abstract The fourth edition of the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides) devoted only a single page to the rating of thrombotic and hemorrhagic disorders, based primarily on analogous impairments from other organ systems and impairments in the activities of daily living (ADLs). The AMA Guides, Fifth Edition, rated thrombotic disorders by the effects on the organ system; criteria for rating hemorrhagic and platelet disorders were based on the treatment required and interferences in ADLs. The sixth edition introduced significant changes, among which are the following: The ratings for hematological disorders were deliberately reduced, especially the highest ratings, because of the inevitable involvement of other organ systems. The goal is for evaluators to rate each organ system based on its defined criteria rather than combining multiple organ system difficulties into a single organ system rating. Also in the AMA Guides, Sixth Edition, separate tables are used to rate hemorrhagic conditions caused by platelet disorders, hemophilia, and clotting disorders. Further, major changes were required because of global methodological changes in this edition, including a significant expansion of the methodology used in rating clotting defects. Finally, the rating for the use of warfarin was altered, and thrombotic disorders are rated using the specific coagulation defect.

IgG4-related disease: a great mimicker of lung cancer

2021 ◽  
Vol 14 (2) ◽  
pp. e239976
Author(s):  
Natalie Louise Simon ◽  
Mostafa Negmeldin
Keyword(s):  
Lung Cancer ◽  
Inflammatory Infiltrate ◽  
Haematological Malignancy ◽  
Multiple Organ ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Ct Findings ◽  
Organ Systems ◽  
Pulmonary Manifestations ◽  
Storiform Fibrosis

IgG4-related disease is a rheumatological disorder, affecting multiple organ systems, and displaying dense lymphoplasmacytic inflammatory infiltrate and storiform fibrosis on histology. The pulmonary manifestations of IgG4-related disease are varied. Most commonly, bronchovascular consolidation and perilymphatic parenchymal thickening occur. IgG4-related disease can present as a solitary parenchymal mass and is often mistaken for a primary pulmonary or haematological malignancy. This report presents a case of IgG4-related disease in a patient with 6 months reported haemoptysis symptomatology and CT findings of perihilar lymphadenopathy, multiple pulmonary parenchymal nodules and a single parenchymal mass. Clinician cognisance of the histopathological presentation of IgG4-disease is important. It should be considered as a differential in patients for which investigations have ruled out malignancy, as it is responsive to glucocorticoids in the majority of cases.

scholarly journals Recent advances in understanding and managing IgG4-related disease

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 185 ◽  
Author(s):  
Anna R. Wolfson ◽  
Daniel L. Hamilos
Keyword(s):  
Clinical Presentation ◽  
Serum Markers ◽  
Emission Tomography ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Follicular Helper ◽  
Positron Emission ◽  
Medical Disciplines ◽  
Treatable Condition

IgG4-related disease was only recently discovered, so its description, management, and new discoveries related to its etiology are rapidly evolving. Because IgG4 itself is a unique antibody which is intimately related to the diagnosis of the disease, the role of plasmablasts in the pathophysiology remains an active area of discussion. Recent studies have uncovered a possible role for CD4-positive cytotoxic T lymphocytes, T follicular helper cells, and M2 macrophages. The clinical presentation is variable and can be vague, as this disease affects many organs and new presentations are continuing to be described. The diagnosis depends on clinical and histopathological assessment. The mainstay of treatment is with glucocorticoids, but rituximab has recently shown promise. Monitoring disease activity using imaging modalities (including positron emission tomography) and serum markers is imperative, as relapses are common. IgG4-related disease spans many medical disciplines but is a treatable condition with which all clinicians should be familiar.

scholarly journals A rare case of long-term paraesthesia diagnosed as a paraneoplastic syndrome by anti-SOX1 antibody determination

2019 ◽  
Vol 12 (7) ◽  
pp. e228916
Author(s):  
Oriol Mirallas ◽  
Nuria Rial ◽  
Berta Martín-Cullell ◽  
Jesus Recio-Iglesias
Keyword(s):  
Lung Cancer ◽  
Cervical Lymphadenopathy ◽  
Strength Loss ◽  
Malignant Tumour ◽  
Multiple Organ ◽  
Rare Presentation ◽  
Organ Systems ◽  
Myasthenic Syndrome ◽  
Chest X Ray

Paraneoplastic syndromes (PS) are a rare presentation of cancer, most commonly associated with small cell lung cancer (SCLC), breast cancer and haematologic malignancies. The diagnosis of PS is challenging because it could affect multiple organ systems and it may present before the tumour is visible by imaging. We report a malignant tumour diagnosed in a male patient who referred long-term paraesthesia and proximal muscle strength loss. After ruling out common causes of polyneuropathy, the anti-SOX1 antibody gave light to the diagnosis. A pulmonary opacity in the upper right lobe was observed in the chest X-ray and a pulmonary tumour was later confirmed by CT scan. The biopsy of the cervical lymphadenopathy determined an SCLC, which caused a PS called Lambert-Eaton myasthenic syndrome (LEMS). Our case raises awareness of a rare PS presentation, which can be diagnosed by specific antibodies, allowing early diagnosis and treatment of lung cancer.

scholarly journals Coronavirus Disease 2019 (COVID-19): Review on Transmission, Clinical Presentations, Treatments and Vaccines

2021 ◽  
pp. 92-114
Author(s):  
Syed Arman Rabbani ◽  
Shrestha Sharma ◽  
Supriya Mishra ◽  
Asiya Mahtab
Keyword(s):  
Clinical Presentation ◽  
Close Contact ◽  
Multiple Organ ◽  
Published Data ◽  
The People ◽  
Number Of Factors ◽  
Clinical Presentations ◽  
Organ Systems ◽  
Asymptomatic Infections ◽  
Respiratory Droplets

Coronavirus disease 2019 (COVID-19) has become one of the most prevalent and significant global health concerns since its origin in Wuhan, China in the December 2019. As on 05th April, this disease has affected over 131 million people and has resulted in more than 2.85 million deaths worldwide till date. The disease is transmitted from the infected patients to the people in close contact through respiratory droplets. There are a number of factors which affect the transmission of this disease. The clinical presentation of COVID-19 can range from asymptomatic infections to critical disease leading to respiratory failure, septic shock and multiple organ failure. The disease essentially affects the respiratory system but other organ systems may also be involved. Currently, the therapeutic management of the disease is based on the severity of the infection and employs various strategies like monoclonal antibodies, corticosteroids, antivirals, stem cell therapy and immunomodulators. Scientists and researchers all around the world have raced against the time and developed a number of vaccines for COVID-19.  At present, there are many COVID-19 vaccines, which have been authorized or approved for use by the regulators of the different countries. This article reviews the current published data on the COVID-19 transmission, clinical presentations, treatments and vaccines.

Organ system effects and reinfection of COVID-19: A systematic review

2021 ◽  
Vol 9 (1) ◽  
pp. 6-6
Author(s):  
Inderbir Padda ◽  
Nimrat Khehra ◽  
Urooj Jaferi ◽  
Dina Mosabbeh ◽  
Harshan Atwal ◽  
...  
Keyword(s):  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Kidney Injury ◽  
Multiple Organ ◽  
Organ System ◽  
Fulminant Myocarditis ◽  
Preventative Measures ◽  
Organ Systems ◽  
The Central Nervous System

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its effects on the organ systems have been summarized in recent literature with predominant pulmonary characteristics as a hallmark of the COVID-19 virus. Considering its accelerated appearance from Wuhan, China, in December 2019, extrapulmonary effects have been reported globally of SARS-CoV-2 involving the central nervous system (CNS), cardiovascular, gastrointestinal, renal, and hematologic systems; thus, the potential mechanisms, pathophysiology, clinical characteristics, management, outcome, and case reports per organ system are summarized in depth. The authors interpreted articles composed of case reports, case-series, meta-analysis, cohort studies, retrospective studies, and narrative reviews focusing on COVID-19 confirmed cases and their effects on the organ systems. Prevalent clinical organ system complexities include pneumonia, acute respiratory distress syndrome (ARDS), pulmonary hypertension, pulmonary embolism, hypertension, cardiac arrhythmias, myocarditis progressing to fulminant myocarditis, anorexia, nausea, vomiting, diarrhea, liver dysfunction, encephalopathy, encephalitis, meningitis, intracerebral hemorrhage, acute kidney injury, and hypercoagulability causing stroke and disseminated intravascular coagulation (DIC). This comprehensive literature review article will help clinicians and researchers gain insight about SARS-CoV-2 and its diverse effects on multiple organ systems involved, therefore help implement prospective management and preventative measures.

scholarly journals Acute respiratory failure as primary manifestation of antineutrophil cytoplasmic antibodies-associated vasculitis

2014 ◽  
Vol 4 (2) ◽  
Author(s):  
Evdokia Sourla ◽  
Vasilis Bagalas ◽  
Helias Tsioulis ◽  
Asimina Paspala ◽  
Sofia Akritidou ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Acute Respiratory Failure ◽  
Distress Syndrome ◽  
Clinical Presentation ◽  
Ventilatory Support ◽  
Multiple Organ ◽  
Alveolar Hemorrhage ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Organ Systems ◽  
Hypoxic Respiratory Failure

The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a lifethreatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.

Arteriovenous Hemofiltration in Children with Multiple Organ System Failure

1987 ◽  
Vol 10 (4) ◽  
pp. 233-238 ◽  
Author(s):  
G. Zobel ◽  
M. Trop ◽  
E. Ring ◽  
H.M. Grubbauer
Keyword(s):  
Critically Ill ◽  
Caloric Intake ◽  
Multiple Organ ◽  
Organ System ◽  
Critically Ill Children ◽  
System Failure ◽  
Multiple Organ System ◽  
Organ Systems ◽  
Organ System Failure ◽  
Multiple Organ System Failure

In the last year nine critically ill children with multiple organ system failure (MOSF) were treated by continuous arteriovenous hemofiltration (CAVH). The mean number of organ systems involved was five per patient (range 4 to 7). Mean duration of hemofiltration was 136 hours (range 10 to 432 h.). Mean ultrafiltration rates of 6.4 ± 3.0 ml/m2 b.s./min achieved mean serum creatinine levels of 2.39 ± 1.49 mg/dl. Hypervolemia and pulmonary edema were corrected rapidly by CAVH. Total parenteral nutrition with a mean caloric intake of 62 ± 15 kcal/kg b.w./day was provided throughout the hemofiltration period. In addition high ultrafiltration rates allowed delivery of large amounts of i.v. medications. CAVH is an ideal extracorporeal renal replacement system to control azotemia, fluid, electrolyte, and acid-base status in critically ill children. It carries the potential to improve survival rate in children with MOSF.

Pheochromocytoma is an Eventful Tumor: A Case Description

2010 ◽  
Vol 2 (3) ◽  
pp. 139-141
Author(s):  
Bhargav PRK ◽  
Amar V
Keyword(s):  
Unique Feature ◽  
Multiple Organ ◽  
Organ System ◽  
Case Description ◽  
Organ Systems ◽  
Major Organ ◽  
Adrenal Pheochromocytoma ◽  
Successful Removal ◽  
Hypertensive Crises ◽  
Major Organ System

ABSTRACT Pheochromocytoma is a tumor arising from adrenal medulla in > 90% of cases. It is a great mimic, featuring in differential diagnosis of disorders of multiple organ systems. Here we present a prototypical case of right adrenal pheochromocytoma, highlighting this unique feature of the tumor. It presented with repeated hypertensive crises with predominant involvement of one major organ system in each episode. All of them were reversible with timely intervention and successful removal of the tumor, highlighting the functional basis rather than organic pathology.

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