A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

Abstract The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

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A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. II. Clinical manifestations

Blood ◽

10.1182/blood.v58.5.1021.bloodjournal5851021 ◽

1981 ◽

Vol 58 (5) ◽

pp. 1021-1026 ◽

Cited By ~ 3

Author(s):

RT Schooley ◽

MA Flaum ◽

HR Gralnick ◽

AS Fauci

Keyword(s):

Hypereosinophilic Syndrome ◽

Clinical Manifestations ◽

Initial Therapy ◽

Multiple Organ ◽

Cytotoxic Agents ◽

Grading System ◽

Idiopathic Hypereosinophilic Syndrome ◽

Splenic Enlargement ◽

Organ Systems ◽

Clinical Grading

The idiopathic hypereosinophilic syndrome, a disorder characterized by peripheral blood and bone marrow eosinophilia associated with single or multiple organ system dysfunction attributable to tissue invasion by eosinophils has, in the past, been associated with an extremely poor prognosis. Recently, we reported the favorable impact of a therapeutic protocol consisting of prednisone and/or hydroxyurea on the morbidity and mortality of this syndrome. We have reviewed the clinical and hematologic features upon admission and the subsequent clinical courses of 32 patients with this disease referred to the NIH between 1965 and 1979 in an effort to determine which features suggest a more rapidly progressive course. A grading system based on 22 clinical features involving the 8 organ systems commonly affected by the illness was devised. The disease followed a more aggressive course in patients with evidence of cardiac or neurologic dysfunction at the time of initial NIH evaluation. Although splenomegaly, in and of itself, caused little morbidity, splenic enlargement at presentation appeared to be a predictor of a more aggressive course. The clinical grading system accurately predicted which patients would require no specific antihypereosinophilic therapy, which patients would respond adequately to corticosteroids, and which patients would require therapy with cytotoxic agents. It is proposed that this clinical grading system, and the hematologic grading system outlined in the accompanying report be used as aids in the selection of initial therapy in this group of patients.

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Leukoencephalopathy and cerebral edema as the presenting manifestations of SLE in an ANA-negative adolescent female: A case report and review of literature.

10.21203/rs.2.22477/v1 ◽

2020 ◽

Author(s):

Alexandra Theisen ◽

Paroma Bose ◽

Christina Knight ◽

Melissa Oliver

Keyword(s):

Cerebral Edema ◽

Lupus Erythematosus ◽

Primary Care Physicians ◽

Neuropsychiatric Symptoms ◽

Clinical Manifestations ◽

Central Nervous System Involvement ◽

Multiple Organ ◽

Small Subset ◽

Adolescent Female ◽

Organ Systems

Abstract Background: Systemic lupus erythematosus (SLE) is an autoimmune disease with various clinical manifestations involving multiple organ systems. Neuropsychiatric manifestations of SLE has been associated with increased morbidity and mortality, thus it is important to recognize and diagnosis the disease entity and treat early. When neuropsychiatric symptoms are involved, typically there are many other systemic features to aid in the diagnosis of SLE. Many autoantibodies have been discovered and are used to help diagnose SLE. The antibody present in most cases of pediatric SLE, as well as in many other rheumatic diseases, is the nonspecific antinuclear antibody (ANA), making it a commonly used screening tool by primary care physicians when evaluating a patient with a possible rheumatic disorder. However, a small subset of SLE patients, 1-5%, present with a negative ANA, and it is important to keep SLE on the differential diagnosis in specific instances when a thorough infectious and neurological workup has been completed and proven to be inconclusive. Case Presentation: This case involves a Hispanic adolescent female with a negative ANA who presented with diffuse cerebral edema secondary to leukoencephalopathy due to SLE with central nervous system involvement. She had an extensive workup while inpatient involving metabolism, infectious disease, rheumatology, and neurology prior to obtaining the diagnosis of SLE. She was treated with both cyclophosphamide and rituximab and showed improvement. Conclusions: A review of the literature revealed 8 cases with SLE presenting with or developing diffuse cerebral edema and/or leukoencephalopathy. Our patient’s case differs in that she was also ANA negative despite other autoantibody positivity. While she did have low complements and transient leukopenia, she did not present with other signs of organ involvement, which made the diagnosis of SLE with neuropsychiatric involvement quite challenging. We discuss the importance of keeping SLE on the differential despite a negative ANA in complex cases without any other cause and to consider initial screening with not only the ANA but also dsDNA and complements to avoid missed diagnoses.

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Biologics in the Treatment of Lupus Erythematosus: A Critical Literature Review

BioMed Research International ◽

10.1155/2019/8142368 ◽

2019 ◽

Vol 2019 ◽

pp. 1-17 ◽

Cited By ~ 12

Author(s):

Dominik Samotij ◽

Adam Reich

Keyword(s):

Clinical Trials ◽

Lupus Erythematosus ◽

Unmet Need ◽

Clinical Manifestations ◽

Immunosuppressive Drugs ◽

Multiple Organ ◽

Biologic Agents ◽

Drug Induced ◽

Treatment Regimes ◽

Organ Systems

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease affecting multiple organ systems that runs an unpredictable course and may present with a wide variety of clinical manifestations. Advances in treatment over the last decades, such as use of corticosteroids and conventional immunosuppressive drugs, have improved life expectancy of SLE sufferers. Unfortunately, in many cases effective management of SLE is still related to severe drug-induced toxicity and contributes to organ function deterioration and infective complications, particularly among patients with refractory disease and/or lupus nephritis. Consequently, there is an unmet need for drugs with a better efficacy and safety profile. A range of different biologic agents have been proposed and subjected to clinical trials, particularly dedicated to this subset of patients whose disease is inadequately controlled by conventional treatment regimes. Unfortunately, most of these trials have given unsatisfactory results, with belimumab being the only targeted therapy approved for the treatment of SLE so far. Despite these pitfalls, several novel biologic agents targeting B cells, T cells, or cytokines are constantly being evaluated in clinical trials. It seems that they may enhance the therapeutic efficacy when combined with standard therapies. These efforts raise the hope that novel drugs for patients with refractory SLE may be available in the near future. This article reviews the current biological therapies being tested in the treatment of SLE.

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Idiopathic hypereosinophilic syndrome presenting with multiple organ damage

Medicine ◽

10.1097/md.0000000000014532 ◽

2019 ◽

Vol 98 (10) ◽

pp. e14532

Author(s):

Peng Wu ◽

Yuanguo Chen ◽

Haibo Zhang ◽

Chen Li

Keyword(s):

Hypereosinophilic Syndrome ◽

Organ Damage ◽

Multiple Organ ◽

Idiopathic Hypereosinophilic Syndrome

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Response to Vincristine Treatment in a Case of Idiopathic Hypereosinophilic Syndrome with Multiple Clinical Manifestations

Acta Haematologica ◽

10.1159/000206350 ◽

1984 ◽

Vol 72 (1) ◽

pp. 21-25 ◽

Cited By ~ 19

Author(s):

E. Cofrancesco ◽

M. Cortellaro ◽

E. Pogliani ◽

C. Boschetti ◽

M. Salvatore ◽

...

Keyword(s):

Hypereosinophilic Syndrome ◽

Clinical Manifestations ◽

Idiopathic Hypereosinophilic Syndrome

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Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Blood ◽

10.1182/blood-2014-03-561381 ◽

2014 ◽

Vol 124 (4) ◽

pp. 483-492 ◽

Cited By ~ 285

Author(s):

Eli L. Diamond ◽

Lorenzo Dagna ◽

David M. Hyman ◽

Giulio Cavalli ◽

Filip Janku ◽

...

Keyword(s):

Clinical Management ◽

Clinical Laboratory ◽

Clinical Manifestations ◽

Multiple Organ ◽

Future Research ◽

Consensus Guidelines ◽

Erdheim Chester Disease ◽

Organ Systems ◽

Erdheim Chester ◽

Chester Disease

Abstract Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis. Recent findings suggest that ECD is a clonal disorder, marked by recurrent BRAFV600E mutations in >50% of patients, in which chronic uncontrolled inflammation is an important mediator of disease pathogenesis. Although ∼500 to 550 cases have been described in the literature to date, increased physician awareness has driven a dramatic increase in ECD diagnoses over the last decade. ECD frequently involves multiple organ systems and has historically lacked effective therapies. Given the protean clinical manifestations and the lack of a consensus-derived approach for the management of ECD, we provide here the first multidisciplinary consensus guidelines for the clinical management of ECD. These recommendations were outlined at the First International Medical Symposium for ECD, comprised of a comprehensive group of international academicians with expertise in the pathophysiology and therapy of ECD. Detailed recommendations on the initial clinical, laboratory, and radiographic assessment of ECD patients are presented in addition to treatment recommendations based on critical appraisal of the literature and clinical experience. These formalized consensus descriptions will hopefully facilitate ongoing and future research efforts in this disorder.

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Breast abscess due to Salmonella species: a brief review of epidemiology, clinical manifestations, serological correlation and management

International Journal of Community Medicine and Public Health ◽

10.18203/2394-6040.ijcmph20211283 ◽

2021 ◽

Vol 8 (4) ◽

pp. 2077

Author(s):

Kinjal P. Patel ◽

Rutuja Darne ◽

Reshma Poojary ◽

Virendra Kinjale

Keyword(s):

Case Reports ◽

Clinical Manifestations ◽

Multiple Organ ◽

Oral Antibiotic ◽

Breast Abscess ◽

Drug Of Choice ◽

Gastrointestinal Pathogen ◽

Organ Systems ◽

Antibiotic Agents ◽

Intestinal Complication

Breast abscess due to Salmonella is an uncommon but well recognized extra-intestinal complication of enteric fever. Salmonella species is commonly identified as a gastrointestinal pathogen causing bacteremia but inappropriately treated cases can led to dissemination in multiple organ systems resulting in localized abscess formation. Case reports have been described in literature about extra-intestinal abscesses by both S. enterica serotype typhi and paratyphi. Whilst recently, there has been an upsurge in the frequency of case reports related to Salmonella breast abscess, reason may be due to emergence of resistant Salmonella strains. Usually uncomplicated abscesses show effective clearance with single course of oral antibiotic agents together with surgery. Reviews have recommended azithromycin as a drug of choice especially in the presence of fluoroquinolones resistance. So, acknowledging prevalence of breast abscess due to salmonella is utmost important.

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Characterizing Long COVID: Deep Phenotype of a Complex Condition

10.1101/2021.06.23.21259416 ◽

2021 ◽

Author(s):

Rachel R Deer ◽

Madeline A Rock ◽

Nicole Vasilevsky ◽

Leigh C Carmody ◽

Halie M Rando ◽

...

Keyword(s):

Meta Analysis ◽

Clinical Manifestations ◽

Analysis Data ◽

Clinical Findings ◽

Multiple Organ ◽

Diverse Range ◽

Human Phenotype ◽

Organ Systems ◽

Novel Coronavirus

Importance: Since late 2019, the novel coronavirus SARS-CoV-2 has given rise to a global pandemic and introduced many health challenges with economic, social, and political consequences. In addition to a complex acute presentation that can affect multiple organ systems, there is mounting evidence of various persistent long-term sequelae. The worldwide scientific community is characterizing a diverse range of seemingly common long-term outcomes associated with SARS-CoV-2 infection, but the underlying assumptions in these studies vary widely making comparisons difficult. Numerous publications describe the clinical manifestations of post-acute sequelae of SARS-CoV-2 infection (PASC or long COVID), but they are difficult to integrate because of heterogeneous methods and the lack of a standard for denoting the many phenotypic manifestations of long COVID. Observations: We identified 303 articles published before April 29, 2021, curated 59 relevant manuscripts that described clinical manifestations in 81 cohorts of individuals three weeks or more following acute COVID-19, and mapped 287 unique clinical findings to Human Phenotype Ontology (HPO) terms. Conclusions and Relevance: Patients and clinicians often use different terms to describe the same symptom or condition. Addressing the heterogeneous and inconsistent language used to describe the clinical manifestations of long COVID combined with the lack of standardized terminologies for long COVID will provide a necessary foundation for comparison and meta-analysis of different studies. Translating long COVID manifestations into computable HPO terms will improve the analysis, data capture, and classification of long COVID patients. If researchers, clinicians, and patients share a common language, then studies can be compared or pooled more effectively. Furthermore, mapping lay terminology to HPO for long COVID manifestations will help patients assist clinicians and researchers in creating phenotypic characterizations that are computationally accessible, which may improve the stratification and thereby diagnosis and treatment of long COVID.

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Hemophagocytic Lymphohistiocytosis: A Life Threatening Cytopenic Condition

Faridpur Medical College Journal ◽

10.3329/fmcj.v15i2.53897 ◽

2021 ◽

Vol 15 (2) ◽

pp. 98-102

Author(s):

Suranjit Kumar Saha ◽

MM Shahin Ul Islam ◽

Nasir Uddin Ahmed ◽

Prativa Saha

Keyword(s):

Hemophagocytic Lymphohistiocytosis ◽

Clinical Manifestations ◽

Specific Treatment ◽

Gene Mutations ◽

Multiple Organ ◽

Treatment Modalities ◽

Hematopoietic Stem ◽

Organ Systems ◽

Life Threatening ◽

Poor Outcomes

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that occurs in many underlying conditions in all age. This is characterized by unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells and macrophages resulting in raised cytokine level. Those cytokines and immune mediated injury occur in multiple organ systems. It may be primary and secondary. Primary HLH is familial, childhood presentation and associated with gene mutations. Secondary HLH is acquired, adulthood presentation that occurs in infections, malignancies inflammatory and autoimmune diseases etc. Clinical manifestations include fever, splenomegaly, lymphadenopathy, neurologic dysfunction, coagulopathy, features of sepsis etc. Laboratory investigation includes cytopenias, hypertriglyceridemia, hyperferritinemia, abnormal liver function, hemophagocytosis, and diminished NKcell activity. Treatment modalities include immunosuppressive, immunomodulatory agents, cytostatic drugs, T-cell antibodies, anticytokine agents and hematopoietic stem cell transplantation (HSCT). Besides those, aggressive supportive care combined with specific treatment of the precipitating factor can produce better outcome. With treatment more than 50% of children who undergo transplant survive, but adults have quite poor outcomes even with aggressive management. Faridpur Med. Coll. J. 2020;15(2): 98-102

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