scholarly journals Accuracy of the Criteria for Hemophagocytic Lymphohistiocytosis

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S106-S107
Author(s):  
A Aksionau ◽  
E Wei
Keyword(s):  
Diagnostic Criteria ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Hemophagocytic Syndrome ◽  
Treatment Options ◽  
Medical Documentation ◽  
Genetic Abnormalities ◽  
Human Pathology ◽  
Spleen Sample ◽  
Threatening Condition ◽  
Life Threatening

Abstract Introduction/Objective Regular assessment and analysis of diagnostic criteria for any type of human pathology is a prerequisite for ensuring the highest level of patient care. Hemophagocytic lymphohistiocytosis, also known as hemophagocytic syndrome, is a life-threatening condition. The syndrome can develop in critically ill patients with malignancies, severe infections, during chemotherapy, and may be associated with currently known genetic abnormalities, but this list is incomplete. We draw attention to the accuracy of diagnostic criteria, association with a variety of clinical conditions, pathophysiological mechanisms, and outcomes of the disease. Methods From the medical records in our hospital, we were able to extract several cases over a 10-year period. Based on hemophagocytosis features, our list included 13 patients representing 11 bone marrow aspirates, 4 lymph node biopsies, 1 liver biopsy, and 1 spleen sample; repeated examination of the slides confirmed the accuracy. Analyzing medical documentation, we evaluated the sequence and competence of the criteria used, the time required for diagnosis, management, and outcomes. Results We found that not all criteria were used for diagnosis, and the most sensitive and specific tests were bypassed. The preliminary diagnosis was made by a consultant (a rheumatologist or an oncologist-hematologist) on the 5th day of the hospital stay that delayed treatment. Of all the available treatment options, only a few were used. Conclusion The hemophagocytic syndrome is a very rare and fatal entity, it requires highly sensitive and specific diagnostic criteria for prompt diagnosis, targeted management, and thorough follow up. Every patient admitted to the hospital with a life-threatening condition should be suspected and tested for hemophagocytic syndrome on the first day. The criteria for hemophagocytic lymphohistiocytosis should be revised, with the most sensitive and specific ones are done in 100% of cases. Subsequently, each patient should be tested for the presence of genetic abnormalities that correlate with the syndrome.

Bradyarrhythmias

2016 ◽  
Author(s):  
Inna Leybell ◽  
Liliya Abrukin
Keyword(s):  
Atrioventricular Block ◽  
Sinus Bradycardia ◽  
Sinus Node ◽  
Treatment Options ◽  
Presenting Symptoms ◽  
Threatening Condition ◽  
Node Disease ◽  
Life Threatening ◽  
Sinus Node Disease ◽  
Sinoatrial Block

Bradyarrhythmias can present as an incidental electrocardiographic (ECG) finding or a life-threatening condition requiring immediate intervention. They are caused by sinus node disease or atrioventricular block. This review covers pathophysiology, stabilization and assessment, diagnosis and treatment options, and disposition and outcomes for patients with bradycardia. Figures in the review demonstrate characteristic ECG tracings. Tables list classifications of sinus node dysfunction as well as of sinoatrial and atrioventricular blocks, presenting symptoms, important information to elicit on history taking, various causes of bradycardia, and specific interventions for toxicologic etiologies. Key words: atrioventricular block, atropine, bradyarrhythmia, bradycardia, first-degree atrioventricular block, second-degree atrioventricular block, sinoatrial block, sinus bradycardia, sinus node disease, tachycardia-bradycardia syndrome, third-degree atrioventricular block This review contains 9 highly rendered figures, 7 tables, and 92 references.

scholarly journals Human Adenovirus 7-Associated Hemophagocytic Lymphohistiocytosis-like Illness: Clinical and Virological Characteristics in a Cluster of Five Pediatric Cases

2020 ◽  
Author(s):  
William R Otto ◽  
Edward M Behrens ◽  
David T Teachey ◽  
Daryl M Lamson ◽  
David M Barrett ◽  
...  
Keyword(s):  
Genetic Predisposition ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Human Adenovirus ◽  
Adenovirus Type ◽  
Definitive Treatment ◽  
Cell Transplant ◽  
Hematopoietic Stem ◽  
Threatening Condition ◽  
Epidemiology Data ◽  
Life Threatening

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of immune dysregulation. Children often suffer from primary genetic forms of HLH, which can be triggered by infection. Others suffer from secondary HLH as a complication of infection, malignancy, or rheumatologic disease. Identifying the exact cause of HLH is crucial, as definitive treatment for primary disease is hematopoietic stem cell transplant. Adenoviruses have been associated with HLH but molecular epidemiology data are lacking. Methods We describe the clinical and virologic characteristics of 5 children admitted with adenovirus infection during 2018–2019 who developed HLH or HLH-like illness. Detailed virologic studies, including virus isolation and comprehensive molecular typing were performed. Results All patients recovered; clinical management varied but included immunomodulating and antiviral therapies. A genetic predisposition for HLH was not identified in any patient. Adenovirus isolates were recovered from 4/5 cases; all were identified as genomic variant 7d. Adenovirus type 7 DNA was detected in the fifth case. Phylogenetic analysis of genome sequences identified 2 clusters—1 related to strains implicated in 2016–2017 outbreaks in Pennsylvania and New Jersey, the other related to a 2009 Chinese strain. Conclusions It can be challenging to determine whether HLH is the result of an infectious pathogen alone or genetic predisposition triggered by an infection. We describe 5 children from the same center presenting with an HLH-like illness after onset of adenovirus type 7 infection. None of the patients were found to have a genetic predisposition to HLH. These findings suggest that adenovirus 7 infection alone can result in HLH.

scholarly journals Severe Fever with Thrombocytopenia Syndrome Virus Infection Associated with Hemophagocytic Lymphohistiocytosis as Poor Prognostic Factor

2017 ◽  
Vol 4 (suppl_1) ◽  
pp. S319-S319
Author(s):  
Sunmin Park ◽  
Juwon Kim ◽  
Hyo Youl Kim ◽  
Young Uh ◽  
Young Keun Kim
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Features ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Inflammatory Diseases ◽  
Clinical Feature ◽  
Poor Prognostic Factor ◽  
Immune Mediated ◽  
Life Threatening ◽  
Christian Hospital ◽  
Severe Fever

Abstract Background Severe fever with thrombocytopenia (SFTS) is an emerging infectious disease caused by a novel bunyavirus designated SFTS virus (SFTSV) with a high fatality rate. Hemophagocytic lymphohistiocytosis (HLH) is an immune-mediated life-threatening disease triggered by infections, neoplasms and noninfectious inflammatory diseases. A few HLH associated with SFTSV were reported. According to the diagnostic criteria of HLH, 11 patients with SFTS were reviewed. Methods During last 2 years (2015–2016), 11 SFTS patients were diagnosed at the Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, South Korea. Clinical features were analyzed using diagnostic criteria of 2004-HLH trial. We described if the prognosis of SFTSV-infected patients was associated with clinical features of HLH. Results Of 11 patients, four patients were fulfillled the diagnostic criteria of 2004-HLH trial (five of eight criteria). Two patients were fulfilled the four criteria. Five patients were fulfilled three or less criteria. Three of six patients who fulfilled four or more criteria were died. There was no mortality in five patients who fulfilled three or less criteria. Hemophagocytosis in bone marrow (BM) was observed in all six patients who were taken BM study. Conclusion In SFTS, HLH was severe clinical feature and it might be associated with poor prognosis. Disclosures All authors: No reported disclosures.

scholarly journals Aortic Dissection in a Pregnant Patient without Other Risk Factors

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Priya V. Patel ◽  
Raanan Alter ◽  
Recia Frenn ◽  
Thaddeus P. Waters
Keyword(s):  
Risk Factors ◽  
Aortic Dissection ◽  
Aortic Rupture ◽  
Treatment Options ◽  
Pregnant Patient ◽  
Physical Exam ◽  
Vascular Events ◽  
Increased Risk ◽  
Threatening Condition ◽  
Life Threatening

Background. An aortic dissection is a life-threatening condition in which the intima of the artery tears causing separation of the intima and media. Pregnancy places women at a significantly increased risk of common vascular events including venous thromboembolism, myocardial infarction, and stroke, while also increasing the risk of rarer vascular events such as aortic dissection and aortic rupture. Case. A 30-year-old previously healthy multiparous woman presenting at 36 weeks of pregnancy with a Type A aortic dissection. She underwent a combined emergent cesarean delivery followed by repair of her aortic root. Conclusions. Aortic dissection should be high on the differential for pregnant patients presenting with the characteristic complaints and physical exam findings given the high mortality rate associated with this vascular event. Teaching Points. (1) This report reviews the characteristic presentation, risk factors, and physical exam findings in a patient with an aortic dissection. (2) The report includes treatment options for pregnant patients based on the classification of the dissection.

Dysphagia as an interdisciplinary problem. Difficulties in the diagnostic process of the vascular anomaly: a right-sided aortic arch with aberrant left subclavian artery (case report)

2018 ◽  
Vol 7 (1) ◽  
pp. 35-43
Author(s):  
Barbara Maciejewska ◽  
Karolina Kania ◽  
Piotr Kowal ◽  
Bożena Wiskirska-Woźnica
Keyword(s):  
Aortic Arch ◽  
Subclavian Artery ◽  
Left Subclavian Artery ◽  
Treatment Options ◽  
Vascular Anomaly ◽  
Differential Diagnostics ◽  
Diagnostic Process ◽  
Arteria Lusoria ◽  
Threatening Condition ◽  
Life Threatening

Dysphagia is an underestimated health problem. At the same time, it is a potentially life-threatening condition. Dysphagia in young adults is rare and thus it is rarely discussed in the literature. Vascular anomalies are much less frequent causes of dysphagia than structural pathologies of the digestive system, iatrogenic lesions or neurological causes. This paper presents a case of a 21-year-old woman with escalating dysphagia in the course of a congenital vascular anomaly in the form of a right-sided aortic arch with retroesophageal left subclavian artery (left arteria lusoria) and compression. The paper highlights delayed symptomatology of the congenital defect, reasons behind the long-lasting diagnostic process, and the role of the laryngological – phoniatric examination in order to exclude oral and pharyngeal causes. The differential diagnostics and treatment options are discussed thereinafter.

scholarly journals 36-Year-Old Female with Catastrophic Antiphospholipid Syndrome Treated with Eculizumab: A Case Report and Review of Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Marianna Strakhan ◽  
Mariana Hurtado-Sbordoni ◽  
Nahun Galeas ◽  
Kamila Bakirhan ◽  
Karenza Alexis ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Treatment Options ◽  
Immunoglobulin Therapy ◽  
High Rate ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Laboratory Findings ◽  
Intravenous Immunoglobulin Therapy ◽  
Short Period ◽  
Threatening Condition ◽  
Life Threatening

Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor.

scholarly journals Hemophagocytic Lymphohistiocytosis Associated With Parvovirus B19 in a Patient With Acquired Immunodeficiency Syndrome

2019 ◽  
Vol 7 ◽  
pp. 232470961988369
Author(s):  
Precious Macauley ◽  
Mohammad Abu-Hishmeh ◽  
Carissa Dumancas ◽  
Vijay Alexander-Rajan ◽  
Fernando Piedra-Chavez ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Parvovirus B19 ◽  
Signs And Symptoms ◽  
High Serum ◽  
Intrathecal Methotrexate ◽  
Immunodeficiency Virus ◽  
Threatening Condition ◽  
Life Threatening ◽  
Acquired Immunodeficiency ◽  
Immunodeficiency Syndrome

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by widespread inflammation due to massive immune activation and cytokine release. It is of 2 types, primary or familial and secondary or acquired. Diagnosis is made by fulfilling 5 of 8 criteria as determined by the Histiocyte Society. Treatment includes etoposide, dexamethasone, with or without intrathecal methotrexate in the presence of neurologic involvement as well as treating the underlying cause in secondary HLH. We present a case of a 23-year-old female with congenital human immunodeficiency virus (HIV) infection who presents with nonspecific signs and symptoms of cough, fever, leukopenia, and anemia, and a high-serum parvovirus B19 DNA, later diagnosed with HLH and treated with etoposide and dexamethasone. She made clinical improvements and was successfully discharged to home after 26 days of admission.

scholarly journals 1011. Sepsis and Secondary Hemophagocytic Lymphohistiocytosis

2018 ◽  
Vol 5 (suppl_1) ◽  
pp. S301-S301 ◽  
Author(s):  
Farhan Fazal ◽  
Naveet Wig ◽  
Manish Soneja ◽  
Dipendra K Mitra ◽  
Sk Panda ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Nk Cell ◽  
Immunosuppressive Treatment ◽  
Cell Activity ◽  
Unknown Etiology ◽  
Laboratory Features ◽  
Life Threatening ◽  
Non Hodgkins Lymphoma ◽  
Staphylococcal Aureus

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition diagnosed by HLH 2004 criteria. This criterion has common clinical and laboratory features with sepsis and tropical fevers, but there is marked difference in management and outcome of these two entities. The study is conducted to know whether there is any difference in the clinico-laboratory features, management, and outcome of sepsis with or without secondary HLH. Methods This is a prospective observational study where patients presenting with sepsis and bicytopenia are included. The patients underwent relevant investigations according to 2004 HLH diagnostic criteria. The patients are divided into sepsis with or without HLH. The underlying etiology, treatment, and outcome of the two groups are analysed. Results Fifty sepsis patients are included in the study, out of which 28 fulfilled the HLH diagnostic criteria which comprised of 18 men and 10 women. The etiology were bacterial (three enteric fever, three tuberculosis, two scrub typhus, one Staphylococcal aureus), viral (one dengue fever, two HIV, two encephalitis), fungal (one aspergillosis, one mucormycosis, two others), parasites (three malaria, one leishmania) malignancy (two hodgkin lymphoma, one non-Hodgkins lymphoma), and unknown etiology in six patients, with >1 etiology in three patients (Figure 2). The percentage of each criterion fulfilled in both groups is given in Figure 1, showing an increased occurrence of splenomegaly, low NK cell activity, hypertriglyceridemia in HLH patients. Steroids along with supportive treatment was given to 53% and etoposide was added in 7%. Treatment for underlying etiology alone without immunosuppressive treatment was given in 39%. The mortality in those with HLH vs. without HLH was 42% and 31%, respectively. The median duration of hospital stay was 18 and 36 days in HLH and without HLH group, respectively. Conclusion HLH should be suspected in sepsis patients with bicytopenia specially in tropical fevers. There is increased mortality if the sepsis patients fulfil HLH criteria. Early diagnosis and management is of paramount importance. Disclosures All authors: No reported disclosures.

The Power of Words: Discussing Decision Making and Prognosis

2013 ◽  
pp. 442-446
Author(s):  
Don S. Dizon ◽  
Mary C. Politi ◽  
Anthony L. Back
Keyword(s):  
Decision Making ◽  
Personalized Medicine ◽  
Treatment Options ◽  
Care Planning ◽  
Shared Decision ◽  
Patient Relationship ◽  
Threatening Condition ◽  
Life Threatening ◽  
Advance Care ◽  
Power Of Words

One of the most important skills in medicine is communication. It lies at the heart of the doctor-patient relationship, and is particularly important when one has been diagnosed with a potentially life-threatening condition. Words are powerful and too often can be interpreted in ways not intended. In our session at the 2013 ASCO Annual Meeting, we discuss the communication of cancer and ways we might want to consider discussions regarding treatment options and prognosis. We recognize that all patients are different and that approaches should be individualized, to reflect each person's needs (what they want to know) while respecting their limits (how much they want to know). We discuss the concept of shared decision making, and how it can be used when there is uncertainty in what treatments may (or may not) accomplish. Finally, we discuss the skills that constitute a toolkit for communication, which we hope can be tailored to meet the variable needs of those we are caring for and by doing so, can be of help to clinicians in their own practices. In the era of personalized medicine, treatments may become more complex, and more options may be available. We hope to encourage providers to welcome patients as active participants in their care by sharing information, requesting their input, and by engaging them in important processes such as advance care planning—to ensure their needs and wishes are respected in the present and for whatever may come in the future.

Neuroleptic Malignant Syndrome: A Review for Intensivists

1994 ◽  
Vol 9 (5) ◽  
pp. 227-234 ◽  
Author(s):  
Laura M. Prager ◽  
Frederick H. Millham ◽  
Stern A. Theodore
Keyword(s):  
Risk Factors ◽  
Intensive Care ◽  
Diagnostic Criteria ◽  
Neuroleptic Malignant Syndrome ◽  
Critically Ill ◽  
Critically Ill Patients ◽  
Treatment Options ◽  
Accurate Diagnosis ◽  
Case Examples ◽  
Life Threatening

Neuroleptic malignant syndrome (NAIS) is arare, potentially life-threatening disorder that results from the use of neuroleptics, a class of drugs that is being used with increasing frequency in intensive care units (ICUs). Unfortunately, critically ill patients typically have coexisting conditions that make accurate diagnosis of NAIS difficult and complicate treatment decisions. Diagnostic criteria, risk factors, and treatment options for NMS are discussed; case examples are provided.

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