scholarly journals Clinical Characteristics of Hemophagocytic Syndrome: 46 Cases Report and Literature Re-View

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5916-5916
Author(s):  
Weibin Zhuo ◽  
Ya Gao ◽  
Chunyan Yang ◽  
Ying Xu ◽  
Yintian Zhang ◽  
...  
Keyword(s):  
Survival Rate ◽  
Hemophagocytic Syndrome ◽  
Conflicts Of Interest ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Survival Group ◽  
Β2 Microglobulin ◽  
Short Period ◽  
Death Group

Abstract Objective HPS is caused by a variety of diseases, the most common causes including infections, malignancies and autoimmune diseases. It has an acute onset, a dangerous condition, rapid progress, and a high mortality rate. The main clinical manifestations are persistent fever, hepatosplenomegaly, hematocytopenia and tissue cell phagocytosis.Most of the current research is focused on children, and there are few reports on adult HPS.And lack of multi-center, prospective research, its pathogenesis, diagnosis, treatment is still poorly understood. Methods To analyze the clinical manifestations,laboratory data, therapy and prognosis in patients with hemophagocytic syndrome, 46 patients (over 18 years old) with HPS in our hospital from June 2011 to September 2017 were analyzed.The scope of the study included the patient's age, gender, etiology, clinical manifestations, laboratory indicators, treatment and prognosis, and analyzed its clinical, laboratory and prognostic characteristics. Result Of the 46 patient cases, 19 cases were secondary to cancer and 11 cases secondary to infection,10 cases were considered as malignant lymphoma by PET- CT,other 6 cases of unknown etiology.A total of 31 patients with EBV infection were diagnosed by EBV-DNA in all patients.Forty-six patients had various clinical manifestations, all had fever, mostly persistent irregular hyperthermia, and antibiotic treatment was not effective.There are also splenomegaly, serous effusion, respiratory symptoms, hemorrhage, central nervous system symptoms, renal dysfunction。According to HPS -2004 criteria,the coincidence rate of indices were: fever (100%), high level of serun ferritin (100% ),cytopenias (93.48% ),splenomegaly (91.30% ), hemophagocytosis in bone marrow,spleen or lymph nodes (84.78% ),hypofibrinogenemia (67.39% ), hypertriglyceridemia (54.05% ). Among cancer,infection and unknown etiology group,there were statistically differences on Ferritin and β2 microglobulin (P<0.05), It can be seen that the levels of Ferritin and β2 microglobulin in the tumor-associated HPS group were significantly higher than those in the unknown etiology and infection-related HPS group. The overall survival rate of the 46 patients was 11.1%.Causes of death included respiratory and circulatory failure (36.4%), cardiac arrest (18.2%), multiple organ failure (18.2%), hemorrhage, cerebral palsy (18.2%), and liver failure (9.1%).Comparing the laboratory indicators of the survival group and the death group at the initial diagnosis, the Lactic dehydrogenase and Triglyceride levels in the death group were higher than those in the survival group, while the Fibrinogen level in the death group was lower than that in the survival group. Conclusion The secondary HPS occurs from various underlined disease,many associated with Epstein- Barr virus infection.Cancer,especially NK/T- cell lymphoma is the main cause.Adult HPS progresses rapidly due to the disease, which makes it difficult to confirm the diagnosis in time. Some patients have deteriorated before the diagnosis is perfected.Increased sensitivity indicators such as sCD25 and NK cell activity can help early diagnosis. The 1-month survival rate of the chemotherapy group was higher than that of the anti-infective group alone, indicating that chemotherapy and controlled hemorrhage are meaningful for controlling the disease in a short period of time, while the simple anti-infection effect is poor.Adult HPS has only gradually gained attention in recent years. How to identify the underlying cause of HPS and to formulate a treatment strategy based on different incentives and prognostic factors is essential. Disclosures No relevant conflicts of interest to declare.

Clinical Characterization of Staphylococcus Septicemia-Associated Hemophagocytic Lymphohistiocytosis

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4905-4905
Author(s):  
Hongxia Qiu ◽  
Meng Song ◽  
Fang Ni ◽  
Jujuan Wang
Keyword(s):  
Bone Marrow ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Therapeutic Response ◽  
Gamma Globulin ◽  
Conflicts Of Interest ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Hepatic Function ◽  
Risk Of Death ◽  
Medical University

Abstract Abstract Objective: To analyze the epidemic characters, pathogenesis, clinical manifestations, laboratory data, therapeutic response, and prognosis in patients with staphylococcus septicemia-associated hemophagocytic lymphohistiocytosis(HLH). Methods A r Objective To analyze the epidemic characters, pathogenesis, clinical manifestations, laboratory data, therapeutic response, and prognosis in patients with staphylococcus septicemia-associated hemophagocytic lymphohistiocytosis(HLH). Methods: retrospective study was performed on 7 patients with staphylococcus septicemia-associated HLH who were admitted to The First Affiliated Hospital of Nanjing Medical University from January 2010 to December 2014. Results Seven out of 633patients(1.11%) with staphylococcus septicemia were confirmed to have HLH. The incidence of HLH in staphylococcus subspecies were:S.caprae, 12.50%;S.warneri, 12.50%;Methicillin-resistant Staphylococcusaureus(MRSA), 3.23%;S.hominis, 1.40%;S.haemolyticus, 1.25%;S.epidermidis, 0.59%, respectively. The main clinical manifestations included persistentfever(100%) and splenomegaly(85.71%). The most prominent laboratory abnormalities werehemocytopenia, abnormal hepatic function, and elevated ferritin. Bone marrow aspirations were performed on 6 patients and all of them hadhemophagocytosis shown in bone marrow smears. Among 7 patients, 4 patientssurvived(57.14%) and 3 patients died(42.86%). All the 4 cured patients were treated with antibiotic plus corticosteroid. And 2 of them used gamma globulin, while one used the COP chemotherapy regimen. Treatment with antibiotic plus corticosteroid was effective in our study. Conclusion Treatment with antibiotic plus corticosteroid was recommended for staphylococcus septicemia-associated HLH, and gamma globulin may also be useful in reducing the risk of death. Disclosures No relevant conflicts of interest to declare.

Splenectomy Exacerbates the Hypercoagulable State and Causes Significant Organ Dysfunction in Patients with Hemoglobin E/β-Thalassemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2014-2014
Author(s):  
Vichai Atichartakarn ◽  
Suporn Chuncharunee ◽  
Napaporn Archararit ◽  
Umaporn Udomsubpayakul ◽  
Atiporn Ingsathit ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Hypercoagulable State ◽  
P Value ◽  
Low Grade ◽  
Hemoglobin E ◽  
Inflammatory State ◽  
Pulmonary Arterial ◽  
Low Grade Inflammation

Abstract Abstract 2014 Poster Board I-1036 Introduction: Clinical manifestations of blood hypercoagulability, such as pulmonary arterial hypertension (PAH) and ischemic stroke, are well known in patients with thalassemia. We herein present evidence that the hypercoagulable state in β-Thalassemia disease patients is to a significant extent the result of prior splenectomy. Patients and Methods: One hundred and ten clinically stable hemoglobin E/β-Thalassemia outpatients, on no medication aside from folic acid and who received no blood transfusion in the preceding 4 weeks were studied. All gave written informed consent, and study protocol was approved by the institution ethics committee on studies in humans (#0774/2548). Echocardiogram was used to estimate systolic PA pressure (SPAP). Clinical features and laboratory data were stratified according to the presence or absence of the spleen, and statistical analysis was done by STATA version 10 (Stata Corp, Texas), considering a P value <0.05 as statistically significant. Results: Of the 110 patients, 61 were asplenic and females comprised 51% in both groups. Selected statistically significant results are shown in the table. Conclusions: In comparison with non-splenectomized hemoglobin E/β-Thalassemia patients, asplenic ones have higher SPAP, more severe hemolysis, more liver impairment and chronic iron overload, significant chronic low grade inflammation, and evidence of coagulation activation. Splenectomy should be more judiciously applied than heretofore, and if performed, measures to blunt or prevent a subsequent hypercoagulable and inflammatory state, which could lead to PAH should be initiated. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Study on Risk Factors for Death from Cardiomyopathy and Effectiveness of Health Information Management

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Lei Wang ◽  
Shuping Zhang ◽  
Yan Wang ◽  
Jin Xuan ◽  
Yanli Han ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Survival Rate ◽  
Information Management ◽  
Independent Risk Factor ◽  
Conventional Group ◽  
Survival Group ◽  
Risk Of Death ◽  
Significant Difference ◽  
Death Group

Objective. To explore risk factors for death from cardiomyopathy and the effectiveness of health information management (HIM). Methods. A total of 80 patients with cardiomyopathy admitted in ICU of our hospital (January 2016–January 2020) were selected as study subjects, and the clinical data of the patients were retrospectively analyzed. The patients were divided into the survival group (n = 72) and the death group (n = 14) according to the treatment outcome. Then, according to the management mode, the survival group was further equally divided into the conventional group and the HIM group to investigate the influence of risk factors on prognosis of patients with cardiomyopathy and the effectiveness of HIM. Results. No significant difference was found in baseline body mass, myocardial enzymes, troponin, infection factors, history of heart disease, and gender between the survival group and the death group ( P  > 0.05). Compared with the survival group, the patients of the death group were older ( P  < 0.05), LVEF of the death group was obviously lower ( P  < 0.05), and the scores of APACHE II and SOFA of the death group were obviously higher ( P  < 0.05). Further logistic regression analysis of the univariate factors influencing the risk of death from cardiomyopathy led to the conclusion that LVEF was an independent risk factor for death in patients with cardiomyopathy. LVEF below 24.69% examined by echocardiography had a high predictive value, with a sensitivity of 98.6% and a specificity of 78.6%. No obvious difference was found in general data between the conventional group and the HIM group ( P  > 0.05). Compared with the conventional group, the disease remission rate, complication rate, awareness rate of health knowledge, ICU length of stay, and scores of self-management efficacy of the HIM group were obviously better ( P  < 0.05). No significant difference was found in 5-year mean survival rate between the conventional group and the HIM group ( P  > 0.05). Conclusion. Older age, lower LVEF, and higher scores of APACHE II and SOFA are all risk factors for death from cardiomyopathy. Lower LVEF is an independent risk factor, and LVEF below 24.69% is an important indicator of increased risk of death. Moreover, HIM can effectively improve short-term treatment efficacy but has little effect on the long-term survival rate.

Investigating and analyzing three cohorts of mushroom poisoning caused by Amanita exitialis in Yunnan, China

2017 ◽  
Vol 37 (7) ◽  
pp. 665-678 ◽  
Author(s):  
J Sun ◽  
H-J Li ◽  
H-S Zhang ◽  
Y-Z Zhang ◽  
J-W Xie ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Latency Period ◽  
Ultra Performance Liquid Chromatography ◽  
Average Concentration ◽  
Mushroom Poisoning ◽  
Survival Group ◽  
The Public ◽  
Laboratory Examinations ◽  
Peptide Toxins ◽  
Death Group

Amanita exitialis is a lethal mushroom found in China. Knowledge regarding taxonomic characterization, toxin detection, general poisoning conditions, clinical manifestations, laboratory examinations, and clinical treatments for this species is currently lacking. We investigated three A. exitialis mushroom poisoning cohorts in Yunnan Province in 2014 and 2015, involving 10 patients. Mushroom samples were identified by morphological and molecular studies. Ultra performance liquid chromatography-electrospray ionization tandem mass spectrometry was used to detect the peptide toxins in the mushroom samples. Epidemiological information, clinical data, and results of laboratory examinations were collected and analyzed. The mushroom samples were all identified as A. exitialis. The average toxin concentration decreased from the cap to the stipe to the volva, and the average concentration of the peptide toxins decreased in the order of α-amanitin > phallacidin > β-amanitin > γ-amanitin. The latency period between ingestion and the onset of symptoms was 13.9 ± 2.1 h, and the time from ingestion to hospitalization was 49.6 ± 8.5 h. The most common symptoms were nausea and vomiting (100%). Four patients died from fulminant hepatic failure. Laboratory examinations showed that the alanine transaminase, aspartate transaminase, prothrombin time, and activated partial thromboplastin time levels peaked on the third day post-ingestion. Total bilirubin and direct bilirubin values peaked on day 7. The death group and the survival group had a similar variation trend of serological indexes, but the death group had a greater change. A. exitialis is an extremely dangerous mushroom and there is a need to educate the public to avoid picking and eating wild mushrooms that have not been definitively identified.

scholarly journals Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Aysin Kokturk
Keyword(s):  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Careful Evaluation ◽  
Behcet's Disease ◽  
Systemic Manifestations

Behçet's disease is a multisystemic inflammatory disease of unknown etiology which usually occurs as a trait of symptoms: aphthous stomatitis, genital ulcerations, and ocular disease. At the beginning of the disease the diagnosis is uncertain because of various clinical manifestations and a long period up to the full clinical picture manifestation. Since neither the laboratory data nor the histopathological signs are truly pathognomonic in Behçet's disease, the differential diagnosis depends on a careful evaluation of the medical history and meticulous physical examination to detect concomitant systemic manifestations. Sometimes, some laboratory test may help establish the diagnosis. Subspecialty referral to ophthalmology, rheumatology, neurology, and gastroenterology should be considered when indicated.

COVID 19: REALS AND CHALLENGES AGAINST DOCTORS

2020 ◽  
Vol 2 (1) ◽  
pp. 65-87
Author(s):  
Zen Ahmad
Keyword(s):  
Virus Disease ◽  
Clinical Manifestations ◽  
World Health ◽  
Unknown Etiology ◽  
Wuhan City ◽  
Traditional Markets ◽  
Pneumonia Case ◽  
The World ◽  
New Type ◽  
Health Organization

Corona Virus Disease (Covid-19) is a contagious disease caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) which was discovered in December 2019 in China. This disease can cause clinical manifestations in the airway, lung and systemic. The World Health Organization (WHO) representative of China reported a pneumonia case with unknown etiology in Wuhan City, Hubei Province, China on December 31, 2019. The cause was identified as a new type of coronavirus on January 7, 2020 with an estimated source of the virus from traditional markets (seafood market). ) Wuhan city

Cerebral Amyloid Angiopathy in Combination with Paroxysmal Atrial Fibrillation

2020 ◽  
Vol 25 (4) ◽  
pp. 31-37
Author(s):  
A. A. Kornilova ◽  
O. V. Lagoda ◽  
M. M. Tanashyan
Keyword(s):  
Atrial Fibrillation ◽  
Cerebral Amyloid Angiopathy ◽  
Paroxysmal Atrial Fibrillation ◽  
Past History ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Cerebral Haemorrhage ◽  
Exclusion Criterion ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid

The present article addresses the definition of cerebral amyloid angiopathy (CAA) and its symptoms based on the analysis of the medical case; the issues of diagnosis and treatment of this pathology are discussed. The Boston criteria, which became the basis for diagnosis, study of clinical manifestations and progression of CAA and approaches to its therapy, are presented. Methods and modes of neuroimaging, including magnetic resonance imaging (MRI), which verify micro cerebral haemorrhage, are described. At the same time, the role and significance of cardiac arrhythmias in the genesis of ischemic stroke are discussed, and scales for assessing the risk of its occurrence are presented. The observation of the neurological, somatic, neuroimaging, neuropsychological status of a 62-year-old patient confirms quite rare combination of probable CAA, paroxysmal atrial fibrillation and repeated hemorrhagic functional apoplexy (FA). The relevance of the case described, is a complex clinical dilemma based on mutually exclusive recommendations for the pharmacological correction of such conditions. It is emphasized that in many multicenter clinical studies on the effectiveness of antithrombotic medication (antiaggregants, anticoagulants) in the treatment and prevention of ischaemic functional apoplexy , an important exclusion criterion is a hemorrhagic stroke in past history (including the multiple changes in haemostasis indicators). Taking into account the obtained clinical and laboratory data in the dynamics, the tactics of treating the described patient were determined. The results of studies related to the treatment of comorbid pathology that should become the subject of the development of a personalized algorithm for managing patients in each specific case, are discussed.

Lymphomatoid Granulomatosis in a Child with Acute Lymphatic Leukemia in Remission

1978 ◽  
Vol 87 (5_suppl) ◽  
pp. 5-10 ◽  
Author(s):  
Seymour R. Cohen ◽  
Stuart Siegel ◽  
Eva Heuser ◽  
Benjamin H. Landing ◽  
Susan Shen ◽  
...  
Keyword(s):  
Survival Rate ◽  
Poor Prognosis ◽  
Lymphoblastic Leukemia ◽  
Lymphomatoid Granulomatosis ◽  
Unknown Etiology ◽  
Acute Lymphatic Leukemia ◽  
Clinical Onset ◽  
Lymphatic Leukemia ◽  
Trachea And Bronchi ◽  
Other Neoplasms

Lymphomatoid granulomatosis, a tumor-like process of unknown etiology, produced progressively destructive disease of the larynx, trachea and bronchi in an eight-year-old girl with acute lymphoblastic leukemia of five years duration. The leukemia had been in remission for 4½ years at the clinical onset of the lymphomatoid granulomatosis. Whether this occurrence suggests that lymphomatoid granulomatosis is a type of neoplasm, or is associated with immunologic depression, cannot be stated. Fortunately rare, and of poor prognosis, the disorder may become more frequent with improved survival rate of patients with leukemia and other neoplasms.

scholarly journals Animal Models of Autosomal Recessive Parkinsonism

Biomedicines ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 812
Author(s):  
Guendalina Bastioli ◽  
Maria Regoni ◽  
Federico Cazzaniga ◽  
Chiara Maria Giulia De Luca ◽  
Edoardo Bistaffa ◽  
...  
Keyword(s):  
Animal Models ◽  
Sleep Disturbances ◽  
Autosomal Recessive ◽  
Clinical Manifestations ◽  
Cognitive Disorders ◽  
Dopamine Neurons ◽  
Substantia Nigra Pars Compacta ◽  
Current Status ◽  
Unknown Etiology ◽  
Neuron Death

Parkinson’s disease (PD) is the most common neurodegenerative movement disorder. The neuropathological hallmark of the disease is the loss of dopamine neurons of the substantia nigra pars compacta. The clinical manifestations of PD are bradykinesia, rigidity, resting tremors and postural instability. PD patients often display non-motor symptoms such as depression, anxiety, weakness, sleep disturbances and cognitive disorders. Although, in 90% of cases, PD has a sporadic onset of unknown etiology, highly penetrant rare genetic mutations in many genes have been linked with typical familial PD. Understanding the mechanisms behind the DA neuron death in these Mendelian forms may help to illuminate the pathogenesis of DA neuron degeneration in the more common forms of PD. A key step in the identification of the molecular pathways underlying DA neuron death, and in the development of therapeutic strategies, is the creation and characterization of animal models that faithfully recapitulate the human disease. In this review, we outline the current status of PD modeling using mouse, rat and non-mammalian models, focusing on animal models for autosomal recessive PD.

Sign in / Sign up

Export Citation Format

Share Document