scholarly journals Intracranial Haemorrhage as a Fatal Complication of Evans Syndrome: A Case Report

2019 ◽  
Vol 2 (2) ◽  
pp. 66-69
Author(s):  
Olita Shilpakar ◽  
Bibek Rajbhandari ◽  
Bipin Karki ◽  
Umesh Bogati

Evans syndrome is a rare hematologic disorder characterized by the presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA), immune-mediated thrombocytopenia and/or immune neutropenia without any known underlying etiology. Spontaneous intracranial hemorrhage is a rare and life-threatening complication in patients with Evans syndrome and very few cases have been reported to date. We report a case of a thirty-two- year-old female with intracranial haemorrhage with underlying Evans syndrome who presented with the clinical manifestations of headache, vomiting and altered sensorium and succumbed to the fatal complication despite resuscitative measures. This also emphasizes the importance of early recognition of symptoms and immediate presentation to health care facilities for aggressive management of the patient.

2011 ◽  
Vol 93 (4) ◽  
pp. e15-e16 ◽  
Author(s):  
Jagwinder Dhaliwal ◽  
Ajanta Jayatunga

Phlegmasia cerulea dolens (PCD) is a limb-threatening venous disorder involving massive proximal venous thrombosis. The clinical manifestations are of oedema, cyanosis and pain of lower extremity. Patients presenting with PCD have an underlying pathological condition that predisposes to the thrombotic process. We report a diabetic patient who presented with PCD and septicaemia due to a spontaneous psoas abscess. Anticoagulation with heparin and treatment of the underlying psoas abscess led to complete resolution of symptoms. An understanding of the underlying pathological process responsible is vital to early recognition and successful outcome in this rare limb- and life-threatening venous disorder.


2020 ◽  
Author(s):  
Atsushi Sakamoto ◽  
Masao Ogura ◽  
Atsushi Hattori ◽  
Kinji Tada ◽  
Reiko Horikawa ◽  
...  

Abstract Background: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is characterized by bleeding and thrombosis in patients, usually associated with autoimmunity or infections. Pediatric LAHPS exhibits various degrees of bleeding, ranging from mild to severe; however, adrenal hemorrhage due to LAHPS and its long-term clinical course have not been sufficiently described. Case presentation: A 9‐year‐old boy presented with prolonged abdominal pain and abnormal coagulation screening tests. The laboratory tests showed prolonged activated partial thromboplastin time and subsequently revealed the presence of lupus anticoagulant, anti-nuclear antibodies, and hypoprothrombinemia, leading to diagnosis of LAHPS. An enhanced computed tomogram demonstrated nodular lesions in the adrenal glands bilaterally, suggestive of adrenal hemorrhage. Laboratory and clinical manifestations exhibited life-threatening adrenal insufficiency that required hydrocortisone administration. The patient developed systemic lupus erythematosus, diagnosed 12 months later. Conclusions: The patient with LAHPS developed rare adrenal failure due to adrenal hemorrhage, a life-threatening event that should be recognized and treated early. In our case, renal dysfunction was also observed when systemic lupus erythematosus was diagnosed one year after LAHPS. Our case emphasizes that early recognition of adrenal failure and careful observation for patients with autoantibodies are required.


2020 ◽  
Vol 07 (04) ◽  
pp. 13-15 ◽  
Author(s):  
BS Mahapatra ◽  

Multisystem Inflammatory Syndrome in Children (MIS-C) is a newly recognized multiorgan disease seen in children, adolescent and young adults presumed to be a delayed immune mediated complication of Corona virus 2 (SARS-CoV-2) infection leading to severe acute respiratory syndrome. MIS-C can be associated with life threatening organ dysfunction requiring complex multidisciplinary care. Early recognition is important in order to prevent complication and serious sequalae. Because it is a post infective complication, in most of the cases RT-PCR comes negative though antibodies to COVID-19 are positive. Although SARS-CoV-2 in children are generally mild and nonfatal, there is increasing evidence of MIS-C. Clinical and laboratory features of MIS-C are similar to those of Kawasaki disease like syndrome and Toxic Shock Syndrome. Pathophysiology of MIS-C is still unclear and mainly due to formation of autoantibody and immune complex which activates inflammation. Most of the MIS-C associated with COVID-19, need treatment with ionotropic agents and anticoagulants. The long-term outcome of MIS-C like coronary artery aneurysm formation remain unknown and needs close follow up.


Blood ◽  
2009 ◽  
Vol 114 (15) ◽  
pp. 3167-3172 ◽  
Author(s):  
Marc Michel ◽  
Valérie Chanet ◽  
Agnès Dechartres ◽  
Anne-Sophie Morin ◽  
Jean-Charles Piette ◽  
...  

Abstract Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as “primary” in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one “second-line” treatment, including splenectomy(n = 19) and rituximab (n = 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders.


Author(s):  
Ivona Butorac Ahel ◽  
Srecko Severinski ◽  
Kristina Lah Tomulic ◽  
Ana Milardovic ◽  
Kristina Baraba Dekanic ◽  
...  

Abstract Objectives Hyperglycemic hyperosmolar state (HHS) is one of the most severe acute complications of diabetes mellitus (DM) characterized by severe hyperglycemia and hyperosmolality without significant ketosis and acidosis. What is new? Since HHS in the pediatric population is rare and potentially life-threatening, every reported case is very valuable for raising awareness among healthcare professionals. Case presentation A 7-year-old boy with previously diagnosed Joubert syndrome was admitted due to vomiting, polydipsia and polyuria started several days earlier. He was severely dehydrated, and the initial blood glucose level was 115 mmol/L. Based on clinical manifestations and laboratory results, he was diagnosed with T1DM and HHS. The treatment with intravenous fluid was started and insulin administration began later. He was discharged after 10 days without any complications related to HHS. Conclusions Since HHS has a high mortality rate, early recognition, and proper management are necessary for a better outcome.


2021 ◽  
Vol 34 (3) ◽  
Author(s):  
Nicola Clementi ◽  
Sreya Ghosh ◽  
Maria De Santis ◽  
Matteo Castelli ◽  
Elena Criscuolo ◽  
...  

SUMMARY Several viruses target the human respiratory tract, causing different clinical manifestations spanning from mild upper airway involvement to life-threatening acute respiratory distress syndrome (ARDS). As dramatically evident in the ongoing SARS-CoV-2 pandemic, the clinical picture is not always easily predictable due to the combined effect of direct viral and indirect patient-specific immune-mediated damage. In this review, we discuss the main RNA (orthomyxoviruses, paramyxoviruses, and coronaviruses) and DNA (adenoviruses, herpesviruses, and bocaviruses) viruses with respiratory tropism and their mechanisms of direct and indirect cell damage. We analyze the thin line existing between a protective immune response, capable of limiting viral replication, and an unbalanced, dysregulated immune activation often leading to the most severe complication. Our comprehension of the molecular mechanisms involved is increasing and this should pave the way for the development and clinical use of new tailored immune-based antiviral strategies.


2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Chuanchen Zhang ◽  
Xiaoyong Huang ◽  
Shuhua Li ◽  
Hengchen Yao ◽  
Bin Zhang

Pulmonary artery dissection is extremely rare but it is a really life-threatening condition when it happens. Most patients die suddenly from major bleeding or tamponade caused by direct rupture into mediastinum or retrograde into the pericardial sac. What we are reporting is a rare case of a 46-year-old female patient whose pulmonary artery dissection involves both the pulmonary valve and right pulmonary artery. The patient had acute chest pain and severe dyspnea, and the diagnosis of pulmonary artery dissection was confirmed by ultrasonography and CT angiography. Moreover, its etiology, clinical manifestations, and management are also discussed in this article.


2005 ◽  
Vol 39 (7-8) ◽  
pp. 1346-1348 ◽  
Author(s):  
Minh Huynh ◽  
Karen Chee ◽  
Derick HM Lau

OBJECTIVE To report a case of thrombotic thrombocytopenic purpura (TTP) caused by the antipsychotic quetiapine on 2 occasions in the same patient and review the hematologic adverse events associated with quetiapine. CASE SUMMARY A 25-year-old African American male with a history of bipolar disorder was treated with quetiapine and developed thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure consistent with a diagnosis of TTP on 2 occasions 2 years apart. On each occasion, TTP was successfully treated with plasmapheresis. DISCUSSION Many medications, including antibiotics, immunosuppressants, and antineoplastics, have been implicated as causative agents of TTP. Although, as of this writing, a review of the medical literature reveals no previous report of TTP associated with quetiapine, the Food and Drug Administration database of the Adverse Event Reporting System has compiled, as of this writing, 3 cases of TTP occurring in patients on quetiapine as their sole medication. In addition, this database has recorded other common hematologic adverse effects, including neutropenia and thrombocytopenia, that are possibly associated with quetiapine. In our patient, quetiapine-associated TTP presented within a few days after exposure to the drug, with early thrombocytopenia followed by delayed appearance (2–3 days) of microangiopathic hemolysis. CONCLUSIONS An objective causality assessment suggests that quetiapine was the highly probable cause of TTP in this patient. Early recognition, discontinuation of the drug, and institution of plasmapheresis are paramount for prompt resolution of this life-threatening hematologic disorder.


2021 ◽  
Vol 49 (5) ◽  
pp. 030006052110122
Author(s):  
Yimin Ma ◽  
Duanming Zhuang ◽  
Zhenguo Qiao

Celiac disease (CD) is a chronic immune-mediated intestinal disease that is characterized by production of autoantibodies directed against the small intestine. The main clinical manifestations of CD are typically defined as those related to indigestion and malabsorption. These manifestations include unexplained diarrhea or constipation, abdominal pain, bloating, weight loss, anemia, failure-to-thrive in children, and decreased bone density. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by heterogeneous clinical manifestations, which may also involve the gastrointestinal tract. Comorbidity of CD and SLE is rare, and the overlapping symptoms and nonspecific clinical presentation may pose a diagnostic challenge to clinicians. We report here a case of SLE with CD, which mainly manifested as recurrent diarrhea, uncorrectable electrolyte disorders, and severe malnutrition. Through review, we hope to further improve our understanding and diagnostic level of this combination of diseases.


2021 ◽  
Vol 8 (1) ◽  
pp. e000628
Author(s):  
Kurt Boeykens ◽  
Ivo Duysburgh

BackgroundPercutaneousendoscopic gastrostomy is a commonly used endoscopic technique where a tube isplaced through the abdominal wall mainly to administer fluids, drugs and/orenteral nutrition. Several placement techniques are described in the literaturewith the ‘pull’ technique (Ponsky-Gardener) as the most popular one.Independent of the method used, placement includes a ‘blind’ perforation of thestomach through a small acute surgical abdominal wound. It is a generally safetechnique with only few major complications. Nevertheless these complicationscan be sometimes life-threatening or generate serious morbidity.MethodAnarrative review of the literature of major complications in percutaneousendoscopic gastrostomy.ResultsThis review was written from a clinical viewpoint focussing on prevention andmanagement of major complications and documentedscientific evidence with real cases from more than 20 years of clinical practice.ConclusionsMajorcomplications are rare but prevention, early recognition and popper management areimportant.


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