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https://www.readbyqxmd.com/read/29344060/neuro-ophthalmic-presentation-of-neuro-sweet-disease
#1
Padmaja Sudhakar, Stuart Tobin, William O Connor, Sachin Kedar
Acute febrile neutrophilic dermatosis (Sweet syndrome) is a systemic inflammatory condition usually associated with autoimmune or neoplastic processes and characterised by inflammatory dermatologic lesions such as erythematous plaques and papules associated with fever and leukocytosis. Neurological and ophthalmological involvement is rare. The authors describe an unusual case of Sweet syndrome associated with microscopic polyangiitis presenting with papilloedema, anterior uveitis, and skin rash. Years later, he developed acute posterior multifocal placoid pigment epitheliopathy...
August 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/29234548/steroid-responsive-encephalopathy-associated-with-autoimmune-thyroiditis-presenting-with-fever-and-confusion
#2
Chiranthi Kongala Liyanage, Tilak Manthi Janake Munasinghe, Adsareswary Paramanantham
Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) is a diagnostic conundrum as it may present with a myriad of nonspecific clinical features and laboratory and neuroimaging investigations are not diagnostic. We report a case of a 65-year-old female who presented with an acute febrile illness associated with headache and confusion, tangential thoughts, and loose association. Based on neutrophil leukocytosis in the full blood count and elevated inflammatory markers, she was commenced on empirical intravenous antibiotics suspecting meningoencephalitis...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/29215577/acute-encephalitis-in-an-adult-with-diffuse-large-b-cell-lymphoma-with-secondary-involvement-of-the-central-nervous-system-infectious-or-non-infectious-etiology
#3
Surinder S Moonga, Kenneth Liang, Burke A Cunha
Both infectious and non-infectious etiologies of acute encephalitis have been described, as well as their specific presentations, diagnostic tests, and therapies. Classic findings of acute encephalitis include altered mental status, fever, and new lesions on neuroimaging or electroencephalogram (EEG). We report an interesting case of a 61-year-old male with a history of diffuse large B-cell lymphoma with secondary involvement of the central nervous system (SCNS-DLBCL). He presented with acute encephalitis: altered mental status, fever, leukocytosis, neuropsychiatric symptoms, multiple unchanged brain lesions on computed tomography scan of the head, and EEG showed mild to moderate diffuse slowing with low-moderate polymorphic delta and theta activity...
December 7, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28966522/histiocytoid-sweet-syndrome-in-a-child-without-underlying-systemic-disease
#4
Seung Dohn Yeom, Hye Soo Ko, Jong Hyuk Moon, Min Ji Kang, Ji Won Byun, Gwang Seong Choi, Jeonghyun Shin
Sweet syndrome (acute, febrile, neutrophilic dermatosis) is characterized by the acute onset of an eruption of painful nodules or erythematous or violaceous plaques on the limbs, face and neck. These symptoms are accompanied by fever. The diagnostic features include histopathological findings of dermal neutrophilic infiltration without leukocytoclastic vasculitis or peripheral blood leukocytosis. Sweet syndrome is associated with infection, malignancies, autoimmune disease, pregnancy, and drugs. Patients with Sweet syndrome demonstrate a complete and rapid response to systemic steroid administration...
October 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28825890/case-245-erdheim-chester-disease
#5
Mark D Mamlouk, Mariam S Aboian, Christine M Glastonbury
History A 53-year-old man experienced headache and double vision that progressed over 1 year. After a traumatic fall, he was hospitalized, and proptosis was identified at physical examination. Laboratory tests were remarkable for leukocytosis. Hematocrit level, thyroid stimulating hormone level, autoimmune antibody level, erythrocyte sedimentation rate, and C-reactive protein level were normal. Computed tomography (CT) of the head revealed bilateral intraconal masses, for which magnetic resonance (MR) imaging of the orbits was subsequently performed ( Fig 1 )...
September 2017: Radiology
https://www.readbyqxmd.com/read/28638005/sclerosing-mesenteritis-causing-chylous-ascites-and-small-bowel-perforation
#6
Huei-Wen Lim, Keith S Sultan
BACKGROUND Sclerosing mesenteritis (SM) is a rare idiopathic inflammation and fibrosis of the mesentery. Its etiology and pathogenesis remain unclear. It has been linked to abdominal trauma related to peritoneal dialysis, recent abdominal surgery, idiopathic inflammatory disorder, paraneoplastic syndrome, ischemia/infection, and autoimmunity. Abdominal CT is the most sensitive imaging modality, and diagnosis is usually confirmed by surgical biopsy. Patients most often present with abdominal pain, nausea, vomiting, diarrhea, and weight loss, and less commonly with chylous ascites and small bowel obstruction...
June 22, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28473932/acquired-thrombotic-thrombocytopenic-purpura-in-a-patient-with-pernicious-anemia
#7
Ramesh Kumar Pandey, Sumit Dahal, Kamal Fadlalla El Jack Fadlalla, Shambhu Bhagat, Bikash Bhattarai
Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests' results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28440198/the-role-of-neutrophil-proteases-in-lps-induced-production-of-regulatory-rheumatoid-factor-that-suppresses-autoimmunity
#8
Elena Stolyarova, Liubov Beduleva, Alexandr Sidorov, Igor Menshikov, Alexey Terentiev, Kseniya Vachrusheva
BACKGROUND: We recently identified a regulatory rheumatoid factor (regRF), the production of which is associated with autoimmune disease resistance and remission. In studies of regRF in the blood of healthy rats, spontaneous increases in the regRF level were noted. We suggest that in the normal state, a mechanism exists for maintaining the activity of the pool of regRF-producing lymphocytes at a level that makes it possible to control the expansion of autoreactive lymphocytes. OBJECTIVE: The purpose of this study was to test the hypothesis that the endogenous stimulator of regRF production is Fc fragments of IgG that are formed upon exposure to the proteases of neutrophils...
April 24, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28197781/sclerosing-mesenteritis-a-systematic-review-of-192-cases
#9
REVIEW
Prabin Sharma, Siddhartha Yadav, Christine Marie Needham, Paul Feuerstadt
INTRODUCTION: Sclerosing mesenteritis includes a spectrum of inflammatory disorders involving the adipose tissue of the bowel mesentery. AIM: To perform a systematic review of previously reported cases of sclerosing mesenteritis (SM) to determine the epidemiology, risk factors, methods of diagnosis, treatment patterns and outcomes for this disease. METHODS: Medline, PubMed, Google Scholar and Cochrane database were searched using keywords mesenteric panniculitis, retractile mesenteritis, mesenteric lipodystrophy and sclerosing mesenteritis...
April 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/28195263/kikuchi-fujimoto-disease-a-clinicopathologic-update
#10
F Pepe, S Disma, C Teodoro, P Pepe, G Magro
Kikuchi-Fujmoto disease (KFD), also known as "histiocytic necrotizing lymphadenitis", is a rare lymphadenitis of unknown origin, but with an excellent prognosis. It is more common in Asia, but isolated cases are also reported in America, Africa and Europe. The disease can have an acute or subacute course, usually develops in 1 to 3 weeks, with spontaneous resolution in 1-4 months. The main clinical sign is cervical lymphadenopathy, especially in the posterior cervical triangle with bulky and painful lymph nodes, usually affecting only one side; rare cases of generalized lymphadenopathy can be seen...
September 2016: Pathologica
https://www.readbyqxmd.com/read/27866578/leukocytosis-and-leukemia
#11
REVIEW
Page Widick, Eric S Winer
Leukocytosis is among the most common findings on peripheral blood smear. A wide range of causes may mediate this finding, and careful clinical and laboratory evaluation assist in differentiating between benign and malignant causes of increased white blood cell counts. In this article, various nonmalignant causes are explored, including infectious, inflammatory, autoimmune, and allergic. In addition, malignant causes of leukocytosis are discussed, including myeloproliferative disorders, acute leukemia, and chronic leukemia, as well as treatment and monitoring for patients with these diseases...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27581436/synergistic-neutralization-of-pertussis-toxin-by-a-bispecific-antibody-in-vitro-and-in-vivo
#12
Ellen K Wagner, Xianzhe Wang, Andre Bui, Jennifer A Maynard
Bispecific antibodies are a rapidly growing class of therapeutic molecules, originally developed for the treatment of cancer but recently explored for the treatment of autoimmune and infectious diseases. Bordetella pertussis is a reemerging pathogen, and several of the key symptoms of infection are caused by the pertussis toxin (PTx). Two humanized antibodies, hu1B7 and hu11E6, bind distinct epitopes on PTx and, when coadministered, mitigate disease severity in murine and baboon models of infection. Here we describe the generation of a bispecific human IgG1 molecule combining the hu1B7 and hu11E6 binding sites via a knobs-in-holes design...
November 2016: Clinical and Vaccine Immunology: CVI
https://www.readbyqxmd.com/read/27459964/goodpasture-s-syndrome-with-absence-of-circulating-anti-glomerular-basement-membrane-antibodies-a-case-report
#13
Rui Fernandes, Sara Freitas, Pedro Cunha, Gloria Alves, Jorge Cotter
BACKGROUND: Goodpasture's syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours...
July 27, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27352260/paraneoplastic-pemphigus-as-a-presentation-of-acute-myeloid-leukemia-early-diagnosis-and-remission
#14
Salahuddin Siddiqui, Mohammad Bilal, Zachary Otaibi, Farshaad Bilimoria, Nihar Patel, James Rossetti
Skin lesions are frequently encountered in clinical practice which can be a presentation of systemic diseases not excluding an occult malignancy. Commonly reported paraneoplastic dermatologic manifestations include acanthosis nigricans, dermatomyositis, erythroderma, hypertrophic osteoarthropathy, Sweet syndrome, and paraneoplastic pemphigus (PNP). PNP is a rare autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphic skin eruptions, and associated underlying neoplasms most commonly non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman disease...
September 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/27000538/bartonella-henselae-infection-presenting-with-a-picture-of-adult-onset-still-s-disease
#15
Areum Durey, Hea Yoon Kwon, Jae-Hyoung Im, Sun Myoung Lee, JiHyeon Baek, Seung Baik Han, Jae-Seung Kang, Jin-Soo Lee
We report a patient with a clinical picture of suggestive for adult-onset Still's Disease (ASOD) due to Bartonella infection. A 42-year-old immunocompetent man was admitted with fever, rash, arthralgia and sore throat. As his clinical picture suggested ASOD except unusual skin manifestation, we treated him on steroid and ibuprofen. His fever and constitutional symptoms responded immediately within 24hrs of commencing therapy, yet rash and leukocytosis remained. Meanwhile, Bartonella infection was proved by culture of bone marrow...
May 2016: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/26793930/hodgkin-s-lymphoma-presenting-as-a-cavitary-lung-lesion
#16
Joseph Garrity, Anna Batterson, Tamera Sturm
Hodgkin lymphoma (HL) typically presents in the nodal regions at an early stage of the disease. Occasionally, patients can present with disease in extranodal regions. We report a case of HL in a 25-year-old female who presented to the emergency department following a motor vehicle accident with incidental findings of pulmonary cavitary lesions, fever, tachycardia, anemia, leukocytosis and thrombocytosis. This case illustrates the difficulty in diagnosis when the disease presents in extranodal regions with nonspecific systemic symptoms...
December 2015: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/26356690/acute-splenic-infarction-at-an-academic-general-hospital-over-10-years-presentation-etiology-and-outcome
#17
Ami Schattner, Schattner Ami, Meital Adi, Adi Meital, Ella Kitroser, Kitroser Ella, Abraham Klepfish, Klepfish Abraham
Few case series provide a current, comprehensive, and detailed description of splenic infarction (SI), an uncommon condition.Retrospective chart review complemented by imaging evaluation and patient follow-up.All adult patients with a confirmed diagnosis of acute SI discharged over 10 years from a single academic center were studied. A systematic literature review was done to compile a complete list of SI etiologies.SI was found in 32 patients, 0.016% of admissions. Ages ranged from 18 to 86 (median 64) years...
September 2015: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26317390/neutrophilic-epitheliotropism-is-a-histopathological-clue-to-neutrophilic-urticarial-dermatosis
#18
Sigrid M C Broekaert, Almut Böer-Auer, Katrin Kerl, Ilka Herrgott, Xenia Schulz, Gisela Bonsmann, Randolf Brehler, Dieter Metze
BACKGROUND: Neutrophilic urticarial dermatosis (NUD) comprises a particular autoinflammatory condition within the spectrum of aseptic neutrophilic dermatoses characterized by a distinct urticarial eruption clinically and a neutrophilic dermatosis histopathologically. OBJECTIVE: In this study, we reviewed skin biopsies of lesional skin of patients seen in our outpatient clinic for autoimmune dermatoses and in allergy department from 1982 to 2014 that fulfilled these criteria...
January 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/26155624/-hemophagocytic-syndrome-in-a-patient-with-adult-onset-still-s-disease-diagnostic-problems
#19
A Yu Zakharova, Z Yu Mutovina, A V Gordeev, I N Shestakova
Hemophagocytic syndrome (HPS) is a rare life-threatening condition caused by massive cytokine release from activated macrophages and lymphocytes. The paper depicts the development of HPS in different infections, malignancies, and autoimmune diseases. It describes a clinical case of hemophagocytic syndrome in a 63-year-old female patient with adult-onset Still's disease and high fever accompanied by neutrophil leukocytosis and a drastic left leukocyte count shift, high procalcitonin levels, hepatosplenomegaly, edematous syndrome, and progressive multiple organ dysfunction with the development of disseminated intravascular coagulation and adult respiratory distress syndrome...
2015: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/25929927/the-pathologic-findings-of-skin-lymph-node-liver-and-bone-marrow-in-patients-with-adult-onset-still-disease-a-comprehensive-analysis-of-40-cases
#20
Hyoun-Ah Kim, Jee Eun Kwon, Hyunee Yim, Chang-Hee Suh, Ju-Yang Jung, Jae Ho Han
Adult-onset Still disease (AOSD) is characterized by fever, skin rash, and lymphadenopathy with leukocytosis and anemia as common laboratory findings. We investigated the characteristic pathologic findings of skin, lymph node, liver, and bone marrow to assist in proper diagnosis of AOSD.Forty AOSD patients were included in the study. The skin (26 patients), lymph node (8 patients), liver (8 patients), or bone marrow biopsies (22 patients) between 1998 and 2013 were retrospectively analyzed. AOSD patients were diagnosed according to the Yamaguchi criteria after excluding common infections, hematological and autoimmune diseases...
May 2015: Medicine (Baltimore)
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