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Hemophagocytic lymphohistiocytosis

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https://www.readbyqxmd.com/read/29344491/hemophagocytic-lymphohistiocytosis-in-adults-with-intraocular-involvement-clinicopathologic-features-of-3-cases
#1
M Adelita Vizcaino, Charles G Eberhart, Fausto J Rodriguez
Background/Aims: Hemophagocytic lymphohistiocytosis (HLH) is an infrequent inflammatory multisystemic syndrome. Only rare cases with ophthalmic involvement describing their pathologic features have been previously reported. Methods: We report 3 cases of adult-onset HLH with bilateral ocular involvement and describe their clinicopathologic features. Results: Three adult males - 2 with a history of viral infection - developed persistent fever, fatigue, bone marrow abnormalities, and irreversible multiorgan failure...
December 2017: Ocular Oncology and Pathology
https://www.readbyqxmd.com/read/29342997/-x-linked-inhibitor-of-apoptosis-deficiency-manifested-as-crohn-s-disease-a-case-report-and-literature-review
#2
L J Xu, Y Y Luo, J D Yu, J G Lou, Y H Fang, J Chen
Objective: To analyze the clinical characteristics of X-linked inhibitor of apoptosis (XIAP) deficient patients with clinical manifestation of Crohn's disease. Methods: Clinical manifestations, laboratory investigations, genetic testing and therapeutic interventions of one case of XIAP deficiency who was admitted to Department of Gastroenterology in Children's Hospital, Zhejiang University School of Medicine in May 2016 were summarized. PubMed and Chinese database for articles published from January 2016 to June 2017 were searched using the key words of'Crohn's disease'and'XIAP', and the relevant literature was reviewed...
January 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29332707/cutaneous-hemophagocytosis-clinicopathologic-features-of-21-cases
#3
Francesca Boggio, Viviana Lora, Carlo Cota, Amanda Pereira, Robert Müllegger, Lucia Prieto-Torres, Lorenzo Cerroni
BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD...
February 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29326099/interleukin-18-diagnostically-distinguishes-and-pathogenically-promotes-human-and-murine-macrophage-activation-syndrome
#4
Eric S Weiss, Charlotte Girard-Guyonvarc'h, Dirk Holzinger, Adriana A de Jesus, Zeshan Tariq, Jennifer Picarsic, Eduardo J Schiffrin, Dirk Foell, Alexei A Grom, Sandra Ammann, Stephan Ehl, Tomoaki Hoshino, Raphaela Goldbach-Mansky, Cem Gabay, Scott W Canna
Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS) are life-threatening hyperferritinemic systemic inflammatory disorders. Though profound cytotoxic impairment causes familial HLH (fHLH), the mechanisms driving non-fHLH and MAS are largely unknown. MAS occurs in patients with suspected rheumatic disease, but the mechanistic basis for its distinction is unclear. Recently, a syndrome of recurrent MAS with infantile enterocolitis caused by NLRC4 inflammasome hyperactivity highlighted the potential importance of Interleukin (IL)-18...
January 11, 2018: Blood
https://www.readbyqxmd.com/read/29324572/epstein-barr-virus-ebv-induced-b-cell-lymphoproliferative-disorder-mimicking-the-recurrence-of-ebv-associated-hemophagocytic-lymphohistiocytosis
#5
Yuki Yatsushiro, Takuro Nishikawa, Aki Saito, Yozo Nakazawa, Ken-Ichi Imadome, Shunsuke Nakagawa, Yuichi Kodama, Yasuhiro Okamoto, Hirokazu Kanegane, Yoshifumi Kawano
We describe the case of a 23-month-old male infant with Epstein-Barr virus (EBV)-associated lymphoproliferative disorder, which mimicked the recurrence of EBV-associated hemophagocytic lymphohistiocytosis. Chemotherapy with dexamethasone, etoposide, and cyclosporine resolved fever, hepatosplenomegaly, and pancytopenia. However, on day 81 of illness, the patient developed similar symptoms. Plasma EBV-DNA levels markedly increased again, but no T-cell clonality was observed. B cells were identified to be infected with EBV...
January 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29319247/infection-associated-secondary-hemophagocytic-lymphohistiocytosis-in-sepsis-syndromes-a-tip-of-an-iceberg
#6
Arun Agarwal, Aakanksha Agarwal
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, underdiagnosed, fatal and devastating hyperinflammatory syndrome that has gained increasing recognition over the past decade. Patients with HLH present with clinical and laboratory evidence of uncontrolled inflammation. Delay in diagnosis and management inevitably leads to a rapidly progressive and fatal course. In this case series, we present 7 cases of secondary HLH (sHLH) in adults with their presentation, course, and outcomes...
October 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29312707/rare-causes-of-hyperbilirubinemia-after-lung-transplantation-our-experience-at-a-single-center
#7
Su Hwan Lee, Moo Suk Park, Jin Gu Lee, Joo Han Song, Kyung Soo Chung, Ji Ye Jung, Eun Young Kim, Young Sam Kim, Se Kyu Kim, Joon Chang, Hyo Chae Paik, Song Yee Kim
Background: Lung transplantation is the last treatment option for end-stage lung disease, and the number of lung transplantations has been steadily increasing. Hyperbilirubinemia is a rare complication after lung transplantation. The aim of this study was to review rare causes of hyperbilirubinemia after lung transplantation at our center. Methods: In this single-center study, we retrospectively reviewed the records of 116 consecutive lung transplantation patients who underwent lung transplantation at Severance Hospital and Gangnam Severance Hospital of Yonsei University College of Medicine in South Korea between December 22, 2010 and January 1, 2016...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29307884/streptococcus-pneumoniae-related-hemophagocytic-lymphohistiocytosis-treated-with-ivig-and-steroids
#8
Carissa Y Dumancas, Hans Alexi Garay Reyes, Juan Cosico, Amrut Savadkar, Soowhan Lah
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition that has a poor prognosis due to the ensuing cytokine storm leading to severe organ damage. Current treatment guidelines suggest using a combination of steroid- and etoposide-based chemotherapy. CASE REPORT The authors present a case of a 41-year-old African-American female who presented with symptoms of foodborne illness and who developed multi-organ dysfunction. HLH was suspected because of poor response to broad-spectrum antibiotics with a constellation of findings, including cytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia...
January 8, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29296930/gene-transfer-into-hematopoietic-stem-cells-reduces-hlh-manifestations-in-a-murine-model-of-munc13-4-deficiency
#9
Tayebeh Soheili, Amandine Durand, Fernando E Sepulveda, Julie Rivière, Chantal Lagresle-Peyrou, Hanem Sadek, Geneviève de Saint Basile, Samia Martin, Fulvio Mavilio, Marina Cavazzana, Isabelle André-Schmutz
Patients with mutations in the UNC13D gene (coding for Munc13-4 protein) suffer from familial hemophagocytic lymphohistiocytosis type 3 (FHL3), a life-threatening immune and hyperinflammatory disorder. The only curative treatment is allogeneic hematopoietic stem cell (HSC) transplantation, although the posttreatment survival rate is not satisfactory. Here, we demonstrate the curative potential of UNC13D gene correction of HSCs in a murine model of FHL3. We generated a self-inactivating lentiviral vector, used it to complement HSCs from Unc13d-deficient (Jinx) mice, and transplanted the cells back into the irradiated Jinx recipients...
December 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296904/soluble-interleukin-2-receptor-is-a-sensitive-diagnostic-test-in-adult-hlh
#10
Anna Hayden, Molly Lin, Sujin Park, Morris Pudek, Marion Schneider, Michael B Jordan, Andre Mattman, Luke Y C Chen
Serum soluble interleukin-2 receptor (sIL-2r) is an important disease marker in hemophagocytic lymphohistiocytosis (HLH), but there are no published data on its diagnostic value in adults. We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for suspected HLH. Serum sIL-2r levels were measured by enzyme-linked immunosorbent assay (adult reference range, 241-846 U/mL). There were 38 patients with HLH and 40 with a non-HLH diagnosis (such as sepsis, liver disease, histiocyte disorders, autoimmune disease, leukemia, or lymphoma)...
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296794/ruxolitinib-for-treatment-of-refractory-hemophagocytic-lymphohistiocytosis
#11
Larisa Broglie, Lauren Pommert, Sridhar Rao, Monica Thakar, Rachel Phelan, David Margolis, Julie Talano
Optimal salvage therapy for refractory HLH is unknown.In our patient, ruxolitinib treatment led to clinical remission of refractory HLH.
August 22, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296722/proliferation-through-activation-hemophagocytic-lymphohistiocytosis-in-hematologic-malignancy
#12
REVIEW
Eric J Vick, Kruti Patel, Philippe Prouet, Mike G Martin
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancy-induced SHL, which have significantly worse outcomes...
May 9, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295642/clinical-characteristics-therapy-response-and-outcome-of-51-adult-patients-with-hematological-malignancy-associated-hemophagocytic-lymphohistiocytosis-a-single-institution-experience
#13
Fryderyk Lorenz, Monika Klimkowska, Ewa Pawłowicz, Agnes Bulanda Brustad, Martin Erlanson, Maciej Machaczka
Hemophagocytic lymphohistiocytosis (HLH) is an underdiagnosed but life-threatening syndrome of hyperinflammation often occurring in adults with hematological malignancies (hM-HLH). The aim of the study was to describe clinical characteristics, therapy response, and outcome of adults with hM-HLH. The study included 51 adults with hM-HLH aged 23-84 years. Hyperferritinemia ≥500 µg/L was present in 96% of patients. The serum concentration of sIL-2Rα ≥ 2400 U/mL was revealed in 94% of patients. Twenty-three patients (45%) responded to therapy and achieved remission of HLH...
January 3, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29280824/a-case-of-relapsing-polychondritis-mimicking-hemophagocytic-lymphohistiocytosis-after-propionibacterium-acnes-infection
#14
William E Monaco, Carey J Field, Thomas H Taylor
We describe a 69-year-old man who presented with a right sternocleidomastoid intramuscular abscess that grew Propionibacterium acnes. Despite initial improvement with antibiotics, he subsequently developed signs and symptoms fulfilling diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH)-fevers, cytopenias, hyperferritinemia, splenomegaly, and low natural killer cell activity. The patient improved spontaneously but at outpatient follow-up complained of erythema over the bridge of his nose as well as bilateral ear redness sparing the earlobes at which point relapsing polychondritis (RP) was diagnosed...
December 27, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29279700/minimal-change-disease-as-initial-presentation-of-alk-positive-anaplastic-large-cell-lymphoma-in-a-pediatric-patient
#15
Karla Diaz, William Slayton, Nirupama Gupta
The association between nephrotic syndrome (NS), hemophagocytic lymphohistiocytosis (HLH), and certain paraneoplastic syndromes has been documented in the literature. However, nephrotic changes as part of paraneoplastic syndromes are rare in lymphoid malignancies, particularly in non-Hodgkin lymphoma. We report the sudden onset of acute renal failure and NS in a 14-year-old male who initially presented with HLH and was subsequently diagnosed with ALK-positive anaplastic large-cell lymphoma (ALCL). The treatment of ALCL reversed both the HLH and NS findings...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29258535/lytic-viral-replication-and-immunopathology-in-a-cytomegalovirus-induced-mouse-model-of-secondary-hemophagocytic-lymphohistiocytosis
#16
Ellen Brisse, Maya Imbrechts, Tania Mitera, Jessica Vandenhaute, Carine H Wouters, Robert Snoeck, Graciela Andrei, Patrick Matthys
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare immunological disorder caused by unbridled activation of T cells and macrophages, culminating in a life-threatening cytokine storm. A genetic and acquired subtype are distinguished, termed primary and secondary HLH, respectively. Clinical manifestations of both forms are frequently preceded by a viral infection, predominantly with herpesviruses. The exact role of the viral infection in the development of the hemophagocytic syndrome remains to be further elucidated...
December 19, 2017: Virology Journal
https://www.readbyqxmd.com/read/29241728/an-actin-cytoskeletal-barrier-inhibits-lytic-granule-release-from-natural-killer-cells-in-chediak-higashi-syndrome
#17
Aleksandra Gil-Krzewska, Mezida B Saeed, Anna Oszmiana, Elizabeth R Fischer, Kathryn Lagrue, William A Gahl, Wendy J Introne, John E Coligan, Daniel M Davis, Konrad Krzewski
BACKGROUND: Chediak-Higashi syndrome (CHS) is a rare disorder caused by biallelic mutations in the LYST gene, resulting in formation of giant lysosomes or lysosome-related organelles in several cell types. The disease is characterized by immunodeficiency and a fatal hemophagocytic lymphohistiocytosis due to impaired function of cytotoxic lymphocytes, mainly Natural Killer (NK) cells. OBJECTIVE: We sought to determine the underlying biochemical cause of the impaired cytotoxicity of NK cells in CHS...
December 11, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29239849/hepatitis-during-pregnancy-a-case-of-hemophagocytic-lymphohistiocytosis
#18
Halil Yildiz, Bernard Vandercam, Xave Thissen, Mina Komuta, Nicolas Lanthier, Frederic Debieve, Geraldine Dahlqvist
Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal syndrome that can occur during pregnancy. A 36 years-old woman, at 29 weeks of gestation, presented with itchiness and jaundice since a week. On clinical examination she was apyrexial and frankly icteric. Laboratory data showed evidence of acute hepatitis. A complete work-up was made excluding viral hepatitis (HAV, HEV, HBV, HCV, HHV6, CMV, EBV) and autoimmune liver disease. Liver diseases related to pregnancy were not completely excluded...
November 24, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29239076/allogeneic-hematopoietic-stem-cell-transplantation-is-associated-with-cure-and-durable-remission-of-late-onset-primary-isolated-central-nervous-system-hemophagocytic-lymphohistiocytosis
#19
Sajad Khazal, Veronika Polishchuk, Gary Soffer, Samantha Prinzing, Jonathan Gill, Kris M Mahadeo
Primary isolated CNS presentation of HLH is exceedingly rare and typically associated with significant morbidity and mortality. We describe an adolescent patient with late-onset, primary isolated CNS HLH and a compound heterozygous PRF1 mutation (c50delT (p.L17 fs); c.1229G>C (p.R410P)), not previously reported with this phenotype. He was successfully treated with allogeneic HSCT following a reduced-intensity conditioning regimen, despite a high pre-HSCT comorbidity index. Two years after transplant, he is alive and in disease remission...
December 13, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29226650/multiple-nonleukemic-myeloid-sarcoma-associated-hemophagocytic-lymphohistiocytosis-in-an-adult
#20
Jingwen Zhang, Yunxin Zeng, Xiangzhong Zhang, Wenxing Lai, Yuxin Chen, Ruozhi Xiao
BACKGROUND: Nonleukemic myeloid sarcoma (MS) occurs rarely. Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal condition. We report a rare case of nonleukemic MS associated with HLH. METHODS: Hematologic investigation, 18F-FDG PET/CT, bone marrow aspirate and biopsy, and lymph node biopsy were performed in a 25-year-old male patient. RESULTS: The patient was given a short-term treatment of etoposide and dexamethasone to control HLH...
November 1, 2017: Clinical Laboratory
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