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hemophagocytic syndrome

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https://www.readbyqxmd.com/read/28433836/recent-advances-in-diagnostic-and-therapeutic-guidelines-for-primary-and-secondary-hemophagocytic-lymphohistiocytosis
#1
REVIEW
Smita Ramachandran, Fauzia Zaidi, Archana Aggarwal, Rani Gera
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by excessive activation of macrophages and T cells resulting from defective cytotoxicity. It is potentially life threatening due to the large amounts of cytokines released by the activated macrophages and lymphocytes secondary to a hyperinflammatory response. It has a high fatality in children with an incidence of approximately 1.2cases/million per year. METHOD: The literature was extensively searched in PubMed, MEDLINE and Google scholar...
November 3, 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28409119/severe-dengue-due-to-secondary-hemophagocytic-lymphohistiocytosis-a-case-study
#2
Ujjwayini Ray, Soma Dutta, Susovan Mondal, Syamasis Bandyopadhyay
Dengue, transmitted by the mosquito Aedes aegypti affects millions of people worldwide every year. Dengue induced hemophagocytic lymphohistiocytosis (HLH) is a serious condition and may prove fatal if not detected early and treated appropriately. Diagnosis of HLH is challenging and usually missed as clinical and laboratory findings are nonspecific. Moreover, the pathophysiology of the systemic inflammatory response syndrome and/or sepsis is remarkably similar to HLH. Secondary HLH following infection by the dengue virus is now being increasingly recognized as a cause of severe form of the disease...
2017: IDCases
https://www.readbyqxmd.com/read/28409071/acute-cytomegalovirus-cmv-infection-associated-with-hemophagocytic-lymphohistiocytosis-hlh-in-an-immunocompetent-host-meeting-all-eight-hlh-2004-diagnostic-criteria
#3
Alex K Bonnecaze, Wesley G Willeford, Peter Lichstein, Jill Ohar
Hemophagocytic lymphohistiocytosis (HLH) is a rare and often deadly syndrome characterized by severe inflammation and cytokine dysregulation. The disease is defined by the HLH-2004 criteria, requiring five of eight findings, and is further differentiated into either primary or secondary causes. Primary HLH tends to be of genetic etiology, while secondary HLH results from other insults such as infection. Secondary HLH is most commonly associated with viral infections in immunocompromised patients. Acute cytomegalovirus (CMV) associated HLH in the immunocompetent host is exceedingly rare and only documented in four case reports to date...
March 2, 2017: Curēus
https://www.readbyqxmd.com/read/28404538/haematopoietic-stem-cell-transplantation-in-primary-immunodeficiency-patients-in-the-black-sea-region-of-turkey
#4
Alişan Yıldıran, Mehmet Halil Çeliksoy, Stephan Borte, Şükrü Nail Güner, Murat Elli, Tunç Fışgın, Emel Özyürek, Recep Sancak, Gönül Oğur
OBJECTIVE: Haematopoietic stem cell transplantation is a promising curative therapy for many combined primary immunodeficiencies and phagocytic disorders. MATERIALS AND METHODS: We retrospectively reviewed paediatric cases that were diagnosed with primary immunodeficiencies and scheduled for haematopoietic stem cell transplantation. RESULTS: We identified 22 patients (median age, 6 months; age range, 1 month to 10 years) with various diagnoses who received haematopoietic stem cell transplantation...
April 13, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28403691/novel-nlrc4-mutation-causes-a-syndrome-of-perinatal-autoinflammation-with-hemophagocytic-lymphohistiocytosis-hepatosplenomegaly-fetal-thrombotic-vasculopathy-and-congenital-anemia-and-ascites
#5
Jiancong Liang, Danielle N Alfano, James E Squires, Melissa M Riley, W Tony Parks, Julia Kofler, Areeg El-Gharbawy, Suneeta Madan-Kheterpal, Roxanne Acquaro, Jennifer Picarsic
Autoinflammatory diseases are caused by pathologic activation of the innate immune system. Primary hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation caused by monogenic mutations resulting in cytotoxic cell defects and subsequent failure to eliminate activated macrophages. Secondary HLH is often diagnosed in cases without a known Mendelian inheritance. However, some cases of "secondary" HLH have been shown to harbor mutations with partial dysfunction of the cytotoxic system...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28395106/primary-congenital-immunodeficiency-2015-sh-eahp-workshop-report-part-5
#6
Dita Gratzinger, Elaine S Jaffe, Amy Chadburn, John K C Chan, Daphne de Jong, John R Goodlad, Jonathan Said, Yasodha Natkunam
Objectives: The 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review primary immunodeficiency and related lymphoproliferations. Methods: Primary immunodeficiencies were divided into immune dysregulation, DNA repair defects, low immunoglobulins, and combined immunodeficiencies. Results: Autoimmune lymphoproliferative syndrome (ALPS) is a prototypical immune dysregulation-type immunodeficiency, with defects in T-cell signaling or apoptosis, expansion of T-cell subsets, and predisposition to hemophagocytic lymphohistiocytosis...
February 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28357189/griscelli-syndrome-subtype-2-with-hemophagocytic-lympho-histiocytosis-a-case-report-and-review-of-literature
#7
Priyanka Minocha, Richa Choudhary, Anika Agrawal, Sadasivan Sitaraman
Griscelli syndrome (GS) is a rare autosomal recessive disorder resulting in pigmentary dilution of the skin and hair with variable phenotypes depending upon subtypes. Mutations in 3 distinct genes MYO5A, RAB27A, MLPH are responsible for 3 subtypes (GS1, GS2, and GS3) of GS respectively. GS subtype 2 commonly develops hemophagocytic lymphohistiocytosis (HLH) and recurrent infections due to immunodeficiency. We hereby report a 20 month old male child presenting with silvery gray hair, hypomelanosis and features of hemophagocytosis...
February 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28355352/a-novel-frameshift-mutation-of-chediak-higashi-syndrome-and-treatment-in-the-accelerated-phase
#8
X L Wu, X Q Zhao, B X Zhang, F Xuan, H M Guo, F T Ma
Chediak-Higashi syndrome (CHS) is a rare autosomal recessive immunodeficiency disease characterized by frequent infections, hypopigmentation, progressive neurologic deterioration and hemophagocytic lymphohistiocytosis (HLH), known as the accelerated phase. There is little experience in the accelerated phase of CHS treatment worldwide. Here, we present a case of a 9-month-old boy with continuous high fever, hypopigmentation of the skin, enlarged lymph nodes, hepatosplenomegaly and lung infection. He was diagnosed with CHS by gene sequencing, and had entered the accelerated phase...
March 23, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/28344134/cytomegalovirus-associated-hemophagocytic-syndrome-in-a-59-year-old-woman-with-ulcerative-colitis
#9
Chetan V Vakkalagadda, Ramiro Cadena-Semanate, Lemuel R Non
No abstract text is available yet for this article.
March 23, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28331130/cytopenias-and-clonal-expansion-of-gamma-delta-t-cells-in-a-patient-with-anaplasmosis-a-potential-diagnostic-pitfall
#10
Daniel Marko, Anamarija M Perry, Arjuna Ponnampalam, Michel R Nasr
Human granulocytic anaplasmosis is a rare, tick-borne infectious disease caused by Anaplasma phagocytophilum. Herein, we report a rare case of human granulocytic anaplasmosis associated with cytopenias and clonal expansion of gamma/delta T-cells in the bone marrow. A 77-year old man presented multiple times to the emergency department complaining of muscle weakness. Complete blood count detected cytopenias and peripheral blood smear showed pseudo Pelger-Huet neutrophils. These findings prompted bone marrow evaluation with ancillary studies including flow cytometry, karyotyping and T-cell rearrangement studies...
2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/28318633/capillary-leak-syndrome-etiologies-pathophysiology-and-management
#11
REVIEW
Eric Siddall, Minesh Khatri, Jai Radhakrishnan
In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a "sepsis-like" syndrome with manifestations of diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins...
March 16, 2017: Kidney International
https://www.readbyqxmd.com/read/28317816/hemophagocytic-syndrome-secondary-to-tuberculosis-at-24-week-gestation
#12
Alexandra Arteaga Fernández, David Fernández de Velasco Pérez, M C Jiménez Fournier, J C Moreno Del Prado, B Paraíso Torras, M L Cañete Palomo
Hemophagocytic syndrome is a life-threatening disease characterized by the uncontrolled activation of macrophages, resulting in hemophagocytosis of blood cells in the bone marrow. A 20-year-old gravida at 23-week and 5-day gestation was admitted to hospital to evaluate fever up to 104°F of unknown origin, moderate cytopenia, and elevated levels of liver enzymes. Bone marrow biopsy confirmed hemophagocytic syndrome, and polymerase chain reaction came back positive for Mycobacterium tuberculosis. Supportive care and tuberculosis treatment resulted in clinical improvement...
January 2017: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28296747/adult-onset-still-s-disease-with-atypical-cutaneous-manifestations
#13
Francisco Javier Narváez Garcia, María Pascual, Mercè López de Recalde, Pablo Juarez, Isabel Morales-Ivorra, Jaime Notario, Anna Jucglà, Joan M Nolla
The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28293160/hemophagocytic-histiocytosis-a-clinicopathological-correlation
#14
Waseem Iqbal, Abdulaziz Ajlan Alsalloom, Khalid Shehzad, Faisal Mughal, Zafar Rasheed
OBJECTIVES: Histiocytic hyperplasia with hemophagocytosis (HP) is relatively uncommon condition that has often been mistaken in the past for neoplastic disorders. This study was conducted to investigate the possible etiology of HP, its intensity in the bone marrow (BM), and also its effect on hematological parameters with the extent of disease activity. METHODS: Blood samples were collected and BM examination was performed in 250 patients with varied etiology showing HP...
January 2017: International Journal of Health Sciences
https://www.readbyqxmd.com/read/28278703/diagnostic-usefulness-of-plasma-presepsin-soluble-cd14-subtype-for-diagnosing-hemophagocytic-syndrome-in-hematological-malignancies
#15
Hideo Koh, Satoru Nanno, Takako Katayama, Asao Hirose, Mika Nakamae, Masayuki Hino, Hirohisa Nakamae
No abstract text is available yet for this article.
February 28, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28278074/a-clinical-study-of-21-patients-with-hemophagocytic-syndrome-in-295-cases-diagnosed-with-nasal-type-extranodal-nature-killer-t-cell-lymphoma
#16
Na Li, Li Zhang, Jie Liu, Jing Zhang, Hua-Wei Weng, Hong-Yu Zhuo, Li-Qun Zou
Nasal type, extranodal nature killer (NK)/T cell lymphoma-associated hemophagocytic syndrome (NK/T-LAHS) is a rare and fatal disorder with extremely poor prognosis. To investigate its clinical characteristics and risk factors, we retrospectively analyzed 295 patients with nasal type, extranodal nature killer/T cell lymphoma, of which 21 were diagnosed with hemophagocytic syndrome, with a cumulative incidence of 7.1%. The most frequently clinical characteristics were fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, hyperferritinemia, liver dysfunction, hypertriglyceridemia, hypofibrinogenemia and evaluated lactate dehydrogenase (LDH) level...
February 25, 2017: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/28249810/dynamic-changes-of-laboratory-parameters-and-peripheral-blood-lymphocyte-subsets-in-severe-fever-with-thrombocytopenia-syndrome-patients
#17
Jingwen Liu, Li Wang, Zhaolei Feng, Daying Geng, Ye Sun, Guangying Yuan
OBJECTIVES: The aim of this study was to dynamically investigate laboratory parameters and peripheral blood lymphocyte subsets in severe fever with thrombocytopenia syndrome (SFTS) patients at different stages, to evaluate the significance of these changes in the infection process and its influence on prognosis. METHODS: Case-control study was used in the research. Sixty-nine confirmed thrombocytopenia syndrome virus(SFTSV) infected patients were enrolled. They were divided into two groups, recovery group and poor prognosis group, according to the clinical prognosis of the diseases...
February 27, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28245389/-clinical-value-of-18-f-fdg-pet-ct-for-patients-with-b-cell-lymphoma-associated-hemophagocytic-syndrome
#18
Chong-Yang Ding, Hong-Yu Liu, Zhe Guo, Yang-Yang Li, Wen-Ping Yang, Tian-Nv Li
OBJECTIVE: To investigate the clinical value of (18)F-FDG PET/CT for patients with B cell lymphoma-associated hemophagocytic syndrome. METHODS: The clinical characteristics, laboratory parameters and (18)F-FDG PET/CT data of 23 newly diagnosed patients sufferred from B cell lymphoma-associated hemophagocytic syndrome were retrospectively analyzed. The correlation between PET and laboratory parameters were determined using Spearman correlation test. The prognostic factors were analyzed by the Kaplan-Meier method...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28225498/dengue-associated-hemophagocytic-lymphohistiocytosis-in-an-adult-a-case-report-and-literature-review
#19
REVIEW
Sang Mi Chung, Joon Young Song, Wonshik Kim, Min Joo Choi, Ji Ho Jeon, Seonghui Kang, Eunju Jung, Ji Yun Noh, Hee Jin Cheong, Woo Joo Kim
BACKGROUND: Infection-associated hemophagocytic syndrome (IAHS) is potentially a fatal disease caused by systemic infection complicated by hemophagocyticlymphohistiocytosis (HLH). Here, we report a case of HLH associated with dengue hemorrhagic fever (DHF) after a trip to Thailand. CASE SUMMARY: A 33-year-old healthy female patient presented with 3 days of fever, myalgia, and skin rash. Serotype 3 dengue virus was isolated. Clinical and laboratory findings fulfilled the criteria of HLH...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28219570/living-donor-lung-transplantation-after-bone-marrow-transplantation-for-chediak-higashi-syndrome
#20
Tetsu Yamada, Toyofumi F Chen-Yoshikawa, Shigeharu Oh, Rieko Ito-Taniguchi, Fumiaki Gochi, Masaaki Sato, Akihiro Aoyama, Hiroshi Date
An 8-year-old girl with Chediak-Higashi syndrome (CHS) had pulmonary complications after hematopoietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) and eventually underwent single living-donor lobar lung transplantation (LDLLT). Electron micrographic findings showed vagus nerve tissue in extracted lung having granular inclusions, which are pathognomonic for CHS. Because her mother was the donor for both hematopoietic stem cell and lung transplantations, she was weaned from immunosuppression and is doing well 3 years after lung transplantation...
March 2017: Annals of Thoracic Surgery
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