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Hemophagocytosis

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https://www.readbyqxmd.com/read/28521652/case-report-patient-with-unexplained-high-fever-and-pancytopenia
#1
Kelly De Schuyter, Tom Lodewyck
OBJECTIVE AND IMPORTANCE: We present a case of hemophagocytic lymphohistiocytosis (HLH), giving insight in how to establish diagnosis and start appropiate treatment. CLINICAL PRESENTATION: A 45-year-old male presented at the emergency ward with high fever and pancytopenia. Repeat bone marrow aspirates showed hemophagocytosis. Extensive work-up with exclusion of other infectious and malignant diseases, eventually lead us to the diagnosis of hemophagocytic lymphohistiocytosis...
May 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28494931/hemophagocytic-lymphohistiocytosis-in-acute-african-swine-fever-clinic
#2
Z R Karalyan, Z R Ter-Pogossyan, N Yu Karalyan, Z B Semerjyan, M R Tatoyan, S A Karapetyan, E M Karalova
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) usually has been defined as the combination of a proliferation of cytologically benign, actively phagocytic macrophages in bone marrow, spleen, lymph nodes, etc. in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia. HLH is often triggered by viral infection. The aim of this study was to ascertain the features of HLH involvement in African swine fever virus (ASFV) (genotype II) pathogenesis. METHODS: The serum levels of macrophage colony-stimulating factor (MCSF) and granulocyte-macrophage colony-stimulating factor (GMCSF), as well as the histological constitution (for hemophagocytic macrophages detection) of various organs of pigs infected with ASFV genotype II were investigated...
May 2017: Veterinary Immunology and Immunopathology
https://www.readbyqxmd.com/read/28489771/chronic-active-epstein-barr-virus-infection-associated-with-hemophagocytic-syndrome-and-extra-nodal-natural-killer-t-cell-lymphoma-in-an-18-year-old-girl-a-case-report
#3
Yawei Xing, Junwen Yang, Guanghui Lian, Shuijiao Chen, Linlin Chen, Fujun Li
RATIONALE: Chronic active Epstein-Barr virus infection (CAEBV) associated with hemophagocytic syndrome (HPS) and extra-nodal natural killer (NK)/T-cell lymphoma (ENKL) is a rare life-threatening disorder. This disease is easily misdiagnosed because of its varied presentations. PATIENT CONCERNS: An 18-year-old girl was admitted to our hospital with a history of edema in the lower limbs and intermittent fever lasting for more than 1 month. At admission, she had severe liver injury of unknown etiology...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28459018/visceral-leishmaniasis-associated-hemophagocytosis-a-tale-of-two-unexpected-diagnoses-from-a-nonendemic-region
#4
Rashmi Kaul Raina, Sujeet Raina, Manupriya Sharma
A case of visceral leishmaniasis (VL)-associated hemophagocytic lymphohistiocytosis (HLH) in an immunocompetent native from a nonendemic area was reported. The patient belonged to Ravi river valley area (altitude 996 meters above the mean sea level) of Chamba, Himachal Pradesh, India. VL and HLH were not a differential diagnosis. Identification of the Leishman-Donovan bodies and hemophagocytosis in bone marrow aspirate and biopsy provided the diagnosis. The patient recovered to the treatment with amphotericin B...
January 2017: Tropical Parasitology
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/28376538/-hepatic-manifestation-of-a-macrophage-activation-syndrome-mas
#6
Michael Nagel, Andreas Schwarting, Beate K Straub, Peter R Galle, Tim Zimmermann
Background Elevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure...
May 2017: Zeitschrift Für Gastroenterologie
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/28356667/hemophagocytic-lymphohistiocytosis-secondary-to-hemodialysis-catheter-related-blood-stream-infection
#8
U Anandh, S Johari, B Vaswani
A 57-year-old man on dialysis presented with fever due to Pseudomonas septicemia. Workup revealed very high triglycerides and serum ferritin levels. A bone marrow examination showed hemophagocytosis. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made and steroids were started. He was put on automated peritoneal dialysis. Patients' condition continued to deteriorate and he succumbed to his illness. This case illustrates the development of HLH secondary to infections which are increasingly being recognized in the literature...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28349614/progressive-histoplasmosis-with-hemophagocytic-lymphohistiocytosis-and-epithelioid-cell-granulomatosis-a-case-report-and-review-of-the-literature
#9
Arik Bernard Schulze, Britta Heptner, Torsten Kessler, Birgit Baumgarten, Viorelia Stoica, Michael Mohr, Rainer Wiewrodt, Viktoria Susanne Warneke, Wolfgang Hartmann, Jörg Wüllenweber, Christoph Schülke, Michael Schäfers, Dunja Wilmes, Karsten Becker, Lars Henning Schmidt, Andreas H Groll, Wolfgang E Berdel
Histoplasmosis in central Europe is a rare fungal disease with diverse clinical presentations. Apart from acute pulmonary histoplasmosis and involvement of the central nervous system, the most serious clinical presentation is progressive disseminated histoplasmosis which is generally associated with severe immunodeficiency and, in particular, advanced human immunodeficiency virus infection. Here, we report on an immunocompetent female residing in a non-endemic area, presenting with progressive disseminated histoplasmosis after a remote travel history to Thailand and Costa Rica...
March 27, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28317816/hemophagocytic-syndrome-secondary-to-tuberculosis-at-24-week-gestation
#10
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/28293160/hemophagocytic-histiocytosis-a-clinicopathological-correlation
#11
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/28196537/characterization-of-a-novel-disease-causing-mutation-in-exon-1-of-sh2d1a-gene-through-amplicon-sequencing-a-case-report-on-hlh
#12
Shiyuan Zhou, Hongyu Ma, Bo Gao, Guangming Fang, Yi Zeng, Qing Zhang, GaoFu Qi
BACKGROUND: Hemophagocytic lymphohistocytosis (HLH) is a rare but fatal hyperinflammatory syndrome caused by uncontrolled proliferation of activated macrophages and T lymphocytes secreting high amounts of inflammatory cytokines. Genetic defect is a common cause of HLH. HLH is complicated to be diagnosed as there are many common symptoms with other disorders. CASE PRESENTATION: Here we report on an HLH case caused by 1 bp deletion in gene SH2D1A. Patient was a 3-years-old boy and had fever for more than 8 days...
February 14, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28193996/a-case-report-of-primary-nasal-natural-killer-nk-t-cell-lymphoma-in-an-african-american-patient-presenting-with-hemophagocytic-syndrome
#13
Bowei Tan, Cherif Abdelmalek, James E O'Donnell, Thomas Toltaku, Rashid Chaudhry, Jen C Wang, Vladimir Gotlieb
BACKGROUND Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. CASE REPORT A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1...
February 14, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28191297/leishmania-infantum-and-epstein-barr-virus-co-infection-in-a-patient-with-hemophagocytosis
#14
Zied Gaifer, Mohamed-Rachid Boulassel
The authors describe a rare case of a 27- year old previously healthy male presenting with high grade fever, pancytopenia, hepatosplenomegaly, high levels of ferritin and triglyceride, suggesting a diagnosis of hemophagocytic lymphohistiocytosis (HLH) syndrome. Other investigations showed a positive Leishmania infantum serology and high Epstein-Barr virus (EBV) viremia. The diagnosis of a visceral leishmaniasis was confirmed by bone morrow biopsy, which showed Leishman-Donovan bodies and evidence of HLH. The patient received liposomal amphotericin B and he had a complete resolution of his symptoms and clearance of EBV viremia...
December 31, 2016: Infectious Disease Reports
https://www.readbyqxmd.com/read/28188950/hemophagocytic-lymphohistiocytosis-in-a-neonate-case-report
#15
Pari Zarrini, Ziba Mosayebi, Asghar Ramyar, Hosein Dalili
 Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This article will introduce a neonate with HLH in Iran. We report a case of HLH presenting with respiratory distress and fever, hepatosplenomegaly, jaundice and pancytopenia on the second day of life. Typical clinical and laboratory findings were detected in the neonate. HLH was diagnosed according to HLH-2004 guidelines...
January 2017: Acta Medica Iranica
https://www.readbyqxmd.com/read/28178007/role-of-skin-biopsies-in-the-diagnosis-of-hemophagocytic-lymphohistiocytosis
#16
Aileen Santos-Arroyo, Julián Barrera-Llaurador, Julio E Sánchez, Rafael Martín-García, Jorge L Sánchez
This is a report of a 15-year-old female who presented with a skin eruption and clinical features consistent with severe sepsis and septic shock. A diagnosis of acquired hemophagocytic lymphohistiocytosis (HLH) was made and confirmed by bone marrow analysis. Skin biopsy showed hemophagocytosis and CD163-positive staining of macrophages. We briefly review the clinical, histologic, and laboratory findings of hemophagocytic lymphohistiocytosis in addition to the potential role of skin biopsies in this condition...
January 26, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28149024/extremely-high-ferritinemia-associated-with-haemophagocytic-lympho-histiocytosis-hlh
#17
Neelima Verma, Jyoti Chakraverty, Pankaj Baweja, Alka Girotra, Leena Chatterjee, Manish Chugh
Hyperferritinemia (>10,000 ng/ml) is an important hallmark used as an indicator of infection triggered macrophage activation syndrome leading to hemophagocytic lympho histiocytosis (HLH). Measurement of serum ferritin can be used in diagnosis as well as disease monitoring indicator and prognosis related to HLH, cAPS, sepsis, neoplasm and inflammatory conditions. It is a major contributor to manage critically ill patients as predicting and monitoring indicator. It can be used as acute phase response in conditions of MAS, AOSD, cAPS etc...
March 2017: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/28024491/-children-s-nk-t-cell-lymphoma-associated-hemophagocytic-syndrome-clinical-analysis-of-6-cases
#18
Yong-Zhi Zheng, Hao Zheng, Jian Li, Shao-Hua LE, Xue-Ling Hua, Zai-Sheng Chen, Ling Zheng, Mei Li, Yi-Qiao Chen, Qin-Li Gao, Jing-Hui Yang, Jian-Da Hu
OBJECTIVE: To study the clinical features, treatment and prognosis of patients with NK/T cell lymphoma-associated hemophagocytic syndrome(NK/T-LAHPS). METHODS: Retrospective analysis was used to explore the clinical data of 6 children with NK/T-LAHPS who were admitted in Department of Pediatric Hematology of Fujian Medical University Union Hospital from July 2012 to June 2016. The 6 patients included 4 boys and 2 girls, with a median age of 4 years(range 1.75 to 11)...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27998299/rituximab-etoposide-methylprednisolone-high-dose-cytarabine-and-cisplatin-in-the-treatment-of-secondary-hemophagocytic-lymphohistiocytosis-with-classical-hodgkin-lymphoma-a-case-report-and-review-of-the-literature
#19
REVIEW
Steve Hu, Pranshu Bansal, David Lynch, Cristhiam Mauricio Rojas Hernandez, Zoneddy Dayao
BACKGROUND: Hemophagocytic lymphohistiocytosis is becoming an increasingly recognized disorder in adults. Classical Hodgkin lymphoma is a relatively uncommon etiology of hemophagocytic lymphohistiocytosis and may complicate treatment options. Rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin are discussed here as a treatment regimen. CASE PRESENTATION: A 66-year-old Hispanic man previously in good health presented with a 1-month history of recurrent fevers, chills, and night sweats and a 3-week history of new onset jaundice...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27990393/subcutaneous-panniculitis-like-t-cell-lymphoma-with-macrophage-activation-syndrome-treated-by-cyclosporine-and-prednisolone
#20
Dinesh P Asati, Vaibhav Ingle, Deepti Joshi, Anurag Tiwari
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL; α/β T-cell subtype) is a distinct variantof cutaneous T-cell lymphomas, which presents as inflammatory subcutaneous nodules. A 17-year-old male presented with recurrent fever with concomitant facial swelling, pedal edema, hepatosplenomegaly, and mildly tender subcutaneous plaques in generalized distribution along with patches of scarring alopecia on scalp. There were features of macrophage activation syndrome in the form of hemophagocytosis in the bone marrow, pancytopenia, high serum lactate dehydrogenase levels, low fibrinogen clotting activity, prolonged activated prothrombine time (aPTT), increased serum ferritin, hypoalbuminemia, and hypertriglyceridemia...
November 2016: Indian Dermatology Online Journal
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