keyword
MENU ▼
Read by QxMD icon Read
search

Hemophagocytic lymphohistiocytosis

keyword
https://www.readbyqxmd.com/read/29776323/low-frequency-sensorineural-hearing-loss-in-familial-hemophagocytic-lymphohistiocytosis-type-5
#1
Aren Bezdjian, Hanneke Bruijnzeel, Julia Pagel, Sam J Daniel, Hans G X M Thomeer
INTRODUCTION: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased. CASE PRESENTATION: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature...
June 2018: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/29766733/-hemophagocytic-lymfohistiocytosis-in-adults-review-and-case-report
#2
Jiří Šrámek, Thomas Karvunidis, Daniel Lysák, Martin Harazim, Michal Karas, Pavel Jindra
Hemophagocytic lymfohistiocytosis (HLH) is rare, life-threatening condition, characterized by excessive activation of immune system with subsequent proinflammatory state resulting in multiorgan failure. Most frequently, it appears in infancy as a primary disorder caused by mutation of immune-regulatory genes. Increasingly, HLH is being diagnosed as a secondary - adult - form, which occurs as a result of aberrant immune response. Viral or bacterial systemic infections, malignancy with a predominance of lymphoproliferative disorders and autoimmune diseases are the most common triggers...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29755935/aspergillus-endocarditis-of-the-mitral-valve-with-ventricular-myocardial-invasion-cerebral-vasculitis-and-intracranial-mycotic-aneurysm-formation-in-a-patient-with-hemophagocytic-lymphohistiocytosis
#3
Bibin Varghese, Kevin Ting, Juan Lopez-Mattei, Cezar Iliescu, Joseph Kim, Peter Kim
Aspergillus endocarditis is a rare infection and reported mainly in immunocompromised hosts. We report a case of mitral valve aspergillus endocarditis with ventricular myocardial invasion, cerebral vasculitis and intracranial fungal aneurysm formation in a patient with hemophagocytic lymphohistiocytosis (HLH). This case illustrates the importance of prompt investigation and treatment of masses seen on an echocardiogram for rare infections such as Aspergillus endocarditis in immunocompromised patients.
September 2018: Medical Mycology Case Reports
https://www.readbyqxmd.com/read/29754257/fatal-unexpected-death-due-to-familial-hemophagocytic-lymphohistiocytosis-type-3
#4
Jiao Mu, Chunting Jin, Zhenglian Chen, Jianfeng Li, Bin Lv, Hongmei Dong
Familial hemophagocytic lymphohistiocytosis is a rare autosomal recessive disorder of immune dysregulation associated with uncontrolled activation of cytotoxic T cells and macrophages. Herein, we report a case of a 14-month-old Chinese boy who presented with fever, abdominal distension and thrombopenia, and died within 3 days of admission to the hospital. Postmortem examination revealed pleuroperitoneal fluid, enlarged mesenteric lymph nodes and hepatosplenomegaly. Histopathological examination showed interstitial pneumonia, hepatonecrosis and hemophagocytosis...
May 12, 2018: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/29747294/-a-case-report-of-h-7-n-9-avian-influenza-complicated-by-hemophagocytic-lymphohistiocytosis
#5
W Yang, X J Zhang, W Yang
No abstract text is available yet for this article.
May 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29746438/hemophagocytic-lymphohistiocytosis-associated-with-visceral-leishmaniasis
#6
Nihal Özdemir, Başak Koç, Esra Arslantaş, Işik Odaman Al, Çiğdem Kelleci, Ezgi Pasli Uysalol, Cengiz Bayram, Ali Ayçiçek
This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol. Repeated bone marrow aspiration showed amastigotes on follow-up. In endemic countries, visceral leishmaniasis should be considered in the differential diagnosis to avoid chemotherapy toxicity.
May 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29743400/-epstein-barr-virus-associated-hemophagocytic-lymphohistiocytosis-subsequent-to-rabbit-antithymocyte-globulin-administration-and-successfully-treated-with-rituximab-in-a-patient-with-aplastic-anemia
#7
Hiroaki Nagata, Takahiro Fujino, Ayako Muramatsu, Yuka Kawaji, Kodai Kuriyama, Muneo Oshiro, Yoshiko Hirakawa, Toshiki Iwai, Hitoji Uchiyama
Rabbit antithymocyte globulin (ATG) is an effective immunosuppressive therapy for patients with aplastic anemia (AA). However, Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a rare but serious complication of the therapy. An 81-year-old man was diagnosed with severe AA on the occasion of melena. Because cyclosporine monotherapy did not improve his condition, rabbit ATG was additionally administered. Thirty-one days after the administration of rabbit ATG, the patient presented with fever and general malaise...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29741568/hemophagocytic-lymphohistiocytosis-associated-with-dabrafenib-and-trametinib-combination-therapy-following-pembrolizumab-administration-for-advanced-melanoma
#8
K Sasaki, J Uehara, S Iinuma, H Doi, M Honma, Y Toki, A Ishida-Yamamoto
No abstract text is available yet for this article.
May 7, 2018: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/29712633/how-i-treat-t-cell-chronic-active-epstein-barr-virus-disease
#9
Catherine M Bollard, Jeffrey I Cohen
T cell chronic active EBV is a rare disease in which EBV is present predominantly in T cells which infiltrate the tissues; patients have high levels of EBV in the blood. If untreated, patients often develop liver failure, hemophagocytic lymphohistiocytosis, coronary artery aneurysms, EBV infiltrating T cells impairing organ function, or T cell lymphomas refractory to treatment. At present, hematopoietic stem transplantation is the only curative therapy, and it is critical to make a proper diagnosis and initiate transplantation before the disease progresses to an irreversible stage...
April 30, 2018: Blood
https://www.readbyqxmd.com/read/29705274/early-onset-lysosomal-acid-lipase-deficiency-presenting-as-secondary-hemophagocytic-lymphohistiocytosis-two-infants-treated-with-sebelipase-alfa
#10
Ermelinda Santos Silva, Maja Klaudel-Dreszler, Agnieska Bakuła, Teresa Oliva, Tereza Sousa, Paula Cristina Fernandes, Anna Tylki-Szymańska, Elena Kamenets, Esmeralda Martins, Piotr Socha
Two unrelated infants were diagnosed with and initially treated for hemophagocytic lymphohistiocytosis (HLH), but progressed to cholestasis and liver failure. Early onset lysosomal acid lipase deficiency (EO-LAL-D) was suspected due to lymphocytes with cytoplasmic vacuolation and/or adrenal calcifications and confirmed by enzymatic and genetic analysis. Enzyme replacement therapy with sebelipase alfa was implemented, but both children died, despite initial improvement. Since this inborn error of metabolism progresses rapidly in infants, early diagnosis is crucial, and appropriate treatment should be started as soon as possible...
April 25, 2018: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29702543/a-novel-mutation-c-153-c-a-in-a-tunisian-girl-with-wolman-disease-and-unusual-presentation-hemophagocytic-lymphohistiocytosis
#11
Faten Tinsa, Manel Ben Romdhane, Hela Boudabous, Imen Bel Hadj, Ines Brini, Neji Tebib, Hela Louati, Soumeya Bekri, Khadija Boussetta
Wolman disease is an ultrarare lysosomal storage disease caused by a mutation in the LIPA gene. The clinical features of Wolman disease include early onset of vomiting, diarrhea, failure to thrive, hepatosplenomegaly, and bilateral adrenal calcification. We report the case of a 3-month-old infant who presented clinical features of hemophagocytic lymphohistiocytosis. Genetic sequence analysis of the LIPA gene revealed homozygous mutation c.153 C>A (p.Tyr51*). The parents were heterozygous for this mutation...
April 26, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29695416/deletion-of-inflammasome-components-is-not-sufficient-to-prevent-fatal-inflammation-in-models-of-familial-hemophagocytic-lymphohistiocytosis
#12
Sébastien Fauteux-Daniel, Sébastien Viel, Laurie Besson, Jiang Zhang, Marie Marotel, Anne-Laure Mathieu, Rémi Pescarmona, Emily Charrier, Thomas Henry, Alexandre Belot, Thierry Walzer
Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory condition that occurs in patients with genetic defects of cytotoxicity (familial HLH [FHL]) or secondary to other immunological disorders such as juvenile idiopathic arthritis. HLH is characterized by elevated levels of serum IL-18 and other cytokines. Moreover, a novel clinical entity has been recently identified in which constitutive NLRC4 inflammasome activation leads to severe HLH. Altogether, these clinical observations suggest that inflammasome activation is a central event in the development of all HLH forms and that inflammasome blockade could alleviate inflammation in FHL patients...
April 25, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29692937/ebv-associated-lymphoproliferative-disorder-and-hemophagocytic-lymphohistiocytosis-in-a-patient-with-severe-celiac-disease
#13
John Jacob Kinross-Wright, Kalyan Chakravarthy Potu, Brandy Pownell, Randall Lamfers, Jonathan S Bleeker
Background: Epstein-Barr virus- (EBV-) associated lymphoproliferative disease (LPD) is a rare condition, usually occurring in immunocompromised patients. We report a case of EBV-associated LPD in a patient with severe celiac disease, the first report to describe this syndrome in a patient with this diagnosis. Case Summary: A 69-year-old Caucasian woman with recent diagnosis of celiac sprue presented to our hospital with persistent diarrhea, abdominal pain, weight loss, and fatigue despite adherence to gluten-free diet for a number of weeks prior to presentation...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29687855/lymphocyte-subsets-in-children-with-hemophagocytic-lymphohistiocytosis-hlh-and-its-clinical-significance
#14
Q An, D-H Fang, C-M Xuan, S-M Xu, M-W Jin, Q Ji
OBJECTIVE: To study the peripheral blood lymphocyte subsets in children with hemophagocytic lymphohistiocytosis (HLH) in acute period as well as remission period, and compare them with healthy children to investigate the significance of lymphocyte subsets in the diagnosis, treatment, and prognosis in children with HLH. PATIENTS AND METHODS: From January 2009 to March 2014, 30 HLH patients were enrolled in this study. Among them, 20 were placed in the remission group, while 10 cases were placed in the death group...
April 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29687211/dual-threat-of-epstein-barr-virus-an-autopsy-case-report-of-hiv-positive-plasmablastic-lymphoma-complicating-ebv-associated-hemophagocytic-lymphohistiocytosis
#15
Yusuke Koizumi, Ken-Ichi Imadome, Yasunori Ota, Hitoshi Minamiguchi, Yoshinori Kodama, Dai Watanabe, Hiroshige Mikamo, Tomoko Uehira, Seiji Okada, Takuma Shirasaka
Epstein-Barr virus (EBV) reactivation causes serious diseases in immunocompromised hosts, such as acquired immunodeficiency syndrome (AIDS). We report on a case of plasmablastic lymphoma (PBL) with hemophagocytic lymphohistiocytosis (HLH).A-53-year-old Japanese man was diagnosed with PBL and AIDS. In addition to combined antiretroviral therapy, HyperCVAD (cyclophosphamide, doxorubicin, vincristine, prednisone)/high-dose methotrexate + cytarabine was initiated immediately. Partial remission was attained with chemotherapy...
April 23, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29686911/secondary-hlh-case-report-highlighting-clinical-challenges
#16
Riad El Fakih, Said Y Mohamed, Randa Alnounou, Ghada Elgohary
A 19-year-old patient with relapsed acute myeloid leukemia (AML) developed severe and prolonged cytopenia and unexplained jaundice and fever after salvage chemotherapy. His workup revealed hemophagocytosis on the bone marrow biopsy. He was treated for HLH (hemophagocytic lymphohistiocytosis) secondary to AML and chemotherapy. The patient died on day 56 after starting his salvage chemotherapy. Unexpectedly, after his death, the microbiology laboratory reported positive mycobacterial growth from a bronchoalveolar lavage (BAL) sample taken during the workup of his fever...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/29683949/a-rare-case-of-hemophagocytic-lymphohistiocytosis-associated-with-myelodysplastic-syndrome-and-trisomy-8-in-a-pediatric-patient
#17
Seh Hyun Kim, Dae Yong Yi, Na Mi Lee, Sin Weon Yun, Soo Ahn Chae, In Seok Lim
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting in clinical and biochemical manifestations of extreme inflammation. Myelodysplastic syndrome (MDS) represents a heterogenous group of clonal hematopoietic disorders. The development of MDS is common in children with trisomy of chromosome 8. Here, we report a fatal case of 8-year-old girl who was admitted to the emergency department with status epilepticus, and later diagnosed with HLH associated with MDS and trisomy of chromosome 8. We believe this is the first reported case of HLH associated with MDS and trisomy 8 in a pediatric patient...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29682376/a-case-of-spontaneously-improving-secondary-hemophagocytic-lymphohistiocytosis-in-an-adult-associated-with-t-cell-histiocyte-rich-large-b-cell-lymphoma
#18
Cyrus Askin, Ashley Burris, Clifton Layman, Brian Haney, Jordan Hall
Secondary hemophagocytic lymphohistiocytosis (HLH) in adults is a rare, often fatal syndrome characterized by widespread immune dysregulation. It is seen as a complication of infections, autoimmune diseases, and malignancies. Among the malignancy-related causes, aggressive T-cell or NK-cell neoplasms are most notable, while B-cell lymphomas are less commonly implicated. We present the case of a 32-year-old male transferred to our facility with concern for HLH. During the first week of his hospitalization, his diagnosis was confirmed and the patient demonstrated spontaneous improvement in his symptoms prompting us to delay therapy while searching for a primary cause...
2018: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29676380/fatal-meningococcal-septicemia-without-meningeal-signs-contribution-of-the-peripheral-smear-in-diagnosis-report-of-a-case
#19
Deepti Mutreja, Nikhil Moorchung, S J Manasa, Jeenu Varghese
Acute meningococcemia is characterized by extensive purpurae consisting of both petechiae and ecchymoses. This condition can be rapidly fatal without treatment due to shock and severe consumptive coagulopathy. We report a case of fatal meningococcal septicemia in a military recruit who presented with fever and associated rapidly progressive purpuric rash (purpura fulminans) without any meningeal signs. Evaluation revealed evidence of disseminated intravascular coagulopathy and multiorgan failure. Diplococci were demonstrated in peripheral blood neutrophils and monocytes...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29676379/hemophagocytic-lymphohistiocytosis-presenting-with-acute-liver-failure-and-central-nervous-system-involvement-in-early-infancy
#20
Mukesh Kumar, Nirupama Kothari, B D Gupta, Neeraj Gupta
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal and likely underdiagnosed disease characterized by unregulated histiocyte proliferation, hypercytokinemia and hemophagocytosis, causing life-threatening tissue damage and organ failure. We report a case of a 56-day-old infant presenting with fever, acute liver failure, and neurological manifestations as presenting features that succumbed to rapidly progressive HLH. Our objective is to emphasize the importance of early diagnosis by high suspicion in varied initial presentation of HLH so that life-saving therapy may be instituted in time...
April 2018: Indian Journal of Pathology & Microbiology
keyword
keyword
26835
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"