Search results for "Hemophagocytic lymphohistiocytosi"
showing 10 items of 22 documents
Cytomegalovirus pneumonia in patients with inflammatory bowel disease: a systematic review
2012
Summary Background Inflammatory bowel disease (IBD) patients are at increased risk of cytomegalovirus (CMV) reactivation, and although CMV pneumonia may be a fatal disease in IBD patients, little information is available on this issue. The objectives of this study were to identify risk factors for the development of CMV pneumonia in IBD patients and to find useful information to better manage this potentially fatal complication. Methods A computerized search without language restrictions was conducted using PubMed and SCOPUS. An article was considered eligible for inclusion in the systematic review if it reported detailed data on patients with IBD presenting with pneumonia due to CMV. Resul…
Scrub typhus, acute respiratory distress, and hemophagocytic lymphohistiocytosis
2013
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Measles and Secondary Hemophagocytic Lymphohistiocytosis
2012
To the Editor: We found interesting the article by Lupo et al. about a case of fatal measles in an immunocompetent 29-year-old woman (Fatal measles without rash in immunocompetent adult, France; http://dx.doi.org/10.3201/eid1803.111300). Perhaps, however, the possible diagnosis of secondary hemophagocytic lymphohistiocytosis (HLH) should also have been considered in that setting. HLH is a potentially fatal hyperinflammatory syndrome characterized by histiocyte proliferation and hemophagocytosis. HLH may be inherited (i.e., primary, familial, generally occurring in infants) or may occur at any age secondary to infection, malignancy, or rheumatologic disease. Secondary HLH is determined accor…
Visceral leishmaniasis, hypertriglyceridemia and secondary hemophagocytic lymphohistiocytosis
2016
cytokines, an upregulation of adhesion molecules and MHC I and II molecules on mono/macrophages, and an expansion of inflammatory monocytes. This exaggerated inflammatory response is responsible for necrosis and organ failure and results in uncontrolled proliferation and phagocytic activity of histiocytes [2]. Hypertriglyceridemia (fasting, greater than or equal to 265 mg/100 ml) is one of the current diagnostic criteria for HLH [2]. Several studies link hypertriglyceridemia to inhibition of lipoprotein lipase (LPL) by tumor necrosis factor-α (TNF-α), and TNF-α is a powerful autocrine and paracrine regulator of adipose tissue [3]. Indeed, many different sources of intense and prolonged T-ly…
Peripheral T-cell lymphoma associated consecutively with hemophagocytic lymphohistiocytosis and hypereosinophilic syndrome
2003
: Both hemophagocytic lymphohistiocytosis and hypereosinophilic syndrome have been associated with hematologic neoplasms and are respectively related to an overproduction of the cytokines Thelper 1 (Th1) and Th2 by tumor cells or reactive cells. To our knowledge, this is the first time a case of a peripheral T-cell lymphoma consecutively associated with both paraneoplastic conditions has been reported. Importantly, in this case when the lymphoma exclusively involved the bone marrow, severe paraneoplastic systemic damage, a CD8+ suppressor/cytotoxic phenotype and a hypereosinophilia not related to high levels of interleukin (IL)-5 was found. Interestingly, progression of the lymphoma coinci…
Hemophagocytic syndrome in a patient with disseminated tuberculosis: a case report
2018
Hemophagocytic syndrome (HS) is a rare disorder of the immune system. It is characterized by fever, lymphadenopathy, hepatosplenomegaly, cytopenia and hyperferritinemia. The cause differs in each country suggesting a specific genetic background and epidemiology of infections, and it can be associated with malignant diseases. A rare cause of HS is tuberculosis (TB), we describe a case of HS associated with disseminated Mycobacterium tuberculosis (MT) infection in a patient from Sudan. He presented diarrhea, fever, pancytopenia, thickened and dilated bowel loops and lymph nodes enlargement at ultrasound and computed tomography scan. A bone marrow biopsy performed to rule out a lymphoma reveal…
Sepsis due to Streptococcus pneumoniae associated with secondary hemophagocytic lymphohistiocytosis in a splenectomized patient for spherocytosis
2017
Abstract Rationale: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome that is characterized by an inappropriate hyperinflammatory immune response – primary, as a consequence of a genetic defect of NK cells and cytotoxic T lymphocytes or – secondary, in the progression of infections, rheumatic or autoimmune diseases, malignancies or metabolic diseases. Patient concerns: We present the case of a secondary HLH due to Streptococcus pneumoniae infection in a splenectomised patient for spherocytosis, a 37-year-old patient who was splenectomised in childhood for spherocytosis, without immuneprophylaxis induced by antipneumococcal vaccine. Outcomes: He developed a severe pneumococcal sepsis as…
Secondary hemophagocytic lymphohistiocytosis: forget me not!
2012
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Secondary hemophagocytic lymphohistiocytosis in zoonoses. A systematic review
2012
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome that is often fatal despite treatment. It is caused by a dysregulation in natural killer T-cell function, resulting in activation and proliferation of histiocytes with uncontrolled hemophagocytosis and cytokines overproduction. The syndrome is characterized by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hyperferritinemia. HLH can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. AIM: To focus on secondary HLH complicating zoonotic diseases. MATERIALS AND METHODS: PubMed search of human cases of HLH occurring during zoonotic dise…
Alternative UNC13D Promoter Encodes a Functional Munc13-4 Isoform Predominantly Expressed in Lymphocytes and Platelets
2020
Autosomal recessive mutations in genes required for cytotoxicity are causative of a life-threatening, early-onset hyperinflammatory syndrome termed familial hemophagocytic lymphohistiocytosis (FHL). Mutations in UNC13D cause FHL type 3. UNC13D encodes Munc13-4, a member of the Unc13 protein family which control SNARE complex formation and vesicle fusion. We have previously identified FHL3-associated mutations in the first intron of UNC13D which control transcription from an alternative transcriptional start site. Using isoform specific antibodies, we demonstrate that this alternative Munc13-4 isoform with a unique N-terminus is preferentially expressed in human lymphocytes and platelets, as…