Yrd. Doç. Dr. Talia İleri Ankara Üniversitesi Tıp Fakültesi Pediatrik Hematoloji Bilim Dalı Tel : 0312 595 69 06 Gsm : 0505 597 04 92 E-Posta Adresi : [email protected] Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic condition characterized by activation of lymphohistiocytosis, leading to cytokine overproduction. The main clinical futures of HLH are prolonged fever and hepato...

Hemophagocytic lymphohistiocytosis (HLH) is a clinical condition characterized by macrophage and activated histiocyte proliferation, leading to uncontrolled phagocytosis of hematopoietic precursor cells. The clinical presentation is characterized by fever, pancytopenia, hepatosplenomegaly, and hemophagocytosis in the reticuloendothelial system. In addition to the primary form of the disorder, s...

Autophagy is an evolutionarily conserved intracellular system that maintains cellular homeostasis by degrading and recycling damaged cellular components. The transcription factor HLH-30/TFEB-mediated autophagy has been reported to regulate tolerance to bacterial infection, but less is known about the bona fide bacterial effector that activates HLH-30 and autophagy. Here, we reveal that bacteria...

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias and hepatosplenomegaly, as well as hemophagocytosis by activated, morphologically benign macrophages. HLH may be characterized into two forms, familial and secondary HLH. Familial HLH usually manifests in children with genetic...

X-linked lymphoproliferative syndrome (XLP) is a rare primary immunodeficiency characterized by increased vulnerability to Epstein-Barr virus infection. XLP type 1 is caused by mutations in SH2D1A, whereas X-linked inhibitor of apoptosis (XIAP) encoded by XIAP/BIRC4 is mutated in XLP type 2. In XIAP deficiency, hemophagocytic lymphohistiocytosis (HLH) occurs more frequently and recurrence is co...

Id helix-loop-helix (Id HLH) proteins are negative regulators of basic HLH transcription factors. They are expressed during embryonic development and are important for the regulation of cell phenotypes in adults. They participate in the molecular networks controlling cell growth, differentiation, and carcinogenesis, through specific basic HLH and non-basic HLH protein interactions. Recent in vi...

Background: Human luteinizing hormone (hLH) stimulates steroid biosynthesis of the ovary, triggers ovulation and prepares androgen production of testicular Leydig cells. LH belongs to the family of glycoprotein hormones that are heterodimers consisting of a common α-subunit and specific β-subunit. hLH is necessary for clinical and infertility treatment. Recombinant DNA technology provides a use...

HLH was first described by Farquhar and Claireaux in 1952 as a familial disease [1]. Interestingly in their cases hemophagocytosis, which has given the disease its name, could not be found during lifetime but was prominent on autopsy. Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder with an estimated frequency of 0.12/100 000 children per year [2]. Several g...

Hemophagocytic lymphohistiocytosis (HLH) is a rare inflammatory condition with tissue destruction due to abnormal immune activation. We present a series of 2 cases of cytomegalovirus-induced HLH in children during maintenance chemotherapy for acute lymphoblastic leukemia. These cases emphasize the importance of considering secondary HLH in this high-risk subset of pediatric patients.

Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, characterized clinically by fever, splenomegaly, cytopenia, and high ferritin. Infectious causes have been associated with secondary HLH, with viruses being the most common. We report a case of Mycobacterium avium complex-associated HLH in a sickle cell anemia patient. To the best of our knowledge, this association h...