Disease relevance of Lymphohistiocytosis, Hemophagocytic

• CONCLUSION: Recognition of the acute lupus hemophagocytic syndrome and distinction from an infection-associated hemophagocytic syndrome is important because it responds well to steroid therapy [1].
• Epstein-Barr virus LMP1 inhibits the expression of SAP gene and upregulates Th1 cytokines in the pathogenesis of hemophagocytic syndrome [2].
• Peripheral T-cell lymphoma associated with hemophagocytic syndrome and hemophagocytic lymphohistiocytosis of children: do they share something [3]?
• Parvovirus B19-associated hemophagocytic syndrome [4].
• Recently, we have studied a 20-month-old boy with X-linked lymphoproliferative disease (XLP) who had succumbed to infectious mononucleosis (IM) complicated by fulminant hepatitis and virus-associated hemophagocytic syndrome following EBV infection [5].

High impact information on Lymphohistiocytosis, Hemophagocytic

• Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3) [6].
• The evaluation of patients presenting with a hemophagocytic syndrome should include serologic tests for systemic lupus erythematosus [1].
• Most patients with GS display the hemophagocytic syndrome and have mutations in RAB27A, which codes for a small GTPase [7].
• Severe imbalance of IL-18/IL-18BP in patients with secondary hemophagocytic syndrome [8].
• Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11 [9].

Chemical compound and disease context of Lymphohistiocytosis, Hemophagocytic

• In two other children with a diagnosis of infection-associated hemophagocytic syndrome without any neurologic disturbance, neopterin concentrations were also elevated but only to 10 times the concentrations in controls [10].
• Both patients were treated with oral cyclosporine in an effort to suppress the cytokine release from T-cells that has been thought to induce the hemophagocytic syndrome [11].
• Pancytopenia secondary to hemophagocytic syndrome in rheumatoid arthritis treated with methotrexate and sulfasalazine [12].
• He presented with hemophagocytic syndrome (HPS) at 6 months of age, but relapsed despite the repeated administration of prednisolone, VP-16, cyclosporin A (CsA), and other cytotoxic agents [13].
• Effective control of infection-associated hemophagocytic syndrome with chloroquine [14].

Anatomical context of Lymphohistiocytosis, Hemophagocytic

• Hemophagocytic lymphohistiocytosis, terminology that designates a syndrome that may be familial or sporadic, with or without an associated viral infection, is presented as the prototype of a hemophagocytic syndrome, a condition in which there is uncontrolled activation of the cellular immune system [15].
• A rheumatoid arthritis (RA) patient treated with low-dose methotrexate (MTX) therapy suffered from hemophagocytic syndrome (HPS) associated with B-cell lymphoma (B-LAHS) [16].
• Onset of a hemophagocytic syndrome characterized the course of several human inherited immune disorders, all of them resulting from molecular defects of the perforin-dependent cytotoxic process exerted by both T and Natural Killer (NK) lymphocytes [17].
• Clinical significance of simultaneous determination of serum soluble transferrin receptor, soluble Fas, soluble Fas ligand, hepatocyte growth factor and interleukin 18 levels in a patient with hemophagocytic syndrome [18].

Gene context of Lymphohistiocytosis, Hemophagocytic

• Soluble hemoglobin-haptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome [19].
• We also studied the role of IFN-gamma as a macrophage activator in hemophagocytic syndrome (HPS) [20].
• Mutations in Munc13-4 cause familial hemophagocytic lymphohistiocytosis subtype 3 (FHL3), a disease phenotypically related to GS2 [21].
• These results provide evidence for MIP-1alpha chemokine expression in tissues from patients with HPS and suggest that MIP-1alpha may play an important role in the pathogenesis of the hemophagocytic syndrome [22].
• Mutation analysis was carried out of samples obtained from 14 patients with EBV-associated hemophagocytic syndrome by sequencing the genomic SAP/SH2D1A/DSHP and perforin genes [23].

Analytical, diagnostic and therapeutic context of Lymphohistiocytosis, Hemophagocytic

• Seventeen cases (age at onset, 1 month to 18 years; M/F, 9/8) of hemophagocytic syndrome which received allogeneic hematopoietic stem cell transplantation (SCT) in Japan during the period 1988-1998 are reported [24].
• The latency pattern and EBNA promoter (Wp, Cp, and Qp) usage in EBV-infected cells from three patients with EBV-associated hemophagocytic syndrome were examined by reverse transcription-polymerase chain reaction (PCR) [25].
• We report a case of secondary hemophagocytic syndrome after chemotherapy for multiple myeloma, which responded dramatically to dexamethasone, etoposide, and cyclosporin A [26].
• Virus-associated hemophagocytic syndrome in an international traveler as a differential diagnosis of SARS [27].

References