PMC:6141714 / 29318-32070
Annnotations
{"target":"https://pubannotation.org/docs/sourcedb/PMC/sourceid/6141714","sourcedb":"PMC","sourceid":"6141714","source_url":"https://www.ncbi.nlm.nih.gov/pmc/6141714","text":"Hemophagocytic syndrome is an autoimmune disease characterized by the activation and proliferation of macrophages, T CD8+ lymphocytes and NK cells in the bone marrow and in other endothelial reticular systems (172), leading to the phagocytosis of erythrocytes, leukocytes, platelets, and their precursors and to the exacerbated production of several inflammatory cytokines, including IL-1β, IL-2, IL-6, TNF-α, and IFN-γ (173). The hemophagocytic syndrome may be primary when triggered by genetic factors or secondary when occurs due to infections, neoplasms, rheumatic diseases, HSCT or other autoimmune disorders. The clinical manifestations of the hemophagocytic syndrome include hyperferritinemia, fever, hepatosplenomegaly and cytopenias (174). In this systematic review, hMSCs were used for the treatment of the hemophagocytic syndrome in only two studies (52, 73); both of which had the bone marrow as the source of hMSCs and were conducted in humans. Both the studies selected were single case reports and used the platelet, leukocyte and reticulocyte number, the amount of hemophagocytosis of erythroblasts and myeloid cells and the serum levels of ferritin and lactate dehydrogenase as primary study endpoints. The serum levels of pro-inflammatory and immunomodulatory cytokines such as IL-1β, IL-6, IL-8, IL-10, IL-17, and IL-15 were used as secondary study endpoints as they provide a strong evidence of the efficacy of the hMSCs administration inmodulating autoimmunity. Due to the fact that the hemophagocytic syndrome is a universally fatal disease if untreated, we recommend the use of a primary endpoint that is able to reflect a change in lethality following hMSCs administration, such as the cumulative survival rate. Other outcomes such as neutropenia, occurrence of relapses and serum levels of cytokines should be used in conjunction with the primary endpoint to correctly assess the potential of hMSCs administration for the treatment of hemophagocytic syndrome. After hMSCs administration, a decrease in the severity of the disease was observed in both studies following the administration of hMSCs. Mougiakakos et al. (73) described that this decrease in the severity of the disease was accompanied by a reduction in the serum levels of lactate dehydrogenase, ferritin and triglycerides. This study also described an increase in the levels of the immunomodulatory cytokine IL-10 and a decrease in the levels of the inflammatory cytokines IL-8, IL-6, IL-15, IL-17, and TNF-α after treatment with hMSCs. Table 1 summarizes the methodology employed and the results obtained in the studies selected in this systematic review regarding the effects of the administration of hMSCs for the treatment of GvHD and hemophagocytic syndrome.","tracks":[{"project":"MyTest","denotations":[{"id":"30254638-29181134-35421108","span":{"begin":210,"end":213},"obj":"29181134"},{"id":"30254638-24966707-35421109","span":{"begin":421,"end":424},"obj":"24966707"},{"id":"30254638-17149063-35421110","span":{"begin":743,"end":746},"obj":"17149063"},{"id":"30254638-17869550-35421111","span":{"begin":862,"end":864},"obj":"17869550"},{"id":"30254638-22738174-35421112","span":{"begin":866,"end":868},"obj":"22738174"},{"id":"30254638-22738174-35421113","span":{"begin":2143,"end":2145},"obj":"22738174"}],"namespaces":[{"prefix":"_base","uri":"https://www.uniprot.org/uniprot/testbase"},{"prefix":"UniProtKB","uri":"https://www.uniprot.org/uniprot/"},{"prefix":"uniprot","uri":"https://www.uniprot.org/uniprotkb/"}],"attributes":[{"subj":"30254638-29181134-35421108","pred":"source","obj":"MyTest"},{"subj":"30254638-24966707-35421109","pred":"source","obj":"MyTest"},{"subj":"30254638-17149063-35421110","pred":"source","obj":"MyTest"},{"subj":"30254638-17869550-35421111","pred":"source","obj":"MyTest"},{"subj":"30254638-22738174-35421112","pred":"source","obj":"MyTest"},{"subj":"30254638-22738174-35421113","pred":"source","obj":"MyTest"}]},{"project":"2_test","denotations":[{"id":"30254638-29181134-35421108","span":{"begin":210,"end":213},"obj":"29181134"},{"id":"30254638-24966707-35421109","span":{"begin":421,"end":424},"obj":"24966707"},{"id":"30254638-17149063-35421110","span":{"begin":743,"end":746},"obj":"17149063"},{"id":"30254638-17869550-35421111","span":{"begin":862,"end":864},"obj":"17869550"},{"id":"30254638-22738174-35421112","span":{"begin":866,"end":868},"obj":"22738174"},{"id":"30254638-22738174-35421113","span":{"begin":2143,"end":2145},"obj":"22738174"}],"attributes":[{"subj":"30254638-29181134-35421108","pred":"source","obj":"2_test"},{"subj":"30254638-24966707-35421109","pred":"source","obj":"2_test"},{"subj":"30254638-17149063-35421110","pred":"source","obj":"2_test"},{"subj":"30254638-17869550-35421111","pred":"source","obj":"2_test"},{"subj":"30254638-22738174-35421112","pred":"source","obj":"2_test"},{"subj":"30254638-22738174-35421113","pred":"source","obj":"2_test"}]}],"config":{"attribute types":[{"pred":"source","value type":"selection","values":[{"id":"MyTest","color":"#93ecb9","default":true},{"id":"2_test","color":"#ec9f93"}]}]}}