A scopic review on the diagnostic dilemmas and newer treatment
modalities in Hemophagocytic Lymphohistiocytosis
Abstract
Abstract: Hemophagocytic lymphohistiocytosis is a rare and fatal
systemic illness arising secondary to an immune dysregulation .Primary
HLH is due to genetic defects and secondary HLH is caused due to
unchecked macrophage recruitment following an acquired trigger. It is
often diagnosed late in view of its rarity and similarities of
presentation to sepsis and SIRS. A compelete curative solution to this
problem is hematopoietic stem cell transplant, though secondary cases
are often seen to have sustained remission with immune-chemotherapy
COVID 19 has also been postulated to cause a HLH like scenario with
blunted NK cell number and function. Recent advances in this field
comprising of various immunosuppressant based regimens, myeloablative
therapies preceding stem cell transplant and improved techniques of stem
cell transplant have improved the outcomes. Here we try to present the
pathogenesis, etiology, diagnostic criteria and the dilemmas, various
treatment strategies, prognostic markers and the most recent researches
regarding this rare disease.