Title : Hemophagocitic lymphocitosis, first presentation of hodgin lympoma - Case report and literature review
Abstract:
Hemophagocytic lymphohistiocytosis (HLH) is described as a hyperferritinemic? ? hyperin-inflammatory syndrome, in the pathogenesis of which ,an important role is played by the uncontrolled activation of cells with an important role in the body's immune mechanisms, such as macrophages, lymphocytes, histiocytes, TNF-α, interleukin 6 (IL-6), interferon γ (IFN-γ), and MIP-1α [1].
Clinically, this pathology is characterized by fever, cytopenia, splenomegaly, neurological symptoms, invasion of the bone marrow, liver, or lymph nodes.[2]. The pyramid population showed a bimodal population distribution with peaks in interval 16-30 years of age, and 56-70 years of age. The most frequent associated conditions were malignancy (4953 admissions [30.7%]), infections (3913 admissions [24.3%]), autoimmune conditions (3362 admissions [20.8%]), patients who had been having an organ transplantation (639 admissions [4%]), and congenital immunodeficiency syndromes (399 admissions [2.5%]). Congenital immunodeficiency syndromes had the highest inrahospital mortality rate, (mortality rate 31.1%, adjusted OR 2.36 [1.56-3.59]), followed by malignancies (mortality rate 28.4%, adjusted OR 1.80 [1.46-2.22]), infections (mortality rate 21.4%, adjusted OR 1.33 [1.10-1.62]), other/no trigger (mortality rate 13.6%, adjusted OR 0.73 [0.58-0.92]), autoimmune (mortality rate 13%, adjusted OR 0.72 [0.57-0.92]), and post-transplant (mortality rate 14.1%, adjusted OR 0.64 [0.43-0.97]).
