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      Hemophagocytic Lymphohistiocytosis

      1 , 2
      Annual Review of Pathology: Mechanisms of Disease
      Annual Reviews

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          Serum ferritin is derived primarily from macrophages through a nonclassical secretory pathway.

          The serum ferritin concentration is a clinical parameter measured widely for the differential diagnosis of anemia. Its levels increase with elevations of tissue iron stores and with inflammation, but studies on cellular sources of serum ferritin as well as its subunit composition, degree of iron loading and glycosylation have given rise to conflicting results. To gain further understanding of serum ferritin, we have used traditional and modern methodologies to characterize mouse serum ferritin. We find that both splenic macrophages and proximal tubule cells of the kidney are possible cellular sources for serum ferritin and that serum ferritin is secreted by cells rather than being the product of a cytosolic leak from damaged cells. Mouse serum ferritin is composed mostly of L-subunits, whereas it contains few H-subunits and iron content is low. L-subunits of serum ferritin are frequently truncated at the C-terminus, giving rise to a characteristic 17-kD band that has been previously observed in lysosomal ferritin. Taken together with the fact that mouse serum ferritin is not detectably glycosylated, we propose that mouse serum ferritin is secreted through the nonclassical lysosomal secretory pathway.
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            Reactive hemophagocytic syndrome in adults: a retrospective analysis of 162 patients.

            Current knowledge in reactive hemophagocytic syndrome mainly relies on single-center case series including a relatively small number of patients. We aimed to identify a multicenter large cohort of adult patients with reactive hemophagocytic syndrome and to describe relevant clinical and laboratory features, underlying conditions, and outcome.
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              How I treat hemophagocytic lymphohistiocytosis in the adult patient.

              Hemophagocytic lymphohistiocytosis (HLH) is a devastating disorder of uncontrolled immune activation characterized by clinical and laboratory evidence of extreme inflammation. This syndrome can be caused by genetic mutations affecting cytotoxic function (familial HLH) or be secondary to infectious, rheumatologic, malignant, or metabolic conditions (acquired HLH). Prompt recognition is paramount and, without early treatment, this disorder is frequently fatal. Although HLH is well described in the pediatric population, less is known about the appropriate work-up and treatment in adults. Here, we review the clinical characteristics, diagnosis, and treatment of HLH in adults.
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                Author and article information

                Journal
                Annual Review of Pathology: Mechanisms of Disease
                Annu. Rev. Pathol. Mech. Dis.
                Annual Reviews
                1553-4006
                1553-4014
                January 24 2018
                January 24 2018
                : 13
                : 1
                : 27-49
                Affiliations
                [1 ]Massachusetts General Hospital, Boston, Massachusetts 02114;
                [2 ]Brigham & Women's Hospital, Boston, Massachusetts 02115;
                Article
                10.1146/annurev-pathol-020117-043625
                28934563
                09d9e86a-d744-484e-bc2d-6cbd6faa837e
                © 2018
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