Hemophagocytic lymphohistiocytosis: Overview and diagnostic procedure. A case induced by an expansion of monoclonal EBV-negative NK cells

Griscelli Syndrome: Hemophagocytic Lymphohistiocytosis with Silvery Hair

“…Secondary HLH is seen associated with wide variety of diseases such as viral infection, lymphomas, solid organ malignancies and autoimmune disorders. [12] Genetic disorders like Familial HLH, Chediak Higashi syndrome, X linked lymphoproliferative disease and GS type 2 underlie primary HLH. [2] Primary HLH are usually diagnosed in young, rarely they are recognized at later age as in our case.…”

“…[23] Differential diagnosis include other primary HLH. Giant cytoplasmic granules in leucocytes are evident in Chediak Higashi syndrome; while X linked lymphoproliferative disease and Familial HLH do not exhibit albinism.…”