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Mentions: Fever is associated with multitude etiologies and its diagnosis often requires number of investigations. Of interest, are certain rare entities like Hemophagocytic Lymphohistiocytosis (HLH). Seventeen year female patient presented with fever and chills since a week. She was the first child of a consanguineous marriage. Physical examination revealed pallor, icterus, hepato-splenomegaly, cervical lymphadenopathy and white hair with silvery sheen on scalp, eyebrows and eyelashes [Figure 1]. The routine investigations suggested pancytopenia with Hemoglobin-5.8 gm/dl, White cells-2,180/μL, Platelets-33,0000/μL. Peripheral smear showed no parasites. Serological tests for dengue, typhoid and leptospirosis turned negative. Serum biochemical parameters showed bilirubin-3.3 mg/dl, direct bilirubin-0.3 mg/dl, Alkaline phosphatase-276U/L, Triglycerides-164 mg/dl and LDH-2250U/L. No bacterial or mycobacterial growth was seen on blood culture. Aspiration smears of cervical lymph nodes showed features of reactive lymphadenitis. The patient was referred for bone marrow evaluation. Marrow smears showed hemophagocytosis [Figures 2a–d]. No pathogens were identified on smears. Review of all clinical and laboratory data suggested the diagnosis of Griscelli syndrome (GS) type 2.
Griscelli Syndrome: Hemophagocytic Lymphohistiocytosis with Silvery Hair
Affiliation: Department of Pathology, P E S Institute of Medical Sciences and Research, Kuppam, India.