Open AccessMalignancy-induced hemophagocytic lymphohistiocytosis in a case of signet ring cell carcinoma of the stomach
Author(s) -
S. Ramkumar
Publication year - 2021
Publication title -
indian journal of pathology and microbiology/indian journal of pathology and microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.217
H-Index - 31
eISSN - 0974-5130
pISSN - 0377-4929
DOI - 10.4103/ijpm.ijpm_896_19
Subject(s) - malignancy , hemophagocytic lymphohistiocytosis , perforin , stomach , medicine , signet ring cell carcinoma , pathology , incidence (geometry) , immunology , gastroenterology , cancer , immune system , disease , cd8 , adenocarcinoma , physics , optics
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially life-threatening condition associated with high mortality and can be classified into primary (familial) and secondary HLH. Primary HLH is an inherited, autosomal recessive disorder associated with defects in perforin function, whereas secondary HLH is associated with infections, especially Epstein-Barr virus infection, malignancies, and autoimmune disorders. Patients with malignancy-associated secondary HLH experience symptoms that overlap with those described for other HLH types, which is associated with an increased incidence of misdiagnosis and mortality. Here, we report the case of a patient with secondary HLH associated with a solid malignancy (signet-ring cell carcinoma of the stomach).