An Unusual Case of Ovarian Dysgerminoma Associated with Secondary Hemophagocytic Lymphohistiocytosis (HLH)

Hemophagocytic Lymphohistiocytosis (HLH) is rare fulminant disease with high mortality. High clinical suspicion is required to diagnose HLH. Malignancy can cause secondary HLH. We reported a case of 45 years old lady with hypertension, presented with bilateral loin pain. On presentation to hospital, she was clinically ill, dehydrated and laboratory parameters showed severe acute kidney injury. CECT abdomen showed huge pelvic mass. Throughout ward admission, she had period of unexplained fever and pancytopenia hence diagnosis of HLH was considered. She fulfilled criteria of HLH and decision was made to remove the mass. Her blood counts recovered after ovarian tumour removed which subsequently HPE came back as ovarian dysgerminoma. However, she succumbed due to invasive fungal infection and upper gastro intestinal bleeding as a result of prolonged period of immunosuppression. HLH is uncommon and likely underdiagnosed disease. Diagnosis of HLH is often challenging as disease manifestation is non-specific, hence early recognition and intervention will improve patient outcome and survival.