Hemophagocytic lymphohistiocytosis in pregnancy: a case report and review of the literature.

Hemophagocytic lymphohistiocytosis (HLH) during pregnancy is rare and seldom reported in literature. Here we report a case of pregnancy-associated HLH. A patient was admitted at 26 weeks and 2 days pregnancy complaining of cough and fever was admitted. She was found having bi-cytopenia, elevated ferritin and hypertriglyceridaemia. HLH was not confirmed because of the negative results from the first bone marrow biopsy. As the situation worsened, a timely termination of pregnancy was carried out. The second bone marrow biopsy did reveal hemophagocytosis and then pregnant-related HLH was confirmed. Prompt treatment of dexamethasone and etoposide have the puerpernat in a better situation. From this case, we learned HLH can be likely ignored and not be diagnosed promptly in a pregnant patient. In order to confirm this disease, a bone marrow biopsy should be performed promptly or repeatly for suspicious HLH. The time of termination and treatment is both essential for the foetus and the puerperant. Given the high morbidity and mortality of this disease in recent years, an expert consensus or clinical guideline should be developed.