Patologie rară cu debut atipic şi abordare multidisciplinară

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome developed due to uncontrolled hyper­in­flam­ma­tion and tissue destruction. In most cases, it is a pediatric disorder, but it can also occur in adults. The cli­ni­cal presentation and the laboratory findings are often non­spe­ci­fic, therefore it is difficult to diagnose. Yet, an early diagnosis and treatment can significantly improve sur­vival. Hence, the treatment should be initiated as soon as possible if one has a high suspicion of HLH, even though the patient does not meet yet all the criteria. We pre­sent the case of a patient admitted with nonspecific symp­to­ma­to­lo­gy, namely jaundice, fatigue and significant non-intentional weight loss. The initial laboratory data sug­gested cirrhosis. The attempt of discovering the cause of hepatic dysfunction was initially unsuccessful. We sus­pi­cioned HLH only based on the myelogram. At that point, the patient met the criteria for the diagnosis of HLH, and we star­ted the specific treatment. However, the results were not sa­tis­fac­to­ry, and the patient died three months after the ini­tial presentation.