Acute Liver Failure in Hemophagocytic Lymphohistiocytosis Secondary to Metastatic Renal Cell Carcinoma: A Diagnostic Dilemma

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive hematologic disorder involving hyperstimulation of immune responses and severe inflammation. HLH has been well documented in lymphoid cancers and leukemias, but more rarely in solid tumors. The non-specific clinical characteristics of HLH can cause a diagnostic dilemma and delay in proper treatment, resulting in poor outcomes. We present a case of a patient with metastatic renal cell carcinoma who developed unexplained acute liver failure and was later found to have HLH. This case highlights the importance of including this syndrome on the differential diagnosis for acute liver failure of indeterminate cause and cytopenia in the setting of malignancy to facilitate proper timely treatment to improve outcomes and increase odds of survival.

Keywords: cytopenia; hemophagocytic lymphohistiocytosis; liver failure; malignancy; renal cell carcinoma.

Figure 1. MRI of the liver with contrast

Markedly enlarged liver with abnormal T2 signal (blue arrow) and heterogeneous enhancement with splenomegaly (orange arrow).

Figure 2. Bone marrow aspirate

A large foamy macrophage engulfing numerous erythroid precursors seen within a bone marrow aspirate smear (Wright-Giemsa stain, original magnification x1000).