Hemophagocytic Lymphohistiocytosis Complicating Systemic Sarcoidosis

Omar Abughanimeh,Mouhanna Abu Ghanimeh,Ayman Qasrawi

doi:10.7759/cureus.2838

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by histiocyte proliferation and hemophagocytosis. Primary HLH is caused by genetic defects, whereas secondary HLH occurs in the setting of underlying diseases, such as infections, malignancies, or rheumatic diseases. Rheumatic diseases, such as systemic juvenile arthritis or systemic lupus erythematosus, have been associated with HLH. However, the association between sarcoidosis and HLH has been rarely reported in the literature. Herein, we report a case of a 36-year-old female who was recently diagnosed with sarcoidosis, and she developed fatal HLH that was not responsive to high-dose steroids.

Highlights

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome, which results from histiocyte proliferation and extensive hemophagocytosis as a result of a hyperinflammatory response [1]
HLH can be classified as primary HLH, which is caused by genetic defects, or secondary HLH, which is caused by infections, malignancies, or rheumatic diseases [2]
HLH can be classified according to the cause as primary HLH, which is due to genetic mutations, or secondary HLH, which is due to infections, autoimmune diseases, or malignancies [1]

Summary

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome, which results from histiocyte proliferation and extensive hemophagocytosis as a result of a hyperinflammatory response [1]. HLH can be classified as primary HLH, which is caused by genetic defects, or secondary HLH, which is caused by infections, malignancies, or rheumatic diseases [2] Both primary and secondary HLH exhibit the same presentation of fever, hepatosplenomegaly, and pancytopenia [1,2]. Macrophage activation syndrome (MAS) is a term used to describe HLH that results from rheumatic or systemic diseases [3]. The axillary lymph node biopsy (Figure 1) revealed noncaseating granulomas typical for sarcoidosis. A bone marrow biopsy (Figure 2) demonstrated hypocellular marrow with normal hematopoiesis replaced by proliferation of histiocytes and vague granulomas. The patient did not respond to a trial of intravenous immunoglobulin (IVIG) and 1 g/d methylprednisolone She developed encephalopathy and status epilepticus and was transferred to another facility for escalated care.

Discussion

Conclusions

Disclosures

Findings

Larroche C

10. Athreya BH