A Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma

TL;DR

This paper presents a case of a 39-year-old man with T-cell lymphoma who developed a rare immune condition called hemophagocytic lymphohistiocytosis (HLH), highlighting the importance of early diagnosis and treatment.

Contribution

The paper adds to the understanding of secondary HLH in the context of T-cell lymphoma through a detailed clinical case report.

Findings

The patient met HLH-2004 diagnostic criteria but showed no hemophagocytosis in blood or bone marrow.

The patient had a history of autoimmune disorders and was treated with dexamethasone and etoposide, but the outcome was fatal.

The case emphasizes the need for early recognition of secondary HLH in patients with T-cell lymphoma.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition that results from excessive immune activation and inflammation. This condition may be triggered by various factors, including infections, malignancies, or autoimmune diseases. Here, we report the case of a 39-year-old male who developed HLH secondary to T-cell lymphoma and had a history of multiple autoimmune disorders.

Our patient presented with shortness of breath and weakness which led to an admission for methicillin-resistant Staphylococcus aureus bacteremia. His hospital course deteriorated rapidly due to his worsening condition. He was confirmed to have HLH based on the HLH-2004 criteria with the presence of fever, splenomegaly, hypertriglyceridemia, hypofibrinogenemia, low natural killer cell function, high ferritin, and soluble interleukin 2 receptor levels. Peripheral blood smear and bone marrow biopsy showed atypical lymphocytes consistent with a T-cell lymphoma, but no hemophagocytosis. He was treated with dexamethasone and etoposide. Despite treatment, the patient passed away.

This case aims to contribute further to the understanding of secondary HLH in the setting of T-cell lymphoma. It also illuminates how vital early recognition and treatment are in patients with secondary HLH.

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