Beyond the heart: hidden lymphoma-induced HLH driving cardiogenic shock

Background

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory condition triggered by factors like genetic abnormalities, malignancies, and infections. It leads to uncontrolled macrophage activity and cytokine release, causing tissue damage and multi-organ failure. Prompt recognition and diagnosis are essential due to its nonspecific presentation and high mortality.

Case

A 76-year-old male with known atrial fibrillation, presented with progressive fatigue. The patient was being treated with steroids and methotrexate (MTX) for a recent episode of sudden onset blurry vision while further evaluation of suspected temporal arteritis was being pursued. Upon admission, the patient developed cardiogenic shock, respiratory failure, and mental status changes, requiring intensive care. Lab findings showed thrombocytopenia, elevated ferritin, high triglycerides, and pancytopenia, fulfilling 5 of the 8 HLH diagnostic criteria. A bone marrow biopsy confirmed B-cell non-Hodgkin’s lymphoma, leading to a diagnosis of lymphoma-induced HLH. The patient was treated with etoposide and later transitioned to R-CHOP chemotherapy, leading to significant clinical improvement and stabilization of HLH markers.

Discussion:

This case illustrates the diagnostic complexity of HLH. Early suspicion, diagnosis, and treatment are crucial to improving outcomes in patients with multi-organ dysfunction and nonspecific systemic symptoms.