A Case of Colon Cancer-Induced Hemophagocytic Lymphohistiocytosis with Lymphangitic Carcinomatosis.

INTRODUCTION

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome involving extreme hyperinflammation driven by a cytokine storm, which leads to the pathological autophagocytosis of blood cells. Although secondary HLH is frequently associated with underlying malignancies, the majority of which are hematological, cases triggered by colorectal cancer are quite rare. We report herein a case of HLH induced by ascending colon cancer.

CASE PRESENTATION: An 83-year-old female patient presented to her primary care physician with a 1-month history of anorexia, persistent fever (>38°C), and cognitive decline. Colonoscopy revealed a tumor at the hepatic flexure of the ascending colon, which was identified as a well-differentiated adenocarcinoma on the basis of a biopsy specimen analysis. Ascending colon cancer (cT3N2bM0, cStage IIIc) was finally diagnosed. As the fever was presumed to be a paraneoplastic manifestation, the patient underwent robotic-assisted right hemicolectomy with lymphadenectomy. Recurrences of the fever, persistent anorexia, and generalized edema were observed postoperatively. On POD 25, peripheral blood smears revealed macrophage-mediated hemophagocytosis, confirming the diagnosis of HLH based on the HLH-2004 diagnostic criteria. Despite an escalation in corticosteroid therapy, the patient remained refractory to treatment and died on POD 46.

CONCLUSIONS

Clinicians should include cancer-induced HLH in the differential diagnosis of fever and bicytopenia in the presence of an advanced solid tumor.