Hemophagocytic lymphohistiocytosis: tortuous experience in the diagnosis and treatment of one lymphoma / 中华危重病急救医学

ABSTRACT

The etiology of hemophagocytic lymphohistiocytosis (HLH) is complicated and difficult to diagnose, unexplained HLH often with hematological malignancies. Invasive biopsy can help to find etiology, the results may be affected by the technique and the location of the puncture site. Multiangle puncture can improve the success rate, but the corresponding risk increases. A patient with HLH was admitted to Affiliated Hospital of Zunyi Medical College. The etiology was unknown. Active symptomatic support treatment was conducted, at the same time, finding the evidence of viral infection, autoimmune disease related detection, blood culture, bone marrow puncture smear and spleen biopsy were performed respectively to find the pathogen basis. Spleen hemorrhage was not being controlled after spleen biopsy in patients, and emergency splenectomy was adopted to stop bleeding for saving lives. Finally, the patients died of low protein, pulmonary edema and respiratory failure. The bone marrow puncture and spleen biopsy failed to provide the basis for tumor invasion, while the spleen pathological slices plus immunohistochemical indicate diffuse large B cell lymphoma (DLBCL) after splenectomy, which was identified as malignant tumor-associated hemophagocytic syndrome. Underscoring the high risk of bleeding after tumor-associated splenomegaly puncture and the importance of having emergency plans. Through analyzing the clinical characteristics, diagnosis and treatment of this patient, we hope to improve the clinicians' understanding of HLH and lymphoma.