A Case Report and Literature Review of Hemophagocytic Syndrome Secondary to Garcin-Guillain Syndrome.

Abstract

Background: This article reports on a case of hemophagocytic syndrome (HPS) secondary to multiple myeloma (MM) with extramedullary lesions presenting as the initial symptom. MM with extramedullary lesions, also referred to as Garcin-Guillain syndrome, is extremely rare, occurring in only 4.6% of MM patients. Secondary HPS is uncommon in malignant tumors and is particularly rare in MM.

Methods: The diagnosis was based on the patient's clinical presentation and diagnostic findings, including blood tests, ferritin levels, head MRI, pathological analysis, and bone marrow biopsy. The patient was diagnosed with MM complicated by Garcin-Guillain syndrome, with HPS secondary to recurrent MM.

Results: The patient was hospitalized in September 2020 due to swallowing difficulty, hoarseness, choking while eating for over 10 days, and rightward deviation of the mouth for five days. These symptoms were attributed to the direct spread of MM lesions to the lingual pharyngeal and vagus nerves. PAD chemotherapy (bortezomib, doxorubicin, and dexamethasone) was initiated. After one cycle, the symptoms of Garcin-Guillain syndrome resolved, and partial remission (PR) was achieved after five cycles. In October 2021, the patient was readmitted with recurrent high fever. Clinical findings and examinations met the diagnostic criteria of HLH-2004, confirming secondary HPS due to MM relapse. Treatment with bortezomib, dexamethasone, etoposide, and lenalidomide showed limited efficacy, and the patient subsequently died.

Conclusions: HPS secondary to MM recurrence is extremely rare, characterized by severe disease, rapid progression, poor treatment response, and poor prognosis with a short survival time. Despite advances in drug therapies, there is no established treatment protocol for such cases. Further research is needed to improve the outcomes of these patients.