Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored.
Hye-Ji HanKyung Taek HongHyun Jin ParkBo Kyung KimHong Yul AnJung Yoon ChoiHyoung Jin KangPublished in: Children (Basel, Switzerland) (2021)
It is complicated to establish a consensus on the management and diagnosis of malignancy-triggered hemophagocytic lymphohistiocytosis (M-HLH) in children, as an initial presentation of malignancy is complicated. In this paper, we analyze the clinical characteristics and outcomes of eight pediatric patients in which M-HLH was the initial presentation of malignancy. All patients had hematologic malignancies: three subcutaneous panniculitis-like T-cell lymphomas, two acute lymphoblastic leukemias, two anaplastic large cell lymphomas, and a systemic EBV + T-cell lymphoma of childhood. The incidence rate of M-HLH among leukemia and malignant lymphoma patients in our institution was 1.9%. From the initial diagnosis of HLH, the median time taken to be diagnosed as a malignancy was about 1.3 months. The majority of patients received HLH-targeted immunosuppression and/or etoposide at first. The patients' clinical response to treatment for HLH and malignancies were varied. Five out of the eight patients died, one of whom died due to HLH-related cerebral edema after the initiation of chemotherapy. The median overall survival was 1.6 years. In order to improve the survival rate, the early detection of M-HLH, rapid screening for malignancy, and complete control of M-HLH with HLH-directed therapy followed by a thorough response monitoring are required.
Keyphrases
- end stage renal disease
- prognostic factors
- peritoneal dialysis
- stem cells
- squamous cell carcinoma
- young adults
- type diabetes
- radiation therapy
- subarachnoid hemorrhage
- hepatitis b virus
- diffuse large b cell lymphoma
- case report
- cancer therapy
- blood brain barrier
- smoking cessation
- locally advanced
- combination therapy
- early life