Lymphoma associated hemophagocytic syndrome: A single‑center retrospective study

Abstract

To improve the understanding of lymphoma associated hemophagocytic syndrome (LAHS) and find an effective treatment for this fatal disease, 57 patients with LAHS were retrospectively reviewed. The most common histopathological type was extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL) (45.61%). Patients with B-cell LAHS were significantly older (P<0.001), and exhibited a higher triglyceride level (P=0.012), lower serum ferritin level (P=0.014) and lower plasma Epstein-Barr virus DNA (P<0.001) compared with patients with T/NK-cell LAHS. The median survival time of all patients was 43 days, and patients with B-cell (n=14) and T/NK-cell (n=43) LAHS had a median survival time of 55 and 40 days, respectively (P=0.797). Compared with patients who were treated based on HLH-2004 protocols combined with multidrug chemotherapy, those without chemotherapy had a reduced prognosis (P=0.002). The patients that underwent hematopoietic stem cell transplantation (HSCT) following chemotherapy had a significantly improved overall survival (OS) compared with patients that did not undergo HSCT (P=0.001). Patients with B-cell LAHS treated with rituximab (P=0.015) and patients with ENKL treated with L-asparaginase/pegaspargase (L-asp/peg) (P=0.009) had an improved prognosis compared with patients not treated with these drugs. In the T/NK-cell LAHS group, patients treated with chemotherapy containing gemcitabine did not exhibit an improved OS compared with those not treated with gemcitabine (P=0.326). Furthermore, multivariate analysis demonstrated that long diagnosis time and poor performance status were independent prognosis factors for all patients with LAHS. The present study indicated that survival time does not differ between patients with B-cell LAHS and patients with T/NK-cell LAHS. Early diagnosis and appropriate immunochemotherapy plus HSCT are essential to achieve improved outcomes. The outcome of patients with B-cell LAHS may be significantly improved following treatment with rituximab. L-asp/peg-containing regimens are promising treatments for patients with NK/T-cell LAHS.

Malignancy-associated hemophagocytic lymphohistiocytosis in children: a 10-year experience of a single pediatric hematology center

“…Nearly half of our M-HLH patients had EBV infection with a high plasma load of EBV-DNA. In some studies, the EBV-DNA load was higher in patients with T/NK-cell M-HLH compared to those with B-cell M-HLH; however, it did not affect the OS in M-HLH patients [26,27]. We compared the clinical manifestations and laboratory parameters of EBV positive and negative M-HLH children.…”

“…Until now there is still no standard treatment for M-HLH and most clinicians select the HLH-94/04 regimen for initial treatment. Other disease-specific chemotherapies, like CHOP, SMILE, and ALL/AML BFM can be used for malignancy-trigged patients [9,15,26,29]. However, Ch-HLH could be controlled with steroids, IVIG treatment and antimicrobial therapy.…”

Hemophagocytic lymphohistiocytosis with advanced malignant melanoma accompanied by ipilimumab and nivolumab: A case report and literature review

“…Most cases of MAHS are lymphoma‐associated HPS, and the first case was reported in 1983 4 . All of T‐cell lymphoma, natural killer (NK)‐cell lymphoma, and B‐cell lymphoma could cause lymphoma‐associated HPS, and extranodal NK/T‐cell lymphoma, nasal type, is reported as the most common cause, accounting for 45% of all the cases 5 . More than half of the cases of AAHS occur secondary to systemic lupus erythematosus or adult‐onset Still's disease, and in many cases, the AAHS manifests when primary disease activity is high.…”

Effect of gga-miR-155 on Cell Proliferation, Apoptosis and invasion of Marek’s disease Virus (MDV) transformed cell line MSB1 by targeting RORA

“…It is also a tumor suppressor and therefore inactivated during malignant transformation, and tumor initiation and metastasis [47,48] . Several targets of miRNA-155 have been identified in recent years, such as the BRG1 [49] and FOXO3a [50] in lymphoma, and SOCS1 in severe acute pancreatitis [51] . Parnas et al predicted 9 common target genes of MDV1-miR-M4, KSHV-miR-K11 and HAS-miR-155, and 4 of MDV1-miR-M4 and HAS-miR-155 in human B cells and chicken T cells, which includes RORA [32] .…”