• diagnosis & prognosis
• hemophagocytic lymphohistiocytosis
• liver transplant
• metagenomic next-generation sequencing
• toxoplasmosis

• Hemophagocytic lymphohistiocytosis
• Solid organ transplantation

• Female
• Humans
• Young Adult
• Adult
• Cytokines
• Lymphohistiocytosis, Hemophagocytic/diagnosis
• Lymphohistiocytosis, Hemophagocytic/etiology
• Interleukin-18
• Liver Transplantation/adverse effects
• Hepatitis, Autoimmune/complications
• Hepatitis, Autoimmune/diagnosis
• Macrophage Activation
• Cytokine Release Syndrome
• Neopterin
• Macrophage Activation Syndrome/diagnosis
• Macrophage Activation Syndrome/etiology
• Macrophage Activation Syndrome/therapy

• hemophagocytic lymphohistiocytosis
• liver transplantation
• ruxolitinib

Liver hepatocellular carcinoma (LIHC) is the predominant type of liver cancer, and its treatment still faces great challenges presently. Mitochondrial inner membrane protein MPV17 is reported to be involved in multiple biological activities of cancers. Here, we seek to investigate the specific role and functions of MPV17 in LIHC progression.

Firstly, MPV17 expressions in various tumors and corresponding normal samples and LIHC groups with various clinical features were analyzed, respectively. Next, the relationship between MPV17 expression and LIHC survival was analyzed and verified by AUC curves. Besides, differentially expressed genes (DEGs) for LIHC were screened from TCGA and then analyzed by GO and KEGG. Then, MPV17 was analyzed by prognostic model, Cox analysis, predictive nomogram, pathway correlation, and immunoassay. Finally, the functions of MPV17 were determined by CCK-8 and Tranwell assays.

In most tumors, MPV17 expression was higher than that in the normal group, and it was related to LIHC clinical features. In the LIHC survival analysis, highly expressed MPV17 was associated with a poor prognosis. Besides, 314 upregulated and 193 downregulated DEGs are mainly involved in the TNF signaling pathway and tyrosine metabolism. Through prognostic model, Cox analysis, and predictive nomogram, MPV17 had the prognostic value for LIHC. Gene-pathway correlation analysis showed that MPV17 had the strongest correlation with the G2M_checkpoint pathway. In an immunoassay, MPV17 had a strong correlation with many immune cells. Functional assays showed that MPV17 reduction in LIHC cells could inhibit cell invasion, migration, and proliferation.

MPV17, as a tumor promoter, could be a new biomarker for LIHC diagnosis and prognosis and probably shed new light on the exploration of LIHC therapies.

• donor-transmitted toxoplasmosis
• hemophagocytic syndrome
• transplant complications

• complications of liver transplantation
• hemophagocytic lymphohistiocytosis
• human herpesvirus-8
• liver transplantation
• post-transplant lymphoproliferative disorder

• autoimmune hemolytic anemia
• hemophagocytic lymphohistiocytosis (HLH)
• living donor liver transplant
• pediatric liver transplantation
• solid organ transplant

Hemophagocytic syndrome (HPS) is a rare and potentially fatal complication following liver transplantation.

A 63-year-old woman with decompensated liver cirrhosis secondary to hepatitis B virus infection underwent living donor liver transplantation using the right posterior section of her husband’s liver (graft volume, 581 g; 56.8% of the recipient’s standard liver volume). She developed small-for-size syndrome on postoperative day (POD) 7, and HPS was diagnosed on POD 12 by bone marrow aspiration (white blood cells, 300/μL; neutrophils, 30/μL). Given that she tested negative for viral (hepatitis B virus and cytomegalovirus) and bacterial infections, it was considered likely to be secondary HPS. Steroid pulse therapy was initiated, and her white blood cell count increased to 4290/μL on POD 15, indicating that her peripheral blood leukocytes had improved. There were no surgical complications, but the patient died of prolonged graft dysfunction with bacterial sepsis on POD 14.

We report a rare case of HPS occurring 2 weeks after living donor liver transplantation with a right posterior section graft, diagnosed early via bone marrow aspiration. This clinical course implies an association between HPS and graft dysfunction such as small-for-size syndrome. Further studies of the mechanism of hypercytokinemia-induced HPS are required to confirm the optimal treatment for HPS.

• Hemophagocytic syndrome
• Liver transplantation
• Small-for-size syndrome

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome that develops as a primary (familial/hereditary) or secondary (non-familial/hereditary) disease characterized in the majority of the cases by hereditary or acquired impaired cytotoxic T-cell (CTL) and natural killer responses. The molecular mechanisms underlying impaired immune homeostasis have been clarified, particularly for primary diseases. Familial HLH (familial hemophagocytic lymphohistiocytosis type 2-5, Chediak-Higashi syndrome, Griscelli syndrome type 2, Hermansky-Pudlak syndrome type 2) develops due to a defect in lytic granule exocytosis, impairment of (signaling lymphocytic activation molecule)-associated protein, which plays a key role in CTL activity [e.g., X-linked lymphoproliferative syndrome (XLP) 1], or impairment of X-linked inhibitor of apoptosis, a potent regulator of lymphocyte homeostasis (e.g., XLP2). The development of primary HLH is often triggered by infections, but not in all. Secondary HLH develops in association with infection, autoimmune diseases/rheumatological conditions and malignancy. The molecular mechanisms involved in secondary HLH cases remain unknown and the pathophysiology is not the same as primary HLH. For either primary or secondary HLH cases, immunosuppressive therapy should be given to control the hypercytokinemia with steroids, cyclosporine A, or intravenous immune globulin, and if primary HLH is diagnosed, immunochemotherapy with a regimen containing etoposide or anti-thymocyte globulin should be started. Thereafter, allogeneic hematopoietic stem-cell transplantation is recommended for primary HLH or secondary refractory disease (especially EBV-HLH).

• Alemtuzumab
• Anti-thymocyte globulin
• Cyclosporine A
• Epstein-Barr virus
• Etoposide
• Hematopoietic stem-cell transplantation
• Hemophagocytic lymphohistiocytosis
• Hereditary diseases
• Immunochemotherapy
• Intravenous immunoglobulin
• Molecular diagnosis
• Rituximab
• Steroids