Ruxolitinib as first-line therapy in secondary hemophagocytic lymphohistiocytosis and HIV infection
- PDF / 967,221 Bytes
- 4 Pages / 595.276 x 790.866 pts Page_size
- 66 Downloads / 212 Views
CASE REPORT
Ruxolitinib as first‑line therapy in secondary hemophagocytic lymphohistiocytosis and HIV infection Sergio Gálvez Acosta1 · Mariana Javalera Rincón2 Received: 6 February 2020 / Revised: 1 April 2020 / Accepted: 3 April 2020 © Japanese Society of Hematology 2020
Abstract Secondary lymphohistiocytosis is a severe inflammatory condition characterized by uncontrolled inflammatory response hyperactivation of antigen-presenting cells, hemophagocytosis, cytopenias, and multiorgan failure, and is secondary to subjacent pathologies such as cancer, infection, or immune disease. Standard treatment includes chemotherapy; however, this is not always possible or safe. Ruxolitinib, a Janus kinase (JAK) 1/2 inhibitor can reduce inflammation and cytokine activity and has been used in refractory cases and first-line treatment. Here, we present a 35-year-old patient with HIV infection and secondary lymphohistiocytosis due to CMV/EBV infection who was successfully treated with ruxolitinib as first-line therapy. He rapidly showed clinical and biochemical improvement and transfusion independence without complications. Keywords Secondary hemophagocytic lymphohistiocytosis · Ruxolitinib · HIV infection · CMV/EBV infection
Background Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe and potentially fatal condition without treatment entity, characterized by an uncontrolled inflammatory response, hyperactivation of antigen presenting cells, hemophagocytosis, cytopenias, and multiorgan failure [1]. HLH can be primary, due to genetic mutations or secondary (sHLH) to subjacent pathologies like cancer, infection or immune diseases [2]. Treatment options include etoposide, cyclosporine, and dexamethasone; however, many patients have refractoriness or high toxicities due to chemotherapy. Since 2017 ruxolitinib, a Janus kinase (JAK) 1 and 2 inhibitor, has been used in refractory sHLH to multiple lines of treatments [2–5] and afterwards in first-line for two separated patients with sHLH Sergio Gálvez Acosta, Mariana Javalera Rincón equal contribution. * Sergio Gálvez Acosta [email protected] 1
Hospital General de Tijuana, Secretaría de Salud de Baja California, Avenida Centenario 10851, Zona Río, CP 22680 Tijuana, Baja California, Mexico
Hospital General Regional 20, Instituto Mexicano del Seguro Social, Boulevard Díaz Ordaz y Lázaro Cárdenas, La Meza, CP 22450 Tijuana, Baja California, Mexico
2
in context of rheumatoid arthritis and histoplasmosis [1, 6]. It has never been used as front-line therapy for patients with human immunodeficiency virus (HIV) infection. Here, we present the case of a patient with HIV infection and sHLH due to cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infection.
Case report A 35-year-old male from Cameroon, recently residing in Mexico, with a history of pulmonary tuberculosis diagnosed and treated in his origin country a year before, he denied other illness and risk factors for other entities; he also denied personal and family history of hematological pathologies and speci
Data Loading...
