Biomaterial Database

Central nervous system involvement in adult-onset relapsing hemophagocytic lymphohistiocytosis responsive to maintenance treatment with anakinra. 2021

Juan Diego Guerra Hiraldo, and Ana Domínguez-Mayoral, and Francisco Javier García-Gómez, and Natalia Fouz-Rosón, and Eloy Rivas-Infante, and Marco Antonio Montes Cano, and Alicia Rodríguez Fernández, and Salvador García Morillo, and Nayua Alkadi Fernández, and Jose Antonio Pérez de León, and Guillermo Navarro Mascarell, and Carmen Vargas Lebrón

Department of Neurology, Hospital Universitario Virgen Macarena, Seville, Spain. Electronic address: juand.guerra.sspa@juntadeandalucia.es.

A 43 year-old male presented with a relapsing and progressive systemic inflammatory disorder with central nervous system (CNS) involvement. After a two years follow up, he was diagnosed with hemophagocytic lymphohistiocytosis (HLH), based on clinical, laboratory and radiological findings. Treatment was started with anakinra, a recombinant humanised interleukin-1 (IL-1) receptor antagonist. Clinical response was good. Laboratory and radiological findings showed no disease activity throughout a five years follow-up period. Several immunosuppressive agents have been used in HLH without any good outcomes. This is the first case report of HLH with CNS involvement responsive to chronic treatment with anakinra.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D012008	Recurrence	The return of a sign, symptom, or disease after a remission.	Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D005500	Follow-Up Studies	Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.	Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013127	Spinal Nerves	The 31 paired peripheral nerves formed by the union of the dorsal and ventral spinal roots from each spinal cord segment. The spinal nerve plexuses and the spinal roots are also included.	Nerve, Spinal,Nerves, Spinal,Spinal Nerve
D016896	Treatment Outcome	Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.	Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes
D017472	Receptors, Interleukin-1	Cell surface receptors that are specific for INTERLEUKIN-1. Included under this heading are signaling receptors, non-signaling receptors and accessory proteins required for receptor signaling. Signaling from interleukin-1 receptors occurs via interaction with SIGNAL TRANSDUCING ADAPTOR PROTEINS such as MYELOID DIFFERENTIATION FACTOR 88.	IL-1 Receptor,IL-1 Receptors,IL1 Receptor,Interleukin-1 Receptor,Interleukin-1 Receptors,Receptor, Interleukin-1,Receptors, IL-1,IL1 Receptors,Interleukin 1 Receptor,IL 1 Receptor,IL 1 Receptors,Interleukin 1 Receptors,Receptor, IL-1,Receptor, IL1,Receptor, Interleukin 1,Receptors, IL 1,Receptors, IL1,Receptors, Interleukin 1
D051359	Lymphohistiocytosis, Hemophagocytic	A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive.	Familial Hemophagocytic Lymphocytosis,Hemophagocytic Lymphohistiocytosis, Familial,Hemophagocytic Syndrome, Infection-Associated,Hemophagocytic Syndrome, Reactive,Hemophagocytic Syndromes,Reactive Hemophagocytic Syndrome,Erythrophagocytic Lymphohistiocytosis, Familial,Familial Erythrophagocytic Lymphohistiocytosis,Familial Hemophagocytic Histiocytosis,Familial Hemophagocytic Lymphohistiocytosis,Familial Hemophagocytic Reticulosis,Familial Histiocytic Reticulosis,Hemophagocytic Lymphohistiocytosis Familial -1,Hemophagocytic Lymphohistiocytosis, Familial, 1,Hemophagocytic Reticulosis, Familial,Hemophagocytic Syndrome,Primary Hemophagocytic Hymphohistiocytosis,Primary Hemophagocytic Lymphohistiocytosis,Reticulosis, Familial Histiocytic,Erythrophagocytic Lymphohistiocytoses, Familial,Familial Erythrophagocytic Lymphohistiocytoses,Familial Hemophagocytic Histiocytoses,Familial Hemophagocytic Lymphocytoses,Familial Hemophagocytic Lymphohistiocytoses,Familial Hemophagocytic Reticuloses,Familial Histiocytic Reticuloses,Hemophagocytic Histiocytoses, Familial,Hemophagocytic Histiocytosis, Familial,Hemophagocytic Hymphohistiocytoses, Primary,Hemophagocytic Hymphohistiocytosis, Primary,Hemophagocytic Lymphocytoses, Familial,Hemophagocytic Lymphocytosis, Familial,Hemophagocytic Lymphohistiocytoses,Hemophagocytic Lymphohistiocytoses, Familial,Hemophagocytic Lymphohistiocytoses, Primary,Hemophagocytic Lymphohistiocytosis,Hemophagocytic Lymphohistiocytosis Familial 1,Hemophagocytic Lymphohistiocytosis, Primary,Hemophagocytic Reticuloses, Familial,Hemophagocytic Syndrome, Infection Associated,Histiocytic Reticuloses, Familial,Histiocytic Reticulosis, Familial,Histiocytoses, Familial Hemophagocytic,Histiocytosis, Familial Hemophagocytic,Hymphohistiocytoses, Primary Hemophagocytic,Hymphohistiocytosis, Primary Hemophagocytic,Infection-Associated Hemophagocytic Syndrome,Lymphocytoses, Familial Hemophagocytic,Lymphocytosis, Familial Hemophagocytic,Lymphohistiocytoses, Familial Erythrophagocytic,Lymphohistiocytoses, Familial Hemophagocytic,Lymphohistiocytoses, Hemophagocytic,Lymphohistiocytoses, Primary Hemophagocytic,Lymphohistiocytosis, Familial Erythrophagocytic,Lymphohistiocytosis, Familial Hemophagocytic,Lymphohistiocytosis, Primary Hemophagocytic,Primary Hemophagocytic Hymphohistiocytoses,Primary Hemophagocytic Lymphohistiocytoses,Reticuloses, Familial Hemophagocytic,Reticuloses, Familial Histiocytic,Reticulosis, Familial Hemophagocytic