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Hlh up to date8/15/2023 ![]() ![]() The term “central nervous system (CNS) disease” is frequently used in the HLH-related literature but to date, there is no consensus regarding its definition. The incidence of primary HLH in adults or that of secondary HLH is not studied. Primary HLH is rare, with an estimated yearly incidence in Sweden of 0.12–0.15 per 100,000 children. It may result from genetic defects (primary HLH ) or be acquired with infectious, neoplastic, autoinflammatory, autoimmune, and immunodeficiency etiologies (secondary HLH). Hemophagocytic lymphohistiocytosis (HLH) is not a single disease but rather a clinical syndrome of life-threatening hyperinflammation. Since CNS-HLH is a condition leading to the most severe late effects of HLH, early expert consultation is recommended. Hematopoietic stem cell transplantation (HSCT) also represents an important CNS-HLH treatment patients with primary HLH may benefit from immediate HSCT even if there is active disease at time of transplantation, though care should be taken to monitor CNS inflammation through HSCT and treat if needed. An interesting novel approach is an anti-IFN-gamma antibody (NI-0501), which is currently being tested. Therapy must be initiated without inappropriate delay to prevent late effects in HLH. ![]() ![]() In addition, intrathecal treatment with methotrexate and corticosteroids has become a standard care and is likely to be beneficial. Treatment options for CNS-HLH include, but are not limited to, those commonly used in systemic HLH, including corticosteroids, etoposide, cyclosporine A, alemtuzumab, and ATG. In addition to the blood tests required to make a diagnosis of HLH, a lumbar puncture with cerebrospinal fluid (CSF) analysis and magnetic resonance imaging (MRI) should always be done in all cases regardless of the presence or absence of neurological signs or symptom. A careful review of the patient’s history and a thorough neurological examination are essential. To initiate proper treatment, a correct diagnosis must be made. It is also important to consider primary HLH as an underlying cause of “unknown CNS inflammation” as these patients may present with only CNS disease. Reactivations within the CNS are frequent during the course of HLH treatment and may occur concomitant with or independent of systemic relapses. The vast majority of patients with CNS-HLH also have systemic HLH and a large number of patients with primary and secondary HLH have CNS involvement. Central nervous system (CNS)-hemophagocytic lymphohistiocytosis (HLH) is not a disease in itself, but it is part of a systemic immune response. ![]()
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