Recognition and Treatment of Concurrent Active and Neurodegenerative Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation with subsequent tissue damage often requiring chemotherapy. Neurodegenerative LCH presents with neuromuscular, cognitive, and behavioral alterations typically occurring years after diagnosis of active LCH. We present a male child with a 4-year history of growth arrest, polyuria, polydipsia, recurrent otitis media, and seborrheic dermatitis. Cutaneous biopsies confirmed LCH and chemotherapy was initiated. During treatment for active LCH he developed neuropsychiatric decline. White matter changes on brain MRI were consistent with neurodegenerative LCH. Treatment was changed to cytarabine and intravenous immunoglobulin. After 1 year of therapy the patient experienced neuropsychological improvement.

Juvenile xanthogranuloma manifesting with LCH‐associated neurodegenerative disease‐like radiological findings

“…13,16 One possible second-line therapy is cytarabine-containing chemotherapy, in combination with vincristine, dexamethasone, or IVIG, which has been effective for some patients with LCH-associated ND. 13,17,18 Because a BRAF inhibitor may be a promising option for LCH-associated ND, 19 discovery of clinically meaningful therapeutic targets is mandatory.…”

Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review

“…Exclusion criteria included articles that were non‐English or not relevant, reported on nonhuman populations, and did not provide access to full text or extraction of relevant data. All studies were assessed for grade of evidence ( Table 1) 1,3–63 and queried for demographics, otologic manifestations, diagnosis, clinical and radiologic findings, as well as medical and surgical management.…”

“…The most common otologic symptom was otorrhea, in 46% of patients in this review, which is consistent with previous studies reporting 45% to 55%. 4,11 Conductive hearing is more typical when 45 1 1 1 Yavas et al 62 1 1 1 1 1 1 S Jeunon et al 27 1 1 1 U Meyer et al 46 1 1 Alexander et al 50 1 1 1 1 1 U Arsovic, Tomanovic, and Bukurov 53 1 Bozdemir et al 26 1 1 1 1 1 1 1C Coleman et al 13 1 1 1 1C Gungadeen, Kullar, and Yates 16 1 1 1 Nishal, Modi, and Balar 41 1 1 1 Robison et al 48 1 1 1 1 Abdel-Aziz et al 7 12 10 4 8 10 C, 2 M Skoulakis et al 22 1 1 1 1 1 1 Xie et al 43 1 1 1 1 1 U Ehrhardt et al 44 1 1 Henderson et al 19 1 1 1 1 S Baser et al 18 1 45 1 1 1 V I 1 Yavas et al 62 1 1 1 V I 1 M A Jeunon et al 27 1 1 1 1 VI 1 Meyer et al 46 1 1 Alexander et al 50 1 1 CU Arsovic, Tomanovic, and Bukurov 53 1 1 MA 1 Bozdemir et al 26 1 1 1 1 1M A Coleman et al 13 1 1 1 V I 1 Gungadeen, Kullar, and Yates 16 1 1 AZ 1 1 CU Nishal, Modi, and Balar 41 Robison et al 48 1 1 1 1 M A 1 MT 1 CU Abdel-Aziz et al 7 12 12 MA 12 Skoulakis et al 22 1 1 1 1 1M T Xie et al 43 1 1 1 V I 1 C U Ehrhardt et al 44 1 19 1 1 1 1U S Baser et al 18 1 hearing loss is present, since otic capsule involvement is rare and the disease usually manifests in the middle ear or external auditory canal. 1,4 Occurrence of sensorineural hearing loss may represent otic capsule involvement.…”

Histiocytic Neoplasms, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

“…68 In addition, cytarabine in combination with intravenous immunoglobulin or vincristine demonstrated therapeutic potential in children and young adults with CNS-LCH and ND-LCH. 69,70 Multifocal skin disease may respond to systemic therapy treatment for multisystem LCH in general. 14 However, small retrospective studies and case reports .…”