PULMONARY AND INTRACRANIAL RADIOGRAPHIC PRESENTATIONS OF LANGERHANS CELL HISTIOCYTOSIS
Background. Langerhans Cell Histiocytosis is a rare disease that affects 1 to 2 adults per million worldwide and often consists of systemic manifestations including pulmonary, intracranial and osteolytic lesions and endocrinologic abnormalities such as Diabetes Insipidus.
Objective. The objective of this case report was to expand the medical literature of this rare disease.
Methods. A case report of a 51-year-old female patient presenting with systemic symptoms as a result of Langerhans Cell Histiocytosis is presented.
Results. A 51-year-old female presented with epistaxis, fatigue, polydipsia, polyuria, headaches and dyspnea. After initial x-rays showed multiple lung and liver nodules and the patient suffered subsequently from a unilateral pneumothorax, an open lung biopsy was recommended. On a pathological basis, the patient was diagnosed with Langerhans Cell Histiocytosis. This report focuses on the radiological presentations of the manifestations of Langerhans Cell Histiocytosis, particularly the presentations in the lung and intracranial regions.
Conclusions. Langerhans Cell Histiocytosis is an incredibly rare disease that presents systemically. Recognizing and differentiating radiographic presentation of these patients is important to determine the need for confirmation by biopsy and early chemotherapeutic intervention.
Baumgartner I, von Hochstetter A, Baumert B, Luetolf U, Follath F. Langerhans’-cell histiocytosis in adults. Med Pediatr Oncol. 1997;28(1):9-14. https://www.ncbi.nlm.nih.gov/pubmed/8950330
Merad M, Ginhoux F, Collin M. Origin, homeostasis and function of Langerhans cells and other langerin-expressing dendritic cells. Nat Rev Immunol. 2008;8(12):935-47. https://www.ncbi.nlm.nih.gov/pubmed/19029989
Egeler RM, van Halteren AG, Hogendoorn PC, Laman JD, Leenen PJ. Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell-macrophage lineage. Immunol Rev. 2010;234(1):213-32. https://www.ncbi.nlm.nih.gov/pubmed/20193021
Brown NA, Furtado LV, Betz BL, Kiel MJ, Weigelin HC, Lim MS, Elenitoba-Johnson KS. High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis. Blood. 2014;124(10):1655–8. http://www.bloodjournal.org/content/bloodjournal/124/10/1655.full.pdf
Annibali S, Cristalli MP, Solidani M, et al. Langerhans cell histiocytosis: oral/periodontal involvement in adult patients. Oral Dis. 2009;15:596. https://hal.archives-ouvertes.fr/hal-00517934/document
Braier JL, Rosso D, Latella A, et al. Importance of multi-lineage hematologic involvement and hypoalbuminemia at diagnosis in patients with "risk-organ" multi-system Langerhans cell histiocytosis. J Pediatr Hematol Oncol. 2010;32:e122. https://journals.lww.com/jpho-online/Abstract/2010/05000/Importance_of_Multi_lineage_Hematologic.26.aspx
Odame I, Li P, Lau L, et al. Pulmonary Langerhans cell histiocytosis: a variable disease in childhood. Pediatr Blood Cancer. 2006;47:889. https://anzjsurg.onlinelibrary.wiley.com/doi/pdf/10.1002/pbc.20676
Grois N, Fahrner B, Arceci RJ, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 2010;156:873. https://neurocluster-db.meduniwien.ac.at/db_files/pub_art_234.pdf
Kaltsas GA, Powles TB, Evanson J, et al. Hypothalamo-Pituitary Abnormalities in Adult Patients with Langerhans Cell Histiocytosis: Clinical, Endocrinological and Radiological Features and Response to Treatment. J Clin Endocrinol Metab. 2000;85:1370-76. https://academic.oup.com/jcem/article/85/4/1370/2851283
Mittheisz E, Seidl R, Prayer D, et al. Central Nervous System-Related Permanent Consequences in Patients With Langerhans Cell Histiocytosis. Pediatric Blood and Cancer. 2007;48:50-56. https://onlinelibrary.wiley.com/doi/abs/10.1002/pbc.20760
Maghnie M, Genovese E, Bernasconi S, Binda S, Arico M. Persistent high MR signal of the posterior pituitary gland in central diabetes insipidus. AJNR Am J Neuroradiol. 1997;18:1749–1752. http://www.ajnr.org/content/ajnr/18/9/1749.full.pdf
Prayer D, Grois N, Prosch H, Gadner H, and Barkovich AJ. MR Imaging Presentation of Intracranial Disease Associated with Langerhans Cell Histiocytosis. AJNR Am J Neuroradiol. 2004;25:880–891. http://www.ajnr.org/content/25/5/880.long
Ganong WF. Circumventricular organs: definition and role in the regulation of endocrine and autonomic function. Clin Exp Pharmacol Physiol. 2000;27:422– 427. https://www.ncbi.nlm.nih.gov/pubmed/10831247
Gadner H, Grois N, Pötschger U, et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood. 2008;111:2556. http://www.bloodjournal.org/content/bloodjournal/111/5/2556.full.pdf
Authors who sent their manuscript to International Journal of Medicine and Medical Research agree to the following terms:
1. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License CC-BY-NC that allows others to share the work with an acknowledgment of the work's authorship and initial publication in this journal.
2. Authors able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in this journal.
3. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).