Histiositosis Sel Langerhans Multisistem: Laporan Kasus

Mulat Muliasih, Tri Ratnaningsih

Sari


Histiositosis sel Langerhans (HSL) dapat terjadi pada segala usia, sejak neonatus sampai usia dewasa. Lebih dari sepertiga kasus, terutama pada usia kurang dari 3 tahun, cenderung menderita HSL multisistem dengan disfungsi organ. Patogenesis HSL juga masih belum jelas. Diduga berhubungan dengan proses reaksi terhadap infeksi atau merupakan suatu kelainan genetik yang menyebabkan kerusakan sistem imunoregulator. Laporan kasus ini bertujuan untuk menjelaskan penegakan diagnosis HSL pada pasien dengan keterlibatan multipel organ. Anak laki-laki berusia 1 tahun 4 bulan datang dengan dengan keluhan demam hampir satu bulan, perut membesar, dan bercak kemerahan hampir di tubuh. Pemeriksaan fisik didapatkan pucat, kuning, makula & purpuric multipel hampir di seluruh tubuh, terutama leher dan kepala, otitis media, hepatosplenomegali, hidrocele. Hasil laboratorium didapatkan pansitopenia, bone marrow punction ditemukan peningkatan sel histiosit, dan hasil immunohistokima positif pada CD1a dan S100. 

 


Kata Kunci


Anak; Diagnosis; Histiositosis sel Langerhans; Multipel organ

Teks Lengkap:

PDF

Referensi


Abla O, Egeler RM, and Weitzman S. (2010). Langerhans Cell Histiocytosis: Current Concepts and Treatments. Cancer Treatment Reviews, 36(4): 354-359.

Emile, J.F. et al. (2016). Histiocyte Society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood, Jun 2;127(22):2672-81. doi: 10.1182/blood-2016-01-690636.

Gadner H. et al. (2013). Histiocyte Society. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis. Blood, 20;121(25):5006-14. doi: 10.1182/blood-2012-09-455774.

Haupt R. et al. (2013). Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatric blood & cancer, 60(2):175-84.

Herwiq M.C. et al. (2008). Histiocytosis: Update for the Pediatrician. Current Langerhans Cell Histiocytosis of the Orbit: Five Opinion in Pediatrics. Clinicopathologic Cases and Review of the Literature, 20(1): 23-29.

Kumar M. et al. (2015). Bone marrow infiltration in Langerhan's cell histiocytosis - An unusual but important determinant for staging and treatment. Int J Hematol Oncol Stem Cell Res, 1;9(4):193-7.

Krüger C. et al. (2007). The hematopoietic factor GM-CSF (granulocyte-macrophage colony-stimulating factor) promotes neuronal differentiation of adult neural stem cells in vitro. BMC Neurosci, 22;8:88. doi: 10.1186/1471-2202-8-88.

Lusiana. et al. (2019). Langerhans cell histiocytosis: Diagnosis and Management. Journal of General – Procedural Dermatology & Venereology Indonesia, Vol. 4: Iss. 1, Article 4. doi: 10.19100/jdvi.v4i1.161.

Navaneethan, U., Parsi, M.A. (2012). Langerhans cell histiocytosis and choledocholithiasis: is there an association? Gastroenterol Hepatol (N Y), 8(11):768-9.

Ning-Xia, S., Dan, C., Qian, Z., and Wen LI. (2014). Langerhans Cell Histiocytosis on the Vulva: A Case Report and Review of the Literature. Journal of Reproduction & Contraception, 25(2): 123-128.

Ottink, M., Feijen, S., Rosias, P., et al. (2013). Langerhans Cell Histiocytosis Presenting with Complicated Pneumonia, a Case Report. Respiratory Medicine Case Reports, 8: 28-31.

Reddy I.S., Gowrishankar S., Somani V.K., Murthy D.B, (2014). Adult onset Langerhans cell histiocytosis: Report of two patients. IJDVL, 1;80(6):560.

Sahm, F., Capper, D., Preusser, M., Meyer, J., Stenzinger, A., et al. (2012). BRAFV600E mutant protein is expressed in cells of variable maturation in Langerhans cell histiocytosis. Blood, 20;120(12):e28-34. doi: 10.1182/blood-2012-06-429597.

Simko, S.J. et al. (2014). Differentiating skin-limited and multisystem Langerhans cell histiocytosis. J. Pediatr, 165(5):990-6.

Surico, G., Muggeo, P., Muggeo, V., et al. (2000). Ear Involvement in Childhood Langerhans' Cell Histiocytosis. Head & Neck: Journal for the Sciences and Specialties of the Head and Neck, 22(1):42-7.

Uppuluri R. et al. (2017). Excellent remission rates with limited toxicity in relapsed/refractory Langerhans cell histiocytosis with pulse dexamethasone and lenalidomide in children. Pediatr Blood Cancer, 64(1):110-2.




DOI: https://doi.org/10.33024/jikk.v12i6.20458

Refbacks

  • Saat ini tidak ada refbacks.


##submission.copyrightStatement##

##submission.license.cc.by-nc4.footer##

Pendidikan Dokter Universitas Malahayati Lampung


Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.