Manifestasi Anemia Hemolitik Autoimun pada Seorang Penderita Mielofibrosis Primer
DOI:
https://doi.org/10.59188/jurnalsosains.v5i5.32208Keywords:
Primary Myelofibrosis, autoimmune hemolytic anemia, JAK2Abstract
Mielofibrosis primer (PMF) adalah neoplasma mieloproliferatif langka yang ditandai dengan fibrosis sumsum tulang, hematopoiesis ekstrameduler, dan mutasi genetik seperti JAK2. Pada kasus yang jarang terjadi, PMF dapat dipersulit oleh anemia hemolitik autoimun (AIHA), yang menimbulkan tantangan diagnostik dan terapeutik karena mekanisme patofisiologis yang tumpang tindih. Penelitian ini bertujuan untuk mendeskripsikan manifestasi klinis, proses diagnostik, dan pendekatan terapeutik untuk kasus PMF dengan AIHA. Metode laporan kasus deskriptif digunakan, dengan fokus pada pasien wanita berusia 64 tahun yang datang dengan anemia berat, splenomegali, dan hasil positif untuk mutasi JAK2 dan tes Coombs langsung. Diagnosis PMF ditegakkan berdasarkan kriteria WHO 2016 dan didukung oleh temuan biopsi sumsum tulang. Penatalaksanaan yang dilakukan meliputi transfusi sel darah merah, terapi simtomatik, dan pemberian ruxolitinib, inhibitor JAK2. Kasus ini menyoroti perlunya kewaspadaan dalam mendeteksi komplikasi autoimun pada neoplasma mieloproliferatif dan menunjukkan pentingnya strategi diagnostik dan pengobatan yang terintegrasi. Temuan ini diharapkan dapat memberikan kontribusi pada pengetahuan klinis dan menjadi referensi untuk menangani kasus-kasus kompleks serupa dalam praktik hematologi.
References
Abello Polo, V., Saavedra Ramirez, D., Quintero, M., Lobaton, J. F., Sossa Melo, C. L., Lopera, D., Quintero, G., Romero Villa, R., Espinosa, D., Peña, A., Solano, M. H., & Casas, C. P. (2017). Epidemiología de las Neoplasias Mieloproliferativas Crónicas (NMPC): Primer reporte del Registro Colombiano de NMPC. Acta Médica Colombiana, 42(1). https://doi.org/10.36104/amc.2017.624
Alshemmari, S. H., Rajan, R., & Emadi, A. (2016). Molecular pathogenesis and clinical significance of driver mutations in primary myelofibrosis: A review. Medical Principles and Practice, 25, 501–509. https://doi.org/10.1159/000448447
Bakta, I. M. (2012). Hematologi klinik ringkas. Penerbit Buku Kedokteran EGC.
Ballow, M., Ramon, S. S., & Walter, J. E. (2022). Secondary immune deficiency and primary immune deficiency crossovers: Hematological malignancies and autoimmune disease. Frontiers in Immunology, 12. https://www.frontiersin.org/articles/10.3389/fimmu.2021.771003/full
Barcellini, W. (2015). New insight in pathogenesis of autoimmune hemolytic anemia. Transfusion Medicine and Hemotherapy, 42, 287–293. https://doi.org/10.1159/000438825
Barcellini, W., & Fattizzo, B. (2021). Immune phenomena in myeloid neoplasms: An “egg or chicken” question. Frontiers in Immunology, 12. https://www.frontiersin.org/articles/10.3389/fimmu.2021.636363/full
Grinfeld, J., Nangalia, J., Baxter, E. J., Wedge, D. C., Angelopoulos, N., Cantrill, R., Godfrey, A. L., Papaemmanuil, E., Gundem, G., MacLean, C., Cook, J., O’Neil, L., O’Meara, S., Teague, J. W., Butler, A. P., Massie, C. E., Williams, N., Nice, F. L., Andersen, C. L., … Campbell, P. J. (2018). Classification and Personalized Prognosis in Myeloproliferative Neoplasms. New England Journal of Medicine, 379(15). https://doi.org/10.1056/nejmoa1716614
Guglielmelli, P., Rotunno, G., Pacilli, A., Rumi, E., Rosti, V., Delaini, F., Maffioli, M., Fanelli, T., Pancrazzi, A., Pieri, L., Pietra, D., Salmoiraghi, S., Mannarelli, C., Passamonti, F., Rambaldi, A., Barosi, G., Barbui, T., Cazzola, M., & Vannucchi, A. M. (2016). Differences in Clinical and Molecular Characteristics and Outcome in Prefibrotic and Overt Primary Myelofibrosis According to 2016 WHO Criteria. a Study on 639 Patients of the Agimm Group. Blood, 128(22). https://doi.org/10.1182/blood.v128.22.943.943
Hermouet, S., Corbel, E. B., & Gardie, B. (2015). Pathogenesis of myeloproliferative neoplasms: Role and mechanism of chronic inflammation. Clinical and Developmental Immunology. https://doi.org/10.1155/2015/103403
Irhamsyah, M., Muhadi, D., & Arif, M. (2018). Primary myelofibrosis. Indonesian Journal of Clinical Pathology and Medical Laboratory, 25(1).
Johanis, J., & Hajat, A. (2018). Patogenesis Dan Pemeriksaan Laboratoprium Mielofibrosis Primer. Indonesian Journal Of Clinical Pathology And Medical Laboratory, 17(2). https://doi.org/10.24293/ijcpml.v17i2.1025
Kim, T. Y. (2022). Clinical features, gene alterations, and outcomes in prefibrotic and overt primary and secondary myelofibrotic patients.
Leiva, O., Hobbs, G., Ravid, K., & Libby, P. (2022). Cardiovascular Disease in Myeloproliferative Neoplasms: JACC: CardioOncology State-of-the-Art Review. In JACC: CardioOncology (Vol. 4, Issue 2). https://doi.org/10.1016/j.jaccao.2022.04.002
McMullin, M. F., & Anderson, L. A. (2020). Aetiology of myeloproliferative neoplasms. In Cancers (Vol. 12, Issue 7). https://doi.org/10.3390/cancers12071810
Prakash, S., Arber, D. A., Bueso-Ramos, C., Hasserjian, R. P., & Orazi, A. (2023). Advances in myelodysplastic/myeloproliferative neoplasms. In Virchows Archiv (Vol. 482, Issue 1). https://doi.org/10.1007/s00428-022-03465-7
Spivak, J. L. (Ed.). (2018). Harrison’s principles of internal medicine (20th ed.). McGraw-Hill.
Szybinski, J., & Meyer, S. C. (2021). Genetics of Myeloproliferative Neoplasms. In Hematology/Oncology Clinics of North America (Vol. 35, Issue 2). https://doi.org/10.1016/j.hoc.2020.12.002
Tefferi, A. (2020). Primary myelofibrosis: 2021 update on diagnosis, risk-stratification and management. American Journal of Hematology, 96, 145–162. https://doi.org/10.1002/ajh.26035
Zhang, L., Ye, X., Luo, S., Xu, X., Wang, S., Jin, K., Zheng, Y., Zhu, X., Chen, D., Jin, J., & Huang, J. (2023). Clinical features and next-generation sequencing landscape of essential thrombocythemia, prefibrotic primary myelofibrosis, and overt fibrotic primary myelofibrosis: a Chinese monocentric retrospective study. Journal of Cancer Research and Clinical Oncology, 149(6). https://doi.org/10.1007/s00432-022-04067-1
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Ni Luh Mutiara Savrita Dewi
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution-ShareAlike 4.0 International (CC-BY-SA). that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are 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 acknowledgement of its initial publication in this journal.
- 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.
