Leukemic Phase of ALK-Negative Anaplastic Large Cell Lymphoma Presenting as Leukostasis: The First Case Report in Thailand
Leukemic Phase of ALK-Negative ALCL
DOI:
https://doi.org/10.69898/jhtm.35.2025.273239Keywords:
ALK-negative anaplastic large cell lymphoma, Leukemic phase, T-cell lymphomaAbstract
Anaplastic large cell lymphoma (ALCL) is an aggressive T-cell lymphoma characterized by hallmark cells that are CD30-positive. ALCL is subcategorized as anaplastic lymphoma kinase (ALK)-positive and ALK-negative types. The leukemic phase of ALK-negative ALCL is extremely rare and usually has a poor prognosis. We report the first case of the leukemic phase of ALK-negative ALCL in Thailand. A 60-year-old man presented bilateral cervical lymphadenopathy and night sweats for 6 months. He was admitted to the hospital with acute fever, progressive dyspnea on exertion, severe headache and blurred vision. His complete blood count revealed marked leukocytosis (352.5 x 109/L) with numerous atypical medium- to large-sized mononuclear cells. The results of his laboratory investigations were compatible with spontaneous tumor lysis syndrome and disseminated intravascular coagulation. The flow cytometry analysis from bone marrow aspiration along with histopathologic findings and immunohistochemistry from bone marrow study were consistent with ALK-negative ALCL. The patient was treated with leukapheresis, renal replacement therapy and high-dose dexamethasone. After achieving hemodynamic stability, a computed tomography scan of the brain was performed due to persistent alteration of consciousness. The results showed extensive intracerebral hemorrhage with severe brain edema and tonsillar herniation. Due to this complication, the treatment plan was adjusted to palliative care with supportive treatment after discussion with his family. The patient subsequently passed away from septic shock.
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References
Roman E, Smith AG. Epidemiology of lymphomas. Histopathology. 2011;58:4-14.
Pina-Oviedo S, Ortiz-Hidalgo C, Carballo-Zarate AA, Zarate-Osorno A. ALK-Negative Anaplastic Large Cell Lymphoma: Current Concepts and Molecular Pathogenesis of a Heterogeneous Group of Large T-Cell Lymphomas. Cancers (Basel). 2021;13:4667.
Miranda R, Ott G, Cozzolino I. ALK-negative anaplastic large cell lymphoma. In: Lim M, Dave SS, Du MQ. WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Vol 11. 5 ed. Lyon (France) 2024.
Alaggio R, Amador C, Anagnostopoulos I, Attygalle AD, Araujo IBO, Berti E, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36:1720-48.
Qiu L, Medeiros LJ, Tang G, Khanlari M, Li S, Konoplev S, et al. The Leukemic Phase of ALK-Negative Anaplastic Large Cell Lymphoma Is Associated with CD7 Positivity, Complex Karyotype, TP53 Deletion, and a Poor Prognosis. Cancers (Basel). 2021;13:6316.
Lu Y, Zhao X, Wang E, Chen W, Huang Q. ALK-negative anaplastic large cell lymphoma with extensive peripheral blood and bone marrow involvements manifested as "leukemic phase". Leuk Res. 2010;34:475-82.
Zhang XR, Chien PN, Nam SY, Heo CY. Anaplastic Large Cell Lymphoma: Molecular Pathogenesis and Treatment. Cancers (Basel). 2022;14:1650.
Karki NR, Badin K, Savage N, Bryan L. Leukaemic relapse of anaplastic large cell lymphoma, ALK negative. BMJ Case Rep. 2021;14:e239213.
Huang Q, Gaal KK, Nademanee A. Acute leukemic manifestation of recurrent anaplastic large-cell lymphoma 20 years after autologous bone marrow transplantation. J Clin Oncol. 2012;30(3):e34-6.
Yamashita Y, Hori Y, Kosako H, Oiwa T, Warigaya K, Mushino T, et al. Brentuximab vedotin for refractory anaplastic lymphoma kinase-negative anaplastic large cell lymphoma in leukemic phase with RUNX3 overexpression. Hematol Rep. 2020;12(1):8368.
Kasinathan G. Leukemic phase of ALK-negative anaplastic large cell lymphoma in a patient who is on androgenic steroids: A case report. Ann Med Surg (Lond). 2020;49:1-4.
Savage KJ, Harris NL, Vose JM, Ullrich F, Jaffe ES, Connors JM, et al. ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood. 2008;111:5496-504.
Wong WS, Liu BW, Lam FS, Wong KF. ALK-negative anaplastic large cell lymphoma in leukemic phase with near-pentaploidy. Leuk Lymphoma. 2010;51:1927-30.
Horwitz S, O'Connor OA, Pro B, Illidge T, Fanale M, Advani R, et al. Brentuximab vedotin with chemotherapy for CD30-positive peripheral T-cell lymphoma (ECHELON-2): a global, double-blind, randomised, phase 3 trial. Lancet. 2019;393:229-40.
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