scholarly journals Brentuximab vedotin for refractory anaplastic lymphoma kinase-negative anaplastic large cell lymphoma in leukemic phase with RUNX3 overexpression

2020 ◽  
Vol 12 (1) ◽  
Author(s):  
Yusuke Yamashita ◽  
Yoshikazu Hori ◽  
Hideki Kosako ◽  
Takehiro Oiwa ◽  
Kenji Warigaya ◽  
...  

Anaplastic lymphoma kinase (ALK)- negative anaplastic large cell lymphoma (ALCL) is an aggressive CD30-positive non- Hodgkin lymphoma. ALK-ALCL rarely manifests with extensive bone marrow and peripheral blood involvement (known as “leukemic phase”). A 54-year-old woman was diagnosed with ALK-ALCL in leukemic phase, characterized by an extremely poor prognosis. Lymphoma cells in this case showed chromosomal translocation 1p36.1- encoded RUNX3 and overexpression of its protein. She was refractory to CHOP and salvage chemotherapy. Fortunately, she achieved complete remission with three cycles of Brentuximab vedotin (BV) and underwent umbilical cord blood transplantation. However, she died due to treatment-related mortality on day 129. The autopsy findings showed no lymphoma cells. Treatment strategy for ALK-ALCL is controversial, but the efficacy of BV in CD30-positive peripheral T-cell lymphoma not only as salvage regimens, but also in first line, has been reported in recent years. BV may be an effective option for ALK-ALCL in leukemic phase.

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1220
Author(s):  
Shih-Lung Chen ◽  
Kai-Chieh Chan

Anaplastic large-cell lymphoma (ALCL), a form of non-Hodgkin’s lymphoma, is rare in the external auditory canal (EAC). ALCL in the EAC region is challenging for clinicians and pathologists. We report a 34-year-old male presented with the chief complaint of a painless mass in the left EAC for approximately 2 weeks. Anaplastic lymphoma kinase (ALK)- and CD30-positive ALCL were confirmed using computed tomography, positron emission tomography, histopathological examination and IHC staining. We compared the clinicopathological characteristics of our patient with those of previous cases. The biopsy and IHC findings confirmed the rare diagnosis of ALK- and CD30-positive ALCL of the EAC. Radiotherapy and concurrent chemoradiotherapy are indicated for lymphoma depending on the extent of the disease. Brentuximab vedotin as initial salvage therapy should be considered for recurrent or refractory ALK-positive ALCL.


2011 ◽  
Vol 54 (3) ◽  
pp. 599 ◽  
Author(s):  
Sumeet Gujral ◽  
VijayaS Gadage ◽  
PG Subramanian ◽  
KomalS Galani ◽  
KunalK Sehgal ◽  
...  

2004 ◽  
Vol 128 (3) ◽  
pp. 324-327
Author(s):  
Edward H. Rowsell ◽  
Nazila Zekry ◽  
Boleslaw H. Liwnicz ◽  
Jeffrey D. Cao ◽  
Qin Huang ◽  
...  

Abstract Anaplastic large cell lymphoma is a unique diagnostic subcategory of the T-cell lymphomas in the current World Health Organization classification. Representing approximately 3% of adult and 10% to 30% of childhood non-Hodgkin lymphomas, anaplastic large cell lymphoma classically consists of CD30+ large lymphoid cells with abundant cytoplasm and pleomorphic, often horseshoe-shaped or kidney-shaped nuclei. Among the reported nodal and extranodal sites of occurrence, the gastrointestinal tract and central nervous system have rarely been noted. We report a case of primary anaplastic lymphoma kinase–negative anaplastic large cell lymphoma in the brain of a 46-year-old patient with acquired immunodeficiency syndrome. T-cell lineage was confirmed by T-cell receptor γ chain gene rearrangements using polymerase chain reaction, and extra copies of the anaplastic lymphoma kinase gene of chromosome 2 were demonstrated by fluorescence in situ hybridization analysis. To our knowledge, primary anaplastic large cell lymphoma of the brain has not previously been reported in acquired immunodeficiency syndrome.


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