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2014 ; 123
(17
): 2636-44
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How I treat the peripheral T-cell lymphomas
#MMPMID24615779
Moskowitz AJ
; Lunning MA
; Horwitz SM
Blood
2014[Apr]; 123
(17
): 2636-44
PMID24615779
show ga
The peripheral T-cell lymphomas (PTCLs) encompass a heterogeneous group of
diseases that have generally been associated with poor prognosis. The most common
PTCLs, peripheral T-cell lymphoma, not otherwise specified, angioimmunoblastic
T-cell lymphoma, and anaplastic lymphoma kinase (ALK)-negative anaplastic large
cell lymphoma (ALK-negative), despite their unique presentations and histologies,
are currently treated similarly. Here we discuss our general approach to the
treatment of the most common PTCLs. Based on the best data currently available,
which include retrospective analyses and phase 2 prospective studies, our
approach has involved cyclophosphamide, doxorubicin, vincristine,
prednisone-based therapy followed by consolidation in first remission with
autologous stem cell transplant. This treatment strategy likely improves the
outcome for patients compared with historical series; however, progression-free
survival rates remain disappointing, ranging from 40% to 50%. This is currently
an exciting time in the treatment of PTCL due to the advent of recently approved
drugs as well as new targeted agents currently under investigation. In addition,
gene expression profiling is allowing for a better understanding of underlying
disease biology, improved diagnostic accuracy, and prognostication in PTCL. As a
result, over the next few years, we expect a significant shift in our management
of these diseases with a move toward more individualized therapy leading to
improved outcomes.
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