Anaplastic Large Cell
Lymphoma (ALCL) is a type of
lymphoma (abnormal lymphocyte) affecting mainly t-cells.
It is generally treated as an aggressive lymphoma with intent to
NOTE: We urge patients diagnosed with ALCL to
seek guidance (if feasible) at a center that specializes in
cooperative research. See for a
list of centers.
"The definition of anaplastic large cell lymphoma (ALCL) has
evolved since its original description in 1985 by Stein and
colleagues (1) as a lymphoma characterized by large anaplastic
lymphoid cells with uniform, strong expression of CD30 and a
tendency to grow cohesively and invade lymph node sinuses.
Subsequent immunophenotypic and genetic studies resulted in
restriction of the diagnosis to cases of T-cell or null lineage, and
recognition that primary cutaneous and systemic types were
clinically and immunophenotypically* distinctive.
The current World Health Organization (WHO) (2) classification
distinguishes systemic ALCL from primary cutaneous ALCL (cut-ALCL)."
distinctive means differing in stage of maturation
- based on what is expressed on the
abnormal lymphoid cells - as it relates to the cell of
origin - the corresponding normal lymphoid cells.
Based on clinical manifestations, CD30+
ALCL that presents in the skin can be subdivided mainly into
1) primary cutaneous (skin) form without extracutaneous involvement at
2) systemic form with secondary skin involvement at
"ALK-negative ALCL is defined in the WHO
classification as a lymphoma that is morphologically within the
spectrum of ALK-pos ALCL, with strong and uniform expression of
CD30, but lacking ALK protein expression" Source:
Seattle Genetics and Millennium Announce Positive Top-Line
Brentuximab Vedotin (SGN-35) Data from Phase II Trial in
Relapsed or Refractory ALCL
"Based on overall response rates
of 86 percent in ALCL and 75 percent in HL with single-agent
brentuximab vedotin, we intend to discuss regulatory next steps
with the U.S. Food and Drug Administration (FDA) later this year
with the goal of including both indications in our Biologics
License Application (BLA) submission planned for the first half
of 2011," said Clay B. Siegall, Ph.D., President and Chief
Executive Officer of Seattle Genetics. "These remarkable data in
relapsed or refractory patients highlight the broader
opportunity for brentuximab vedotin in CD30-positive hematologic
malignancies and reinforce our ongoing and planned clinical
development activities. We believe that brentuximab vedotin
represents the next step in the evolution of targeted therapy
and could be the first in a new class of advanced generation
Long-term follow-up of patients with peripheral T-cell lymphomas
treated up-front with high-dose chemotherapy followed by autologous
stem cell transplantation.
Leukemia. 2006 Sep;20(9):1533-8. Epub 2006 Jul 27.
... our findings indicate (1) up-front high-dose therapy and ASCT
are feasible, but could induce a high rate of long-term CR only in
patients with ALK-positive ALCL and (2) the achievement of CR before
auto-grafting is a strong predictor of better survival."
Allogeneic haematopoietic stem cell transplantation in relapsed or
refractory anaplastic large cell lymphoma of children and
adolescents--a Berlin-Frankfurt-Munster group report.
Br J Haematol. 2006 Apr;133(2):176-82.
Autologous stem cell transplantation for anaplastic large-cell
lymphomas: results of a prospective trial.
Br J Haematol. 2000 Jun;109(4):736-42.
High-dose therapy and autologous stem cell transplant does
not result in long-term disease-free survival in patients with
recurrent chemotherapy-sensitive ALK-negative anaplastic