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Childhood NHL - Anaplastic Large Cell
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"Cutaneous
CD30+ (Ki-1) anaplastic large-cell lymphoma (cutaneous CD30+ ALCL) is
clinically and pathologically heterogeneous (varying), leading to some
difficulty in its diagnosis and classification.
Expression of CD30 (an
activation marker for B or T cells) in more than 75% of neoplastic
cells characterizes this group of disorders.
Based on clinical manifestations, cutaneous CD30+
ALCL can be subdivided mainly into
1) primary cutaneous (skin) form without extracutaneous involvement at
presentation, or
2) systemic form with secondary skin involvement at
presentation." 1
"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:
bloodjournal.hematologylibrary.org
Treatment
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TREATMENT - Standard of care depends on
cutaneous or systemic presentation,
if cells are ALK -/+), and
clinical circumstances
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BEST PRACTICE (Standard of Care):
NCCN.org
PDF (pages 69-71) requires one-time free registration and login.
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Notable new investigational treatment agent:
SGN-30 (Anti-CD30 mAb) Has a Single-Agent
Response Rate of 21% in Patients with Refractory or Recurrent
Systemic Anaplastic Large Cell Lymphoma (ALCL). Session Type:
Poster Session, Board #896-II
ASH
2006
This phase II study represents one of the largest prospectively
designed trials in relapsed/refractory ALCL and demonstrates good
tolerability and clinically meaningful antitumor activity of
SGN-30, especially in ALK negative patients who have a
particularly poor prognosis.
Resources
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About Cutaneous CD30+ (Ki-1) Anaplastic Large-Cell Lymphoma e-medicine
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Anaplastic large-cell lymphoma: clinical and prognostic
evaluation of 90 adult patients.
J Clin Oncol. 1996 Mar;14(3):955-62. PMID: 8622045 PubMed
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Neutrophil-Rich Ki-1-Positive Anaplastic Large Cell Lymphoma
Medscape
(free login
req. (from American Journal of Clinical Pathology )
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Anaplastic large cell lymphoma, T/null cell, primary cutaneous
or systemic type
Related
PubMed Abstracts
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ASCO 2003 - SGN-30 is a
genetically engineered antibody in clinical trials for the
treatment of patients with CD30+ hematologic malignancies such as
Hodgkin's disease, anaplastic large cell lymphoma and other types
of lymphomas. ASCO
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A population-based study of pediatric anaplastic large cell
lymphoma.
Cancer. 2002 Mar 15;94(6):1830-5. PMID: 11920547 PubMed
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Distinct effects of CD30 and Fas
signaling in cutaneous anaplastic lymphomas: a possible mechanism
for disease progression. J Invest Dermatol. 2000
Dec;115(6):1034-40. PMID: 11121138 PubMed
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Trofosfamide as salvage therapy for anaplastic large cell
lymphoma relapsing after high-dose chemotherapy. Leuk Lymphoma.
2002 Dec;43(12):2351-3.
PMID: 12613523 PubMed
Case study: "This therapy resulted in a complete
remission, still ongoing, 27 months after termination of
intravenous cytotoxic therapy and 16 months after withdrawal of
trofosfamide. Thus, in this particular case, trofosfamide turned
out to be an unexpectedly effective salvage therapy for an
otherwise very aggressive relapsing ALCL."
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Neutrophil-Rich Anaplastic Large Cell Lymphoma of T-Cell
Lineage: A Report of Two Cases Arising in HIV-Positive Patients
Medscape
free login req.
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Anaplastic Large-Cell Lymphoma, T-/Null-Cell Type
Eric Jacobsen, The Oncologist, July 2006
http://bit.ly/aKYe7y
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ALK-negative anaplastic large-cell lymphoma
(ALCL) is different from both ALK-positive ALCL and peripheral
T-cell lymphoma, not otherwise specified
http://bloodjournal.hematologylibrary.org
ALK-positive ALCL patients had a superior outcome compared to
those with ALK-negative ALCL Primary cutaneous ALCL patients had a very favorable 5 y OS
(90%), but with a propensity to relapse (5 y FFS 55%). ALK-negative ALCL should continue to be separated from both ALK-positive
ALCL and PTCL-NOS. Although the prognosis of ALK negative ALCL appears to be better
than PTCL-NOS, it is still unsatisfactory and better therapies
are needed. Primary cutaneous ALCL is associated with an
indolent course.
PubMed Queries on Anaplastic Large Cell Lymphoma
Clinical Trials
Research News
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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.
PMID:
16871285
... 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."
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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.
PMID:
16611309
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Autologous stem cell transplantation for anaplastic large-cell
lymphomas: results of a prospective trial.
Br J Haematol. 2000 Jun;109(4):736-42.
PMID:
10929023
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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
large-cell lymphoma
http://www.nature.com/bmt/journal/v33/n6/full/1704392a.html
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