|
|
 Previous Article | Table of Contents | Next Article 
Blood, 15 August 2001, Vol. 98, No. 4, pp. 1209-1216
NEOPLASIA
Unusual childhood extramedullary hematologic malignancy with
natural killer cell properties that contains tropomyosin
4-anaplastic lymphoma kinase gene fusion
Sandra J. Meech,
Loris McGavran,
Lorrie F. Odom,
Xiayuan Liang,
Lynne Meltesen,
Jacob Gump,
Qi Wei,
Soren Carlsen, and
Stephen P. Hunger
From the Section of Pediatric Hematology/Oncology,
Departments of Pediatrics and Pathology, University of Colorado Health
Sciences Center; The Children's Hospital; and the University of
Colorado Cancer Center, Denver, CO.
This report describes an unusual extramedullary hematologic
malignancy in an 18-month-old child who presented with a capillary leak
syndrome that evolved into hyperleukocytosis with malignant cells. The
circulating tumor cells did not express an antigen profile typical of
any subtype of leukemia commonly observed in children. Tumor cells were
CD3 /CD56+; had germline TCR
genes; and strongly expressed CD30, epithelial membrane antigen, and
anaplastic lymphoma kinase (ALK) consistent with a null cell anaplastic
large cell lymphoma (ALCL). The malignant cells contained a
t(2;19)(p23;p13.1) that interrupted ALK and translocated it
to the der(19). Reverse transcriptase-polymerase chain reaction and
nucleotide sequence analysis revealed fusion of ALK to
tropomyosin 4, an ALK fusion partner not described previously in
hematologic malignancies. The clinical presentation and phenotypic features of this malignancy were not typical for ALCL because tumor
cells expressed both myeloid (CD13, CD33, HLA-DR) and natural killer
(NK) cell antigens. The neoplastic cells most resembled NK cells
because in addition to being CD3/CD56+ with
germline TCR genes, these cells were
CD25+/CD122+/granzyme B+ and
possessed the functional properties of immature NK cells. The unusual
clinical presentation, immunophenotype, and functional properties of
these neoplastic cells suggest that this malignancy may be derived from
the putative myeloid-NK precursor cell. Furthermore co-expression of NK
and ALCL features supports the concept that a minority of null-ALCL may
be derived from NK cells and expands the spectrum of phenotypes that
can be seen in tumors produced by ALK fusion proteins.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
B C Gleason and J L Hornick
Inflammatory myofibroblastic tumours: where are we now?
J. Clin. Pathol.,
April 1, 2008;
61(4):
428 - 437.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Falini and D. Y. Mason
Proteins encoded by genes involved in chromosomal alterations in lymphoma and leukemia: clinical value of their detection by immunocytochemistry
Blood,
January 15, 2002;
99(2):
409 - 426.
[Abstract]
[Full Text]
[PDF]
|
|
|
| |
