- Arch
Pathol Lab Med. 2000 Dec;124(12):1804-8.
CD13-positive anaplastic large cell lymphoma
of T-cell origin--a diagnostic and histogenetic problem.
Popnikolov NK, Payne DA, Hudnall SD, Hawkins HK, Kumar M, Norris
BA, Elghetany MT.
Department of Pathology, The University of Texas Medical Branch,
Galveston 77555-0743, USA.
The expression of myelomonocytic-associated antigens in
anaplastic large cell lymphomas (ALCLs), particularly those
presenting in extranodal sites, can make their distinction from
extramedullary myeloid cell tumors (EMCTs) or histiocytic tumors
problematic. Yet, this distinction is clinically significant
because of its therapeutic and prognostic implications. Herein,
we describe a case of extranodal anaplastic lymphoma kinase-positive
CD30-positive ALCL of T-cell origin in a 12-year-old boy, which
was initially called an EMCT because of the expression of CD13
and HLA-DR detected by flow cytometry and the absence of other
T-cell-related surface markers. However, the detection of
cytoplasmic CD3 by flow cytometry prompted further studies. The
tumor was composed of large cells with abundant slightly
eosinophilic vacuolated cytoplasm and ovoid or reniform nuclei
with a few small nucleoli. Using immunohistochemistry, the tumor
was positive for CD45, CD30, CD45RO, and CD43 with a strong
cytoplasmic and nuclear anaplastic lymphoma kinase stain. The
tumor cells showed a T-cell clonal genotype. Electron microscopy
revealed no ultrastructural features of myelomonocytic or
histiocytic origin. The patient responded well to the
chemotherapy and was in complete remission for 10 months at the
time of submission of this manuscript. Review of the literature
showed inconsistencies regarding the diagnosis, nomenclature,
and, therefore, treatment and prognosis of these tumors. In
addition, the CD13 expression in ALCL raises some histogenetic
questions and may indicate origin from a pluripotent stem cell,
misprogramming during malignant transformation, or a
microenvironmental effect on lymphoid cell expression of surface
antigens. Therefore, ALCL should be considered in the
differential diagnosis of EMCTs or histiocytic tumors,
particularly when surface marker lineage assignment is
ambiguous.
PMID: 11100061 [PubMed - indexed for MEDLINE]
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