Multiple cutaneous lymphoproliferative disorders showing a retained tumor clone by T-cell receptor gene rearrangement analysis: a case series of four patients and review of the literature.
BACKGROUND
Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma (CTCL), followed by CD30+ lymphoproliferative disorders, including lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL). The objective was to report on a series of patients with different types of CTCL at different times in their clinical course, with a focus on clonality studies.


METHODS
Four patients with multiple diagnoses of CTCLs were identified. The clinical information, treatment interventions, and histopathology were reviewed. T-cell receptor (TCR) gene rearrangement studies were performed on all available specimens.


RESULTS
The four patients carried diagnoses of: (1) pcALCL and MF; (2) pcALCL, LyP, and pcALCL; (3) LyP, MF, and pcALCL; (4) LyP, pcALCL, and MF; each with characteristic presentation and histopathologic findings. The results of the TCR polymerase chain reaction showed that all tumors expressed and retained a TCR clone(s) as follows: (1) biallelic clone; (2) single clone; (3) biallelic clone with additional clone; and (4) single clone, respectively.


CONCLUSIONS
We report a series of four cases of individual patients with coexisting diagnoses of some combination of MF, LyP, and pcALCL, whose lesions presented in nontraditional sequence and demonstrated a retained clone by gene rearrangement analysis.
Departments of Pathology, Dermatopathology, Dermatology, and Clinical Pathology, University of Virginia Health System, Charlottesville, VA, USA.
Anne M
AM
Stowman
Department of Dermatology, East Carolina University, Greenville, NC, USA.
Ling-Lun
LL
Hsia
Departments of Pathology, Dermatopathology, and Clinical Pathology, University of South Carolina School of Medicine-Greenville, Greenville, SC, USA.
William A
WA
Kanner
Departments of Pathology, Dermatopathology, Dermatology, and Clinical Pathology, University of Virginia Health System, Charlottesville, VA, USA.
Mani S
MS
Mahadevan
Department of Clinical Pathology, University of Pittsburgh, Pittsburgh, PA, USA.
Grant C
GC
Bullock
Departments of Pathology, Dermatopathology, Dermatology, and Clinical Pathology, University of Virginia Health System, Charlottesville, VA, USA.
James W
JW
Patterson
D000369:Aged, 80 and over; D000483:Alleles; D005260:Female; D015321:Gene Rearrangement; D019672:Genes, T-Cell Receptor; D006801:Humans; D017730:Ki-1 Antigen / Q000032:analysis; D054446:Lymphoma, Primary Cutaneous Anaplastic Large Cell / Q000737:chemistry / Q000235:genetics* / Q000473:pathology; D017731:Lymphomatoid Papulosis / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000737:chemistry / Q000235:genetics* / Q000473:pathology; D016609:Neoplasms, Second Primary / Q000737:chemistry / Q000235:genetics* / Q000473:pathology; D012878:Skin Neoplasms / Q000737:chemistry / Q000235:genetics* / Q000473:pathology; D055815:Young Adult
D017730:Ki-1 Antigen