Abstract
We report 15 patients with CD4-CD8- "double-negative" T-cell lymphoma arising in skin. There were seven women and eight men with a mean age at diagnosis of 53 years (range 19-77 years). All but two patients presented with solitary or multiple cutaneous nodule(s). Initial and recurrent biopsy specimens showed a dense infiltrate centered in the mid-dermis (extending into subcutis when sampled) of small to intermediate-sized lymphocytes with indistinct nucleoli and frequently irregular nuclear contours. Periadnexal infiltration and epidermal ulceration were present in five cases with the intraepidermal cells being primarily reactive CD4+ T cells. All cases were negative for CD30 and terminal deoxynucleotidyl-transferase; one showed expression of CD56, and six of eight tested cases were positive for T-cell receptor-8 expression. Despite systemic chemotherapy, all 12 patients with clinical follow-up showed recurrent or progressive disease with wide-spread cutaneous dissemination in 10 of 12. Eventual dissemination to lymph nodes or bone marrow occurred in two patients each, with at least nine patients dead of disease or treatment complications. Only two patients achieved lasting clinical remission (with 2′-deoxycoformycin/pentostatin and nelarabine, respectively). CD4-CD8- "double-negative" CTCL has distinctive histologic features and cytomorphology with a marked propensity for rapid multifocal cutaneous dissemination.
| Original language | English |
|---|---|
| Pages (from-to) | 225-231 |
| Number of pages | 7 |
| Journal | American Journal of Surgical Pathology |
| Volume | 26 |
| Issue number | 2 |
| DOIs | |
| State | Published - Feb 16 2002 |
| Externally published | Yes |
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Keywords
- Gamma-delta T cells
- Skin disease
- T-cell lymphoma
- Tumor markers
ASJC Scopus subject areas
- Anatomy
- Pathology and Forensic Medicine
Cite this
CD4- CD8- 'double-negative' cutaneous T-cell lymphomas share common histologic features and an aggressive clinical course. / Jones, Dan; Vega, Francisco; Sarris, Andreas H.; Medeiros, L. Jeffrey.
In: American Journal of Surgical Pathology, Vol. 26, No. 2, 16.02.2002, p. 225-231.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - CD4- CD8- 'double-negative' cutaneous T-cell lymphomas share common histologic features and an aggressive clinical course
AU - Jones, Dan
AU - Vega, Francisco
AU - Sarris, Andreas H.
AU - Medeiros, L. Jeffrey
PY - 2002/2/16
Y1 - 2002/2/16
N2 - We report 15 patients with CD4-CD8- "double-negative" T-cell lymphoma arising in skin. There were seven women and eight men with a mean age at diagnosis of 53 years (range 19-77 years). All but two patients presented with solitary or multiple cutaneous nodule(s). Initial and recurrent biopsy specimens showed a dense infiltrate centered in the mid-dermis (extending into subcutis when sampled) of small to intermediate-sized lymphocytes with indistinct nucleoli and frequently irregular nuclear contours. Periadnexal infiltration and epidermal ulceration were present in five cases with the intraepidermal cells being primarily reactive CD4+ T cells. All cases were negative for CD30 and terminal deoxynucleotidyl-transferase; one showed expression of CD56, and six of eight tested cases were positive for T-cell receptor-8 expression. Despite systemic chemotherapy, all 12 patients with clinical follow-up showed recurrent or progressive disease with wide-spread cutaneous dissemination in 10 of 12. Eventual dissemination to lymph nodes or bone marrow occurred in two patients each, with at least nine patients dead of disease or treatment complications. Only two patients achieved lasting clinical remission (with 2′-deoxycoformycin/pentostatin and nelarabine, respectively). CD4-CD8- "double-negative" CTCL has distinctive histologic features and cytomorphology with a marked propensity for rapid multifocal cutaneous dissemination.
AB - We report 15 patients with CD4-CD8- "double-negative" T-cell lymphoma arising in skin. There were seven women and eight men with a mean age at diagnosis of 53 years (range 19-77 years). All but two patients presented with solitary or multiple cutaneous nodule(s). Initial and recurrent biopsy specimens showed a dense infiltrate centered in the mid-dermis (extending into subcutis when sampled) of small to intermediate-sized lymphocytes with indistinct nucleoli and frequently irregular nuclear contours. Periadnexal infiltration and epidermal ulceration were present in five cases with the intraepidermal cells being primarily reactive CD4+ T cells. All cases were negative for CD30 and terminal deoxynucleotidyl-transferase; one showed expression of CD56, and six of eight tested cases were positive for T-cell receptor-8 expression. Despite systemic chemotherapy, all 12 patients with clinical follow-up showed recurrent or progressive disease with wide-spread cutaneous dissemination in 10 of 12. Eventual dissemination to lymph nodes or bone marrow occurred in two patients each, with at least nine patients dead of disease or treatment complications. Only two patients achieved lasting clinical remission (with 2′-deoxycoformycin/pentostatin and nelarabine, respectively). CD4-CD8- "double-negative" CTCL has distinctive histologic features and cytomorphology with a marked propensity for rapid multifocal cutaneous dissemination.
KW - Gamma-delta T cells
KW - Skin disease
KW - T-cell lymphoma
KW - Tumor markers
UR - http://www.scopus.com/inward/record.url?scp=0036158928&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0036158928&partnerID=8YFLogxK
U2 - 10.1097/00000478-200202000-00009
DO - 10.1097/00000478-200202000-00009
M3 - Article
VL - 26
SP - 225
EP - 231
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
SN - 0147-5185
IS - 2
ER -