Cutaneous γδ T-cell lymphomas: a spectrum of presentations with overlap with other cytotoxic lymphomas.
We reviewed our multicenter experience with gamma-delta (γδ) T-cell lymphomas first presenting in the skin. Fifty-three subjects with a median age of 61 years (range, 25 to 91 y) were diagnosed with this disorder. The median duration of the skin lesions at presentation was 1.25 years (range, 1 mo to 20 y). The most common presentation was deep plaques (38 cases) often resembling a panniculitis, followed by patches resembling psoriasis or mycosis fungoides (10 cases). These lesions tended to ulcerate overtime (27 cases). Single lesions or localized areas of involvement resembling cellulitis or pyoderma were reported in 8 cases. The most common anatomic site of involvement was the legs (40 cases), followed by the torso (30 cases) and arms (28 cases). Constitutional symptoms were reported in 54% (25/46) of the patients, including some with limited skin involvement. Significant comorbidities included autoimmunity (12 cases), other lymphoproliferative disorders (5 cases), internal carcinomas (4 cases), and viral hepatitis (2 cases). Lymphadenopathy (3/42 cases) and bone marrow involvement (5/28 cases) were uncommon, but serum lactose dehydrogenase (LDH) was elevated in 55% (22/39) of the patients. Abnormal positron emission tomography and/or computed tomography scans in 20/37 subjects mostly highlighted soft tissue or lymph nodes. Disease progression was associated with extensive ulcerated lesions resulting in 27 deaths including complications of hemophagocytic syndrome (4) and cerebral nervous system involvement (3). Median survival time from diagnosis was 31 months. Skin biopsies varied from a pagetoid pattern to purely dermal or panniculitic infiltrates composed of intermediate-sized lymphocytes with tissue evidence of cytotoxicity. The most common immunophenotype was CD3+/CD4⁻/CD5⁻/CD8⁻/BF1⁻/γ-M1+/TIA-1+/granzyme-B+/CD45RA-/CD7-, and 4 cases were Epstein-Barr virus positive. This is the largest study to date of cutaneous γδ T-cell lymphomas and demonstrates a variety of clinical and pathologic presentations with a predictable poor outcome.
Department of Dermatology and Pathology, Northwestern University Feinberg Medical School, Chicago, IL 60091, USA. j-guitart@northwestern.edu
Joan
J
Guitart
Dennis D
DD
Weisenburger
Antonio
A
Subtil
Ellen
E
Kim
Gary
G
Wood
Madeleine
M
Duvic
Elise
E
Olsen
Jacqueline
J
Junkins-Hopkins
Steve
S
Rosen
Uma
U
Sundram
Doina
D
Ivan
M Angelica
MA
Selim
Laura
L
Pincus
Janyana M D
JM
Deonizio
Mary
M
Kwasny
Youn H
YH
Kim
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001932:Brain Neoplasms / Q000453:epidemiology / Q000473:pathology; D015897:Comorbidity; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008198:Lymph Nodes / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000453:epidemiology / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis* / Q000378:metabolism / Q000401:mortality; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism*; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000401:mortality; D013602:T-Lymphocytes, Cytotoxic / Q000378:metabolism / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D014481:United States / Q000453:epidemiology
D016692:Receptors, Antigen, T-Cell, gamma-delta; D007770:L-Lactate Dehydrogenase