Aggressive GATA-3 Positive Primary Cutaneous Peripheral T-Cell Lymphoma – Not Otherwise Specified Masquerading as CD-4 Positive Small/Medium T-Cell Lymphoproliferative Disorder: Where is the Line of Delineation?
Year: 2025
Type: article
Abstract: Dear Editor, Primary cutaneous peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS) is a diagnosis of exclusion and accounts for less than 2% of cutaneous T-cell lymphomas (CTCLs).[1] GATA-3 and TBX21 are two molecular subgroups in peripheral T-cell lymphomas (PTCLs) that have been identified with poor overall survival and outcome.[2,3] These subgroups have been minimally investigated in PTCL-NOS. We present an 80-year-old female with a 10 days old, 5x3cm violaceous, infiltrated nodule on her left thigh [Figure 1a]. Biopsy [Figure 2a-d] showed diffuse dermal and perivascular infiltration of small- and medium-sized lymphocytes. Immunohistochemistry (IHC) was predominately T-cell phenotype (CD2+,CD3+,CD5+,CD7-) admixed with irregular B lymphocytes (CD20+). Dominant cells were helper cells (CD4+,PD-1+) [Figure 2b and d] and scarce focally grouped CD8+ cells. CD56/CD30 staining was negative. CD-4 positive small/medium T-cell lymphoproliferative disorder (CD4+PCSM-LPD) was diagnosed. After 3 weeks, a violaceous nodule appeared on the right gluteus [Figure 1d]. Biopsy [Figure 2e-h] revealed diffuse dermal infiltrate without epidermotropism, predominant T-cells with variable loss of markers (CD2+/, CD3+,CD5+,CD7+/-), and minimal CD20+ B lymphocytes. Infiltrate was predominantly CD4+ with variable and rare focal expression of PD-1 (unlike the previous biopsy). Rare cytotoxic CD8+, granzyme B+ cells were seen, and CD30/CD56 was negative, but Ki-67 was >80% [Figure 2e]. GATA-3 and LEF1 were positive [Figure 2g and h], but TBX21 was negative. Imaging revealed no pathological elements or non-lymphoid disease. PTCL-NOS (T2N0M0) was diagnosed. Lesions expanded over the course of 65 days [Figure 1b, c, e, f]. The Mini-CHOP protocol was not started due to cardiovascular-associated diseases and rapid deterioration of health. Gemcitabine was suggested, but the patient passed away before starting therapy.Figure 1: (a) Tumour on left upper leg – day 0; (b) Tumour on left upper leg – day 56; (c) Tumour on left upper leg – day 65; (d) Tumour on right gluteus – day 0; (e) Tumour on right gluteus – day 56; (f) Tumour on right gluteus – day 65Figure 2: (a-d) Left inguinal tumour and first biopsy (a) Haematoxylin–eosin staining x40; (b) CD4 x100; (c) CD8 ×100; (d) PD-1 x100; (e-h) right gluteus and second biopsy (e) haematoxylin–eosin staining ×100; (f) Ki-67 x100; (g) GATA3 x100; (h) LEF1 x100CD4+PCSM-LPD accounts for 6% of CTCL but is indolent with an excellent prognosis. PD-1 is used for differentiation[4] and is expressed in CD4+PCSM-LPD. Our PD-1 stain was positive and then negative after 1 month. In a retrospective analysis of PTCL-NOS, PD-1 was rarely expressed; only 12% tumour cells were in solitary/localised tumours.[5] In this study, the dominant type seen in solitary/localised PTCL-NOS was CD4-/CD8- (5/9;56%) and CD4+/CD8- (16/24;67%) in disseminated tumours.[5] Ki-67 was found to be lower in disseminated lesions (45%) than in solitary lesions (63%).[5] In our case, Ki-67 increased in the second biopsy to >80%. Wang et al.[2] reported GATA-3 was associated with a worse prognosis. Kempf et al.[5] noted that GATA-3 was >80% in all solitary diseases and 32% in disseminated diseases (≥T3). In localised (T2) diseases like our case, GATA-3 expression was only available in 1 case (20%) and that leaves an informational gap. Overall, in this study, GATA-3 did not correlate with the outcome (no influence on survival time) and better prognosis was associated with only small-to-medium-sized morphology but not age, gender, clinical stage, and CD4/CD8 phenotype.[5] GATA-3 could be useful as a potential stain in more benign and localised forms of CTCL, like our CD4+ PCSM-LPD, as rapidly aggressive progression to PTCL-NOS is possible and could potentially aid in the treatment decisions. This case adds more information about GATA-3 expression in T2 stage localised PTCL-NOS as limited information has been published. We hope our case will motivate more research on this topic with larger sample sizes. Abbreviations CD4+ PCSM-LPD - CD-4 positive small/medium T-cell lymphoproliferative disorder CHOP - Cyclophosphamide, doxorubicin-hydrochloride, vincristine sulfate, and prednisone CTCL - Cutaneous T-cell lymphomas IHC - Immunohistochemistry PTCL - Peripheral T-cell lymphoma PTCL-NOS - Primary cutaneous peripheral T-cell lymphoma – not otherwise specified (PTCL-NOS). Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Indian Journal of Dermatology
Institutions: Univerzitetski Klinički Centar Srbije, University of Belgrade
Language: English
Subfield: Dermatology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4409530064