scholarly journals Erythrodermic CD4/CD8 Double-Negative Mycosis Fungoides: A Case Report

2021 ◽  
pp. 256-261
Author(s):  
Monira Abdullah Alnasser ◽  
Nour Marwan AlKhawajah ◽  
Nada Ghazi AlQadri ◽  
Asem Mustafa Shadid ◽  
Fahad M. Alsaif

Cutaneous T-cell lymphoma (CTCL) describes a group of lymphoproliferative disorders characterized by localization of neoplastic T lymphocytes to the skin. Mycosis fungoides (MF) represents the most common type of CTCL and accounts for ∼60% of all primary cutaneous lymphomas. Apart from the classic type of MF, many clinical and histopathologic variants have been described. The malignant lymphocytes in MF are usually CD3, CD4 and CD45RO positive and CD8 negative. An unusual immunohistochemical profile of a CD4-negative and CD8-positive mature T-cell phenotype has been reported in a minority of patients; up to 20% of early-stage MF demonstrates a CD8-positive phenotype. There are only a few cases of a double-negative CD4/CD8 MF phenotype reported in the literature. We present the case of a 60-year-old male presenting a double-negative CD4/CD8 MF phenotype.

2014 ◽  
Vol 7 ◽  
pp. CCRep.S15724 ◽  
Author(s):  
Akinsegun A. Akinbami ◽  
Bodunrin I. Osikomaiya ◽  
Sarah O. John-Olabode ◽  
Adewumi A. Adediran ◽  
Olajumoke Osinaike ◽  
...  

Mycosis fungoides (MF), also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. Cutaneous lymphomas are an uncommon, heterogeneous group of non-Hodgkin lymphomas (NHLs) of T- and B-cell origin where the skin is the primary organ of involvement. This is a case of a 60-year-old Nigerian woman, who was diagnosed and managed as a case of chronic dermatitis but further investigations confirmed a diagnosis of MF; she was thereafter managed with topical glucocorticoids/chemotherapy and improved on these treatments. We make a plea for better awareness of the disease among physicians and pathologists in Africa.


2015 ◽  
Vol 172 (6) ◽  
pp. 1637-1641 ◽  
Author(s):  
L. Bekel ◽  
G. Chaby ◽  
C. Lok ◽  
A. Dadban ◽  
D. Chatelain ◽  
...  

Blood ◽  
2003 ◽  
Vol 101 (9) ◽  
pp. 3407-3412 ◽  
Author(s):  
Jorge R. Toro ◽  
David J. Liewehr ◽  
Nina Pabby ◽  
Lynn Sorbara ◽  
Mark Raffeld ◽  
...  

The importance of αβ versus γδ T-cell subset antigen expression in the classification of peripheral T-cell lymphomas is still unclear. The objective of this study was to investigate the prognostic value of T-cell receptor–δ1 (TCRδ1) expression in primary cutaneous T-cell lymphomas. TCRδ1 cellular expression was assessed in skin biopsy specimens of 104 individuals with cutaneous T-cell lymphoma by immunohistochemistry. Both univariate (Kaplan-Meier) and multivariate (Cox regression) analyses were conducted to determine which variables (T-cell subtype, hemophagocytosis, histologic profile, age, sex, and adenopathy) were significantly associated with survival. Univariate analysis indicated that there was a statistically significant difference in survival between the patients with αβ cutaneous T-cell lymphoma and patients with γδ cutaneous T-cell lymphoma (P < .0001). There was also a statistically significant decrease in survival among patients who had subcutaneous involvement compared with patients who had epidermotropic and/or dermal involvement (P < .0001). Cox model analysis indicated that TCRδ1 expression was the factor that was most closely associated with decreased survival (P < .0001). Among those patients with cutaneous γδ T-cell lymphoma (n = 33), there was a trend for decreased survival for patients who had histologic evidence of subcutaneous fat involvement in comparison with patients who had epidermotropic or dermal patterns of infiltration (P = .067). No other prognostic factors were identified as having a notable association with outcome in this subgroup. TCRδ1 expression in primary cutaneous lymphomas is an independent prognostic factor associated with decreased survival.


Blood ◽  
2000 ◽  
Vol 95 (9) ◽  
pp. 2937-2942 ◽  
Author(s):  
Julia J. Scarisbrick ◽  
Alison J. Woolford ◽  
Robin Russell-Jones ◽  
Sean J. Whittaker

Previous cytogenetic studies of primary cutaneous T-cell lymphoma (CTCL) were based on limited numbers of patients and seldom showed consistent nonrandom chromosomal abnormalities. In this study, 54 tumor DNA samples from patients with CTCL were analyzed for loss of heterozygosity on 10q. Allelic loss was identified in 10 samples, all of which were from the 44 patients with mycosis fungoides (10/44 patients; 23%). Of the patients with allelic loss, 3 were among the 29 patients with early-stage myosis fungoides (T1 or T2) (3/29 patients; 10%), whereas the other 7 were among the 15 patients with advanced cutaneous disease (T3 or T4) (7/15 patients; 47%). The overlapping region of deletion was between 10q23 and 10q24. In addition, microsatellite instability (MSI) was present in 13 of the 54 samples (24%), 12 from patients with mycosis fungoides and 1 from a patient with Sezary syndrome. There was also an association between MSI and disease progression in patients with mycosis fungoides, with 6 of 15 (40%) patients with MSI having advanced cutaneous disease and only 6 of 29 (21%) having early-stage disease. Samples with allelic loss on 10q were analyzed for abnormalities of the tumor suppressor genePTEN (10q23.3). No tumor-specific mutations were detected, but homozygous deletion was found in 2 patients. Thus, we found loss of heterozygosity on 10q and MSI in advanced cutaneous stages of mycosis fungoides. These findings indicate that a tumor suppressor gene or genes in this region may be associated with disease progression. Furthermore, abnormalities of PTEN may be important in the pathogenesis of mycosis fungoides, but our data imply that this gene is rarely inactivated by small deletions or point mutations.


2006 ◽  
Vol 55 (2) ◽  
pp. 276-284 ◽  
Author(s):  
Emmilia Hodak ◽  
Michael David ◽  
Leah Maron ◽  
Adina Aviram ◽  
Ella Kaganovsky ◽  
...  

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