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Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma (CTCL) is a type of non-Hodgkin lymphoma of the skin in which T-cells (part of the immune system) grow in an uncontrolled way. Mycosis fungoides is the most common type of CTCL and tends to be slow growing and usually only affects the skin. Sezary syndrome is a less common but more advanced type of CTCL characterised by redness (erythroderma) in large areas of the skin, swollen lymph nodes, and abnormal lymphocytes circulating in the blood.

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    MeSH term: Lymphoma, T-Cell, Cutaneous
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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Kubicki SL, Park KE, Aung PP, Duvic M
Complete Resolution of Primary Cutaneous Anaplastic Large Cell Lymphoma With Topical Imiquimod
J Drugs Dermatol. 2019; 18(5):460-462 [PubMed] Related Publications
Primary cutaneous anaplastic large cell lymphoma (pc-ALCL) is a CD30+ subtype of cutaneous T-cell lymphoma. It typically has a very favorable prognosis; however, traditional treatment can be expensive, invasive, and associated with significant adverse events. Imiquimod is a topical toll-like receptor approved by the Food and Drug Administration (FDA) for genital warts, actinic keratosis, and primary superficial basal cell carcinoma. In previous case reports, imiquimod has been shown to be effective against pc-ALCL. We present a case of complete resolution of pc-ALCL within 8 weeks with topical imiquimod and review the current literature. J Drugs Dermatol. 2019;18(5):460-462.

Hong CH, Lin SH, Lee CH
CCL21 Induces mTOR-dependent MALAT1 Expression, Leading to Cell Migration in Cutaneous T-Cell Lymphoma.
In Vivo. 2019 May-Jun; 33(3):793-800 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Mycosis fungoides (MF) is indolent, but may disseminate to leukemia. We reported that C-C motif chemokine ligand 21 (CCL21) is associated with MF invasion and progression. Metastasis-associated lung adenocarcinoma transcript 1 (MALAT1), a long noncoding RNA, is associated with several cancer types, however, how it interacts with CCL21 to regulate MF progression, remains unclear.
MATERIALS AND METHODS: Expression of long noncoding RNAs MALAT1, antisense noncoding RNA in the INK4 locus (ANRIL), Hox antisense intergenic RNA (HOTAIR), highly up-regulated in liver cancer RNA (HULC), and leukemia-associated non-coding insulin-like growth factor 1 receptor activator RNA 1 (LUNAR1) in tissues from MF was studied using polymerase chain reaction and RNA interference in MF cell line MyLa were used to address this question.
RESULTS: Expression of MALAT1 was selectively increased in MF tissues. C-C Chemokine receptor type 7 (CCR7) expression was found to be increased in MyLa cells. CCL21 was found not only to mediate migration, but also to enhance MALAT1 and mammalian target of rapamycin (mTOR) activation in MyLa cells. Knockdown of MALAT1 abrogated CCL21-mediated migration, but not mTOR activation. In contrast, mTOR inhibition reduced CCL21-mediated migration and MALAT1 expression.
CONCLUSION: CCL21 induced mTOR activation in MyLa cells, followed by expression of MALAT1, causing cell migration. MALAT1 and mTOR are potential therapeutic targets for MF.

Wei S, Huang W, Xu X
Pagetoid reticulosis positive for cytotoxic CD20 and CD30: A case report.
Indian J Pathol Microbiol. 2019 Apr-Jun; 62(2):316-318 [PubMed] Related Publications
Pagetoid reticulosis is an indolent cutaneous T-cell lymphoma and presents as erythema or plaque with a well-defined border on the distal areas of the extremities. Immunophenotypic studies show that in most cases, neoplastic lymphocytes are positive for CD4, whereas CD20 and CD30 double positivity was rarely reported. In this paper, we report an 80-year-old woman who presented with erythema on the extremities for 3 years. Skin biopsy on the right forearm was performed. Histopathologically, the erythematous lesions were characterized by atypical lymphocytes with significant epidermotropism. Immunohistochemical staining showed high proliferation as evidenced by high Ki-67 index and that the tumor cells were positive for CD20 and CD30 but negative for CD7 and CD56. The patient was treated with one cycle of radiotherapy and is currently doing well.

Soltan MY, Sumarni U, Assaf C, et al.
Key Role of Reactive Oxygen Species (ROS) in Indirubin Derivative-Induced Cell Death in Cutaneous T-Cell Lymphoma Cells.
Int J Mol Sci. 2019; 20(5) [PubMed] Free Access to Full Article Related Publications
Cutaneous T-cell lymphoma (CTCL) may develop a highly malignant phenotype in its late phase, and patients may profit from innovative therapies. The plant extract indirubin and its chemical derivatives represent new and promising antitumor strategies. This first report on the effects of an indirubin derivative in CTCL cells shows a strong decrease of cell proliferation and cell viability as well as an induction of apoptosis, suggesting indirubin derivatives for therapy of CTCL. As concerning the mode of activity, the indirubin derivative DKP-071 activated the extrinsic apoptosis cascade via caspase-8 and caspase-3 through downregulation of the caspase antagonistic proteins c-FLIP and XIAP. Importantly, a strong increase of reactive oxygen species (ROS) was observed as an immediate early effect in response to DKP-071 treatment. The use of antioxidative pre-treatment proved the decisive role of ROS, which turned out upstream of all other proapoptotic effects monitored. Thus, reactive oxygen species appear as a highly active proapoptotic pathway in CTCL, which may be promising for therapeutic intervention. This pathway can be efficiently activated by an indirubin derivative.

Alpdogan O, Kartan S, Johnson W, et al.
Systemic therapy of cutaneous T-cell lymphoma (CTCL).
Chin Clin Oncol. 2019; 8(1):10 [PubMed] Related Publications
Cutaneous T-cell lymphomas (CTCLs) are a heterogeneous and relatively rare family of extranodal non-Hodgkin's lymphomas (NHL) that are primarily localized to the skin. Most patients present with cutaneous patches, plaques, tumors, more rarely with erythroderma. The type of skin lesions and the surface extent of skin involvement, as well as the presence of extracutaneous disease, are the most important prognostic factors in patients with CTCL. Patients with early-stage disease can be effectively treated with skin-directed therapies only. As the disease progresses, systemic therapy often becomes necessary. In this review, we will provide an overview of the systemic treatment options for CTCL, including mechanism of action, efficacy, side effects, and discuss current principles for rational combination therapy and sequential use. Systemic therapy strategies have been evolving with the recent FDA approval of new monoclonal antibodies such as brentuximab vedotin and mogamulizumab to established drugs, such as retinoids, interferons (IFNs), and HDAC inhibitors. Numerous new agents are currently being studied in clinical trials. Identifying the genetic, molecular, and immunologic features of CTCL that are associated with disease progression, drug-resistance, and immune impairment, will optimize the utilization of existing therapies, and facilitate the development of new ones.

Hossain C, Jennings T, Duffy R, et al.
The histological prevalence and clinical implications of folliculotropism and syringotropism in mycosis fungoides.
Chin Clin Oncol. 2019; 8(1):6 [PubMed] Related Publications
BACKGROUND: Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, remains a challenge for clinicians to stage and manage. Classically, MF is determined through histopathologic evidence of a neoplastic infiltrate within the epidermis. In certain patients, however, the infiltrate extends into the hair follicles and sweat glands. The objective of this study is to determine the utility of expanding the analysis of histopathology reports to include the reporting of folliculotropism and syringotropism.
METHODS: This is a prospective, observational study conducted in a single facility from 2012-2018. All patients with MF, excluding those treated at this facility for less than six months with the exception of those with incomplete pruritus documentation, or absence of initial biopsy analysis were studied. Modified severity weighted assessment tool (mSWAT) quantified body surface area and treatments attempted per patient were continuously charted. Patients were surveyed for presence and degree of pruritus and pain. Evaluation of these parameters were charted at the initial patient visit and correlated with their primary biopsy for presence or absence of folliculotropism and syringotropism.
RESULTS: Of the 87 patients examined, 70 patients (80%) exhibited syringotropism in their original biopsy and 68 patients (78%) exhibited folliculotropism. Presence of both findings concurrently was present in 56 patients (64.4%), while neither finding was present in 5 patients (5.8%). The singular finding of folliculotropism was found in 12 patients (13.8%), while the singular finding of syringotropism was exhibited in 14 patients (16.1%). A significant association between the presence of folliculotropism and pruritus was established (P=0.043, α=0.05). The general trend towards increase in mSWAT score and pruritus in patients in regard to the mean and median values suggest that increasing the sample population of the study might yield a significant value in the future.
CONCLUSIONS: These presentations are more prevalent than previously recognized and have findings indicative of more severe disease. We propose that MF histopathology reports document the presence of folliculotropism and syringotropism and that these findings be added to the NCCN guidelines as they may aid in predicting severity and progression risk.

Wei H, Liu R, Guo X, et al.
miRNA‑135a regulates Hut78 cell proliferation via the GATA‑3/TOX signaling pathway.
Mol Med Rep. 2019; 19(3):2361-2367 [PubMed] Related Publications
The present study investigated the role of microRNA‑135a (miR‑135a) in cutaneous T‑cell lymphoma (CTCL) proliferation. Compared with the normal T lymphocyte control cell line, the mRNA and protein levels of GATA binding protein 3 (GATA‑3) were markedly increased in the Hut78 cell line and miR‑135a was markedly decreased (P<0.05). Based on bioinformatics, the target gene of miR‑135a was identified as GATA‑3. Dual luciferase and pre‑miR‑135a assays showed that miR‑135a regulated the translation of GATA‑3. In addition, the overexpression of miR‑135a mimics decreased the protein levels of GATA‑3 and thymocyte selection‑associated high mobility group box (TOX). The substantially increased mRNA and protein levels of GATA‑3 may be associated with the downregulation of miR‑135a, leading to T‑cell deregulation and proliferation through GATA‑3/TOX regulation and subsequently causing CTCL.

Fernandez-Flores A, Cassarino DS
Plasmacytoid dendritic cells in granulomatous variant of mycosis fungoides.
J Cutan Pathol. 2019; 46(5):335-342 [PubMed] Related Publications
INTRODUCTION: Granulomatous mycosis fungoides (MF) is a rare variant in which granulomas are associated with other typical signs of MF. Its prognosis is worse than that of classical MF. Plasmacytoid dendritic cells (PDCs) are a subset of interferon-producing dendritic cells that link the innate and the adaptative immune responses. They have also been related to tolerance to certain tumors such as melanoma.
MATERIALS AND METHODS: In this article, we examined for the presence of CD123+ PDC in six cases of granulomatous MF from our archives.
RESULTS: We found clusters of 10 or more positive cells in three of six cases of granulomatous MF (two women and a man, in their sixth and seventh decade). Although in two of these three cases the granulomatous response was extensive, in the other, it only represented 10% of the infiltrate of the biopsy. In all three cases, the granulomas were epithelioid, sarcoidal type.
CONCLUSIONS: CD123+ PDC can be identified in granulomatous MF. The pathogenic and prognostic role of this finding requires further clarification.

Dadoo AS, Steenkamp I
Rapid progression of immune dysregulation in an HIV-infected patient with Sézary syndrome.
Dermatol Online J. 2019; 25(1) [PubMed] Related Publications
A 44-year-old man known to have human immunodeficiency virus (HIV) infection presented to our clinic with erythroderma, generalized lymphadenopathy, and cutaneous nodules and tumors. After a series of investigations, we confirmed that he had Sézary syndrome. In this paper we describe the immune alterations that occur in both Sézary Syndrome and HIV infection and how these changes together resulted in rapid and overwhelming immune dysregulation in our patient.

Abdolkarimi B, Sepaskhah M, Mokhtari M, et al.
Hypo-pigmented mycosis fungoides is a rare malignancy in pediatrics.
Dermatol Online J. 2018; 24(11) [PubMed] Related Publications
Hypopigmented mycosis fungoides (HMF) is an uncommon form of cutaneous T-cell lymphoma. It can be seen in children and is usually mistaken for eczema, vitiligo, or progressive macular hypomelanosis, clinically and histopathologically. We present a boy with HMF confirmed by histopathology. The patient had a course with slow clinical progression without Sezary syndrome.

Shalabi D, Bistline A, Alpdogan O, et al.
Immune evasion and current immunotherapy strategies in mycosis fungoides (MF) and Sézary syndrome (SS).
Chin Clin Oncol. 2019; 8(1):11 [PubMed] Related Publications
Mycosis fungoides (MF) and Sézary syndrome (SS) are two well-characterized skin limited and leukemic subtypes of cutaneous T-cell lymphoma (CTCL), respectively. Progressive global immune dysfunction and a multitude of specific immunological abnormalities have long been recognized as features of MF and SS. Therefore, a variety of immune-based therapies have been explored and used in the clinic for decades in the attempt to restore the immune imbalance in these malignancies. With recent advances in the development of novel immunotherapies in cancer treatment, new treatment modalities have emerged to complement the existing repertoire. Herein, we provide a comprehensive review of immune evasive mechanisms in MF/SS and summarize the established and emerging immunotherapies for these malignancies. We explain the underlying mechanisms of these immune-based therapies and derive recommendation from results of major clinical trials.

Kartan S, Johnson WT, Sokol K, et al.
The spectrum of CD30+ T cell lymphoproliferative disorders in the skin.
Chin Clin Oncol. 2019; 8(1):3 [PubMed] Related Publications
Primary cutaneous CD30+ T cell lymphoproliferative disorders (pcCD30+ T cell LPDs) are a spectrum of pre-malignant to frankly neoplastic lymphoproliferations that comprise lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL), and borderline lesions. Although the atypical T cells that are the hallmark of these disorders share the expression of CD30, as the identifying marker, the clinical presentation, histological features and clinical course are vastly different. Furthermore, histopathologic features of pcCD30+ T cell LPDs may overlap with other cutaneous and systemic lymphomas. While most pcCD30+ T cell LPDs have an excellent prognosis, systemic lymphomas typically have a poorer outcome. CD30 has now been shown to be a reliable therapeutic target in pcCD30+ T cell LPDs. This review will emphasize the structure and function of CD30, along with the clinical and pathological spectrum of pcCD30+ T cell LPDs. It will also highlight other CD30+ lymphoproliferations that must be differentiated by careful clinical and pathological correlation.

Menter T, Ballova V, Caspar C, et al.
ALK-negative anaplastic large cell lymphoma arising in the thrombus of an aortic prosthesis preceeded by clonally related lymphomatoid papulosis.
Virchows Arch. 2019; 474(6):763-767 [PubMed] Related Publications
We report on a 73-year-old male patient with recurrent thrombosis of his infrarenal aortic prosthesis. Histologically, the thrombus contained cells of an ALK-negative anaplastic large cell T cell lymphoma (ALCL). Imaging studies were negative for other lymphoma manifestations; however, 3 months before, the patient had developed skin lesions consistent with lymphomatoid papulosis type A (LypA) which were clonally related to the ALCL. Due to recurrent thrombosis of larger peripheral arteries with the presence of ALCL cells in the thrombi, the patient is now referred to systemic chemotherapy. We present the first case of ALCL manifesting in the thrombus of an aortic prosthesis. This case shows similarities to the well-established entity of breast implant-associated ALCL and anecdotal reports of ALCL occurring at the site of foreign material implants. These cells show a peculiar propensity to aggregate in vessels and thrombi, known primarily from subtypes of diffuse large B cell lymphomas associated with chronic inflammation.

Aria AB, Wilmas K, Kim EJ, et al.
The development of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder at the site of a melanoma excision scar.
Dermatol Online J. 2018; 24(9) [PubMed] Related Publications
Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell lymphoma (CTCL), representing 2% of all primary cutaneous lymphomas. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear. We present the first case of PCSM-LPD, to our knowledge, arising at a past melanoma excision site. A 72-year-old woman with a past medical history significant for melanoma-in-situ excised 36 years ago presented to our clinic for evaluation of a single, erythematous plaque of the posterior arm within a melanoma excision scar. A biopsy was performed, revealing PCSM-LPD. Reports of the development of other T-cell lymphoproliferative disorders after prior skin trauma such as chemical burns, thermal injury, and mechanical trauma exist in the literature. Physicians should be aware of the possibility of the appearance of T-cell lymphoproliferative disorders at the site of scars or prior trauma with a time lag of months to years.

Wiznia LE, Cohen JM, Beasley JM, et al.
Lymphomatoid papulosis.
Dermatol Online J. 2018; 24(12) [PubMed] Related Publications
Lymphomatoid papulosis is often regarded as a low-grade variant of cutaneous T cell lymphoma (CTCL). Given the excellent long-term prognosis, recent consensus guidelines indicate that patients can be monitored off therapy. We report a case of a 67-year-old man who presented with lymphomatoid papulosis, with necrotic papules that have been intermittently present for over forty years.

Feng H, Beasley J, Meehan S, Liebman TN
Folliculotropic mycosis fungoides.
Dermatol Online J. 2018; 24(12) [PubMed] Related Publications
Folliculotropic mycosis fungoides (MF) is a distinct subset of cutaneous T cell lymphoma (CTCL). The disease is typically marked by an aggressive course and is often recalcitrant to skin-direct therapy. We report a case of an 83-year-old woman with folliculotropic MF characterized by erythematous, scaly plaques on the forehead along with poliosis and alopecia of the right medial eyebrow.

Martinez-Escala ME, Amin SM, Sable KA, et al.
Multiple melanocytic nevi restricted to mycosis fungoides patches in pediatric and young-adult patients. The potential role of local immunosuppression.
Pediatr Dermatol. 2019; 36(2):232-235 [PubMed] Related Publications
We described the development of multiple melanocytic nevi within long-standing MF patches in four young patients. Mycosis fungoides (MF) patches are characterized by a regulatory-like cytokine profile leading to local immune suppression. The proliferation of nevomelanocytes is regulated by cellular senescence mechanisms mediated by immune system. The immunosuppressive effect of MF infiltrate in conjunction with the systemic effect of treatments may play a specific role in the nevomelanogenesis of the patients herein described.

Nanni L, Morigi A, Casadei B, et al.
A case report of the long treatment experience of a Sézary syndrome responder patient: 16 years through all the systemic and innovative therapies.
Hematol Oncol. 2019; 37(2):202-204 [PubMed] Related Publications
Existing therapies for Sézary syndrome (SS) are limited in efficacy and in disease control, and patients have very poor prognosis. Here, we report a case report of a patient who has a 16-year history of SS and related treatments (both standard and experimental). In particular, two drugs, one conventional (gemcitabine) and one experimental (mogamulizumab), were able to induce long lasting response. Patient refused to undergo allogeneic stem cell transplantation. After eleven lines of therapeutic approaches, the patient is in very good partial response and free of therapy at the latest available follow-up.

Chen SJT, Tse JY, Harms PW, et al.
Utility of CD123 immunohistochemistry in differentiating lupus erythematosus from cutaneous T cell lymphoma.
Histopathology. 2019; 74(6):908-916 [PubMed] Related Publications
AIMS: Histopathological overlap between lupus erythematosus and certain types of cutaneous T cell lymphoma (CTCL) is well documented. CD123
METHODS AND RESULTS: Skin biopsies of cutaneous lupus erythematosus (CLE, n = 18), lupus erythematosus panniculitis (LEP, n = 17), mycosis fungoides (MF, n = 25) and subcutaneous panniculitis-like T cell lymphoma (SPTCL, n = 9) were retrospectively reviewed and immunostained with CD123. Percentage, distribution and clustering of CD123
CONCLUSIONS: CD123 immunostaining is helpful in differentiating CLE from MF and LEP from SPTCL, but should be interpreted in conjunction with clinicopathological features and other ancillary studies to ensure accurate diagnosis.

Nikolaenko L, Zain J, Rosen ST, Querfeld C
CD30-Positive Lymphoproliferative Disorders.
Cancer Treat Res. 2019; 176:249-268 [PubMed] Related Publications
Primary cutaneous CD30-positive lymphoproliferative disorders (CD30+ LPD) encompass lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma (pcALCL), and borderline lesions [1]. CD30+ LPD are the second most common cutaneous T-cell lymphomas (CTCL) after mycosis fungoides (MF) and represent approximately 25% of all CTCL cases [2]. Their common phenotypic hallmark is an expression of the CD30 antigen, a cytokine receptor belonging to the tumor necrosis factor (TNF) receptor superfamily. Both LyP and pcALCL show numerous clinical, histological and immunophenotypic variants, and generally have an indolent course with a favorable prognosis. Overlapping features of LyP and pcALCL with other CD30+ T-cell lymphomas, inflammatory, and/or infectious conditions emphasize the importance of careful clinicopathologic correlation and staging.

Querfeld C, Zain J, Rosen ST
Primary Cutaneous T-Cell Lymphomas: Mycosis Fungoides and Sezary Syndrome.
Cancer Treat Res. 2019; 176:225-248 [PubMed] Related Publications
Mycosis fungoides and Sézary syndrome are the most common subtypes of all primary cutaneous lymphomas and represent complex diseases that require a multidisciplinary assessment by dermatologists, oncologists, and pathologists. Staging and work-up are critical to guarantee an optimal treatment plan that includes skin-directed and/or systemic regimens depending on the clinical stage, tumor burden, drug-related side effect profile, and patient comorbidities. However, there is no cure and patients frequently relapse, requiring repeated treatment courses for disease control. The study of the tumor microenvironment and molecular mechanisms of these rare neoplasms may assist in the development of new immune therapies providing promising treatment approaches tailored for patients with relapse/refractory disease.

van der Weyden C, McCormack C, Lade S, et al.
Rare T-Cell Subtypes.
Cancer Treat Res. 2019; 176:195-224 [PubMed] Related Publications
There are a number of rare T-cell lymphoma subtypes that may be encountered in clinical practice. In recent years, improved immunohistochemical techniques and molecular tumor profiling have permitted refinement of some of the diagnostic categories in this group, as well as the recognition of distinct conditions not previously well elucidated. In this chapter, we cover the diagnostic and clinical features of some of the more common of these conditions, including subcutaneous panniculitis-like T-cell lymphoma, cutaneous gamma-delta T-cell lymphoma, enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, CD4-positive small/medium T-cell lymphoproliferative disorder, and acral CD8-positive T-cell lymphoma. Given the rarity of these conditions, optimal treatments approaches are not always well established, not least as data from large-scale clinical trials are lacking. In this chapter, we aim to provide a summation of current thinking around best treatment, as well as highlighting some controversies in the management of these diagnoses.

Miccio JA, Wilson LD, Kann BH, et al.
Cutaneous T-Cell Lymphoma: Trends in Radiation Doses and Patterns of Care 2004-2015.
Anticancer Res. 2019; 39(1):253-259 [PubMed] Related Publications
BACKGROUND AND AIM: Radiotherapy is an effective treatment for cutaneous T-cell lymphoma (CTCL). Since 2009, studies have advocated for low-dose radiotherapy (<30 Gy) given it results in similar response rates and less toxicity compared to higher doses (≥30 Gy). We aimed to see if low-dose radiotherapy has been adopted on a national scale in the USA.
MATERIALS AND METHODS: A total of 11,292 adult patients with CTCL were identified in the National Cancer Database. Logistic regression models were created to evaluate predictors for use of low-dose radiotherapy.
RESULTS: A minority of patients received low-dose radiotherapy (22.4%). The annual percentage of patients receiving low-dose radiotherapy increased from 17.2% in 2009 to 38.4% in 2015. High-volume facilities were associated with use of low-dose radiotherapy (5th quintile vs. bottom two quintiles, odds ratio(OR)=1.76, 95% confidence interval(CI)=1.22-2.54, p=0.003).
CONCLUSION: Although the radiotherapy dose administered is decreasing, most patients with CTCL are still receiving doses that may be higher than needed to palliate their disease effectively.

Stewart JR, Desai N, Rizvi S, et al.
Alemtuzumab is an effective third-line treatment versus single-agent gemcitabine or pralatrexate for refractory Sézary syndrome: a systematic review.
Eur J Dermatol. 2018; 28(6):764-774 [PubMed] Related Publications
The efficacy of alemtuzumab for the treatment of refractory Sézary syndrome (SS) versus other third-line agents such as pralatrexate and gemcitabine is poorly characterized. To elucidate the effectiveness of alemtuzumab versus other third-line options for the treatment of refractory SS, we conducted a meta-analysis of existing data. A systematic review was performed in March 2017 based on a search using Ovid-MEDLINE

Fujita Y, Natsuga K, Manabe O, et al.
A Nodular Lesion of the Foot Detected by 18F-FDG PET/CT in Mycosis Fungoides: A Plantar Wart.
Clin Nucl Med. 2019; 44(3):244-245 [PubMed] Related Publications
A 34-year-old Japanese woman presented with widespread scaly erythema that had enlarged over 2 years. A skin biopsy revealed the diagnosis of mycosis fungoides (patch stage, T1b N0 M0 B0), a most frequent cutaneous T-cell lymphoma. F-FDG PET/CT scan unexpectedly showed intense uptake on the left sole, which suggested a tumorous mycosis fungoides lesion (SUVmax = 6.2). Careful examination revealed the mass to be a typical plantar wart of 2 cm in diameter that the patient had not recognized. With repeated cryotherapy, the wart disappeared in 6 months, and follow-up F-FDG PET/CT showed no abnormal uptake on the left sole.

Magro CM, Olson LC, Momtahen S
Post-thymic CD4 positive cytotoxic T cell infiltrates of the skin: A clinical and histomorphologic spectrum of the unique CD4 positive T cell of immunosenescence.
Ann Diagn Pathol. 2019; 38:99-105 [PubMed] Related Publications
T cell lymphoproliferative disorders that arise in the skin are mainly derived from post thymic T cells most commonly of CD4 subset. Human CD4 positive T cells are dynamic exhibiting phenotypic and functional malleability. For example, with repetitive antigen exposure most commonly associated with age, CD4 positive T cells acquire a cytotoxic phenotype. The authors present four cases characterized by cutaneous infiltrates of cytotoxic CD30 negative CD4 positive T cells in the skin; three cases were forms of malignant lymphoma other than mycosis fungoides and one case was a reactive lymphomatoid photodermatosis associated with underlying collagen vascular disease. The three patients with lymphoma were adult men, two above 50 years of age and all with disseminated cutaneous disease. One of these patients whose biopsy showed a large cell morphology succumbed to the disease while one patient with localized disease responded to local radiation. In all three cases there was a nodular and diffuse pan-dermal infiltrate which was predominated by non-cerebriform atypical lymphocytes ranging from small to intermediate sized cells in two cases to a large cell dominant morphology in one case. The biopsies showed some degree of epidermotropism, and in one case it was striking. Neoplastic cells were positive for CD4, and at least one cytotoxic protein (i.e. granzyme and/or TIA). CD56, CD57 or CD30 were negative. In addition, CD28, the naïve T cell marker, was negative. Based on the few cases reported herein, one might suggest that the prognosis mirrors that seen in other forms of cutaneous T cell lymphoma with mature small cell dominant infiltrates exhibiting an indolent pattern while a CD30 negative large cell T cell lymphoma would be expected to demonstrate an aggressive clinical course.

Zoumberos NA, McMullen E, Wang L, et al.
Merkel cell carcinoma arising in association with cutaneous T-cell lymphoma: A potential diagnostic pitfall.
J Cutan Pathol. 2019; 46(3):199-203 [PubMed] Related Publications
Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine carcinoma with increased prevalence in patients with immunosuppression or B-cell neoplasms. To the best of our knowledge, an association with cutaneous T-cell lymphoma (CTCL) has not been previously described. In this report, we present two cases of MCC arising in the setting of CTCL. The first case was a female during her 70s with previously diagnosed stage IVA1 Sezary syndrome. Biopsy of a scaly patch showed two distinct abnormal cell populations. The first population consisted of hyperchromatic dermal and epidermotropic lymphocytes, expressing CD3 and CD4 with diminished CD7. The second population consisted of intraepidermal clusters of larger atypical cells that expressed synaptophysin, neurofilament, CK20, and Merkel cell polyomavirus transcript. The combination of findings was consistent with intraepidermal MCC in a background of CTCL. Excision showed residual intraepidermal MCC without dermal involvement. The second case was a male during his 50s with a longstanding history of mycosis fungoides, who presented with a new lesion on his right thigh. Biopsy and excision showed dermal MCC without secondary involvement by CTCL. Our cases show that MCC may rarely occur in the setting of T-cell lymphoma, and that intraepidermal MCC may mimic epidermotropic T-cells.

Kim EJ, Aria AB, Wilmas K, et al.
Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder in a pediatric patient successfully treated with low-dose radiation.
Pediatr Dermatol. 2019; 36(1):e23-e26 [PubMed] Related Publications
Primary cutaneous CD4+ small- to medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell proliferation with the average age of diagnosis of 54 years. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear, with < 10 pediatric cases reported. A 13-year-old boy presented to our clinic with a raised tumor with PCSM-LPD histology and was successfully treated with ultra-low-dose radiation therapy. While no standard of care has been established for pediatric PCSM-LPD, this report represents an example of achieving remission in a pediatric tumor with minimal potential for therapy-related long-term toxicity.

Ma LL, Han SX, Fang MY
Primary cutaneous anaplastic large cell lymphoma arising from a long-standing erythema nodosum: A case report.
Medicine (Baltimore). 2018; 97(49):e13211 [PubMed] Free Access to Full Article Related Publications
RATIONALE: Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare subtype of non-Hodgkin lymphoma, which is rarely associated with erythema nodosum (EN).
PATIENT CONCERNS: A 57-year-old woman complained of recurrent rashes involving her abdomen, back, upper and lower limbs for over 20 years, with severity in symptoms for 2 weeks.
DIAGNOSES: The first skin biopsy was performed in 2011 in another hospital and she was diagnosed idiopathic EN. The second skin biopsy was performed in 2014 and she was diagnosed as pcALCL with stage IA.
INTERVENTIONS: She was treated with oral prednisone, cyclosporine, and thalidomide.
OUTCOMES: One month later, the ulcerative lesion was decreased in size and became smooth and the patient achieved partial remission. She is still under treatment and has been monitored closely for 4 years.
LESSONS: The case suggested that stimulation of inflammation in the skin lesions for a long period might be related to clonal transformation into pcALCL and hence should be closely monitored. Immunosuppressive treatment may be effective and safe for patients with pcALCL at early stages.

Nakajima R, Miyagaki T, Kamijo H, et al.
Decreased progranulin expression in Mycosis fungoides: a possible association with the high frequency of skin infections.
Eur J Dermatol. 2018; 28(6):790-794 [PubMed] Related Publications
Progranulin (PGRN) is a multi-functional protein known to be involved in diverse biological processes, including tumourigenesis, anti-inflammation, and anti-infection. PGRN expression in sera or tissues is elevated in a variety of malignancies and is associated with poor prognosis. However, it remains to be determined whether PGRN is involved in Mycosis fungoides (MF). To investigate the roles of PGRN in MF. Serum PGRN levels were measured in patients with MF and normal controls by enzyme-linked immunosorbent assay. PGRN expression in MF and normal skin was examined by quantitative reverse transcription polymerase chain reaction and immunohistochemistry. Moreover, we analysed correlations between the expression levels of PGRN and antimicrobial peptides in lesional skin. PGRN levels were significantly lower in sera of MF patients than those of normal controls. PGRN mRNA levels in lesional skin of MF were also significantly decreased. Immunohistochemical staining revealed that PGRN was expressed in epidermal keratinocytes of normal controls, however, PGRN expression in epidermal keratinocytes was also weaker in MF skin. Furthermore, significant inverse correlations were identified between PGRN and antimicrobial peptide mRNA expression. These results suggest that low PGRN expression may contribute to the frequent occurrence of skin infections in patients with MF.

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