Primary Cutaneous T-cell Research Articles

QoL-O-01 - Update on the development of EORTC/CLTF questionnaires to assess HRQOL in primary cutaneous T-cell and B-cell lymphomas: phase I

QoL-O-01 - Update on the development of EORTC/CLTF questionnaires to assess HRQOL in primary cutaneous T-cell and B-cell lymphomas: phase I

Abstract 4129: Descriptors of primary cutaneous follicular helper T-cell lymphoma (PCFHTCL): Analysis of a pooled database of a rare entity

Abstract Introduction: Primary Cutaneous Follicular Helper T-cell Lymphoma (PCFHTCL) is a rare cutaneous T-cell lymphoma that was recently described. PCFHTCL is comprised of small to medium lymphocytes that express CD4, CD19, Bcl6, PD1, ICOS, CXCR5, and CXCL13. While it tends to have mostly an indolent course, occasionally PCFHTCL may display a more aggressive pattern. We conducted this analysis to explore the key clinicopathologic characteristics of this rare and little-known entity. Methods: In order to study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 57 cases. Kaplan-Meier survival curves were constructed. Cox proportional-hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival and disease-free survival. Results: A total of 57 patients with confirmed PCFHTCL were identified. The median age was 57 years with a peak incidence between ages 53 and 64. There was a male predominance with an M:F ratio of 1.25. PCFHTCL commonly involved the head (54%), followed by the torso (24%) and limbs (12%). Sixteen percent of the lesions were solitary and mostly involved the head. The most common pattern was nodular/tumorous. Histologically, PCFHTCL involved mainly the dermis with occasional focal epidermotropism (27%), folliculotropism (43%), syringotropism (7%), and subcutaneous extension (14%). Extracutaneous involvement comprised lymph nodes (11%), bone marrow (4%), and the peripheral blood (2%). The median duration of symptoms prior to diagnosis was 5 months. The six-year disease-free survival is around 85%. Surgical resection alone resulted in durable remission in 40% of the cases. Recurrent disease was successfully salvaged with re-resection, radiation, and chemotherapy. While solitary lesions tended to have a better disease-free survival, it did not reach statistical significance. Conclusions: This study represents the largest cohort of this rare cutaneous T-cell entity. It describes the demographic and clinical patterns of disease presentation and provides a detailed update on the clinicopathologic features PCFHTCL. Citation Format: Philip A. Haddad, Dalia Hammoud, Kevin Gallagher. Descriptors of primary cutaneous follicular helper T-cell lymphoma (PCFHTCL): Analysis of a pooled database of a rare entity [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2022; 2022 Apr 8-13. Philadelphia (PA): AACR; Cancer Res 2022;82(12_Suppl):Abstract nr 4129.

Lack of PRAME Expression in Cutaneous T-Cell Lymphomas.

Cutaneous T-cell lymphomas (CTCLs) are rare tumors with no established markers that can reliably distinguish between benign and malignant lesions. Preferentially Expressed Antigen in Melanoma (PRAME) is a cancer/testis antigen that is found in many solid and hematologic malignancies. PRAME overexpression typically portends a poor prognosis and lower chemotherapeutic response. To date, no studies have established a role for PRAME in CTCL. An analysis was performed on 47 cases definitively diagnosed as CTCL: 25 cases of mycosis fungoides, 2 of Sezary syndrome, 5 of CD30+ lymphoproliferative disorder, 7 of primary cutaneous anaplastic large T-cell lymphoma, 3 of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder, 1 of subcutaneous panniculitis-like T-cell lymphoma, and 4 of angiocentric T-cell lymphoma. PRAME immunohistochemistry was completely negative in all cases. PRAME expression was not found in any CTCL subtypes, suggesting that the pathogenesis of CTCL is not mediated by PRAME. Further study is required to identify biomarkers that might aid in the diagnosis and prognostication of CTCLs.

Qol-O-06 - Development of EORTC/CLTF questionnaire to assess HRQOL in primary cutaneous T-cell and B-cell lymphomas: phase I

<h3>Background:</h3> Health-related quality of life (HRQoL) in patients with cutaneous lymphoma (CL) is negatively impacted by a large burden of disease symptoms including intractable itch, pain, discomfort and socially stigmatising cosmetic disfigurement which may adversely affect patient self-esteem and interpersonal relationships. There is currently no cure for CL and as such, accurate determination of patient HRQoL is of paramount importance. To date, the impact of both skin disease and of a cancer diagnosis on patient HRQoL in CL is assessed using separate, non-CL-specific questionnaires. A composite HRQoL questionnaire addressing all aspects of the disease is lacking. <h3>Methods:</h3> The EORTC Quality of Life Group questionnaire development guidelines were followed. For phase I, semi-structured interviews were conducted in 15 patients with early-stage mycosis fungoides (IA–IIA), 30 patients with late-stage mycosis fungoides/Sézary syndrome (IIB–IVB) (15 patients <3 years and >3 years from diagnosis) and 15 patients with cutaneous B cell lymphoma. Collaborators communicated during the patient recruitment to ensure balanced patient selection with respect to age, gender, stage, duration of the disease, and number of (previous) treatments. <h3>Results:</h3> Patients were recruited in 5 countries (6 centres) across Europe. So far, 62 patients participated (target 60) from each of the following representative patient groups: early MF (stage IA–IIA), late MF (stage IIB–IVB) and cutaneous B cell lymphoma. Data from patient interviews and questionnaires is being analyzed using basic quantitative analyses, including generation of descriptive statistics (e.g., %missing data, means and standard deviations, floor and ceiling effects, etc.), prevalence (number of patients who experienced each complaint i.e. who scored 2, 3 or 4, divided by the total number of patients who completed that item, multiplied by 100), priority ratings (number of patients and professionals who have given priority ratings to each item) and range of responses for each item. The data from the debriefing questions will be reported both quantitatively and qualitatively (in narrative form). <h3>Conclusion:</h3> Once completed, phase I issues will be operationalised into items and any new items created in a style and format compatible with the EORTC quality of life group translation guidelines (phase II). The preliminary module will be pre-tested in phase III followed by large-scale international field-testing in phase IV. A comprehensive HRQoL questionnaire (module) for patients with CL is being developed to more accurately measure assessment of the impact of HRQoL in patients with skin lymphomas.

Helicobacter pylori and skin disorders: a comprehensive review of the available literature.

Helicobacter pylori is a Gram-negative bacterium identified for the first time about 30 years ago and commonly considered as the main pathogenic factor of gastritis and peptic ulcer. Since then, it was found to be associated with several gastrointestinal and extra-gastrointestinal diseases. Helicobacter pylori is also associated with many skin disorders including, but not limited to, chronic urticaria, rosacea, lichen planus, atopic dermatitis, psoriasis, pemphigus vulgaris, vitiligo, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, primary cutaneous marginal zone B-cell lymphomas and cutaneous T-cell pseudolymphoma. Literature up to September 2020 shows that clear evidence exists only for some of the mentioned associations, while in the majority of cases, data appear contrasting. The aim of this review is to summarize the available studies on the topic and draw possible conclusions. Further clinical and laboratory studies are needed to assess the real plausibility and relevance of these associations, as well as the possible role of Helicobacter pylori with the underlying pathogenic mechanisms.

CD8+ Phenotype of Primary Cutaneous Small-Medium Pleomorphic T-Cell Lymphoproliferative Disorder

CD8+ Phenotype of Primary Cutaneous Small-Medium Pleomorphic T-Cell Lymphoproliferative Disorder

Clinical Remission in a 72-Year-Old Patient with a Massive Primary Cutaneous Peripheral T-Cell Lymphoma-NOS of the Eyelid, Following Combination Chemotherapy with Etoposide Plus COP

Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is the rarest subtype of primary cutaneous lymphoma, accounting for approximately 2% of cutaneous lymphomas. The rarity of primary cutaneous PTCL-NOS means that there is a paucity of data regarding clinical and histopathological features and its clinical course. This malignancy is an aggressive and life-threatening hematological malignancy that often presents mimicking other less severe plaque-like skin conditions. Due to the nonspecific nature of these lesions, CD4-positive cutaneous T-cell lymphoma (CTCL) is often misdiagnosed as either mycosis fungoides or Sezary syndrome. We describe a patient who presented with a large tumoral mass in the right frontal area, with involvement of the right upper eyelid and the ocular globe, causing loss of vision greatly impacting the quality of life. Biopsy revealed primary cutaneous PTCL-NOS, treated successfully with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) plus etoposide combination chemotherapy. As elderly patients are indicated to receive attenuated doses of chemotherapy, CHOP-based regimens represent viable options.

Primary cutaneous gamma-delta T-cell lymphoma masquerading as severe soft tissue infection

N/A- Case report

Primary Cutaneous Gamma Delta T Cell Lymphoma: A Clinicopathological Analysis

Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is a very uncommon and extremely aggressive tumor, and is described in the newly re-vised World health organization for research and treatment of cancer classification of cutaneous lymphomas. A 43-year-old male patient presented with a 4 months history of cutaneous lesion over upper lip, without plaque and any constitutional symptom. Histopathological examination of skin biopsy revealed infiltration of atypical lymphocytes with hyperchromatic irregular nuclei. Immunophenotyping pattern of skin biopsy was compatible with PCGD-TLC. It is a highly aggressive tumor resistant to chemotherapy, immunotherapy, and radiation therapy. The GDTCL is characterized by a worse prognosis with a median survival of 15 months. Early diagnosis is essential and aggressive therapy is necessary.

Disease site as a determinant of survival outcome in patients with primary cutaneous peripheral T-cell lymphoma: An analysis of 4,057 cases from the U.S. National Cancer Database

Disease site as a determinant of survival outcome in patients with primary cutaneous peripheral T-cell lymphoma: An analysis of 4,057 cases from the U.S. National Cancer Database

Primary Cutaneous Peripheral T-Cell Lymphoma in a Sporotrichoid Pattern: A Case Report

We present the extraordinary case of a 72-year-old man with a history of primary cutaneous peripheral T-cell lymphoma not otherwise specified (pcPTCL-NOS) previously controlled with topical agents who developed tumours in a sporotrichoid pattern. Culture of the tumours was negative, and histopathology showed findings consistent with recurrent pcPTCL. The tumours were successfully treated with localised radiation therapy. Sporotrichoid lesions are an extremely rare and atypical presentation of cutaneous lymphoma, with only 2 other cases reported in the literature. Our case reinforces the need to include cutaneous lymphoma in the differential diagnosis of nodules on the extremities spreading in a sporotrichoid pattern. Clinical recognition of this atypical presentation of cutaneous lymphoma allows for prompt, effective treatment, which might include localised radiation therapy.

Primary cutaneous peripheral T-cell lymphoma mimicking pyoderma gangrenosum: A case report

Primary cutaneous peripheral T-cell lymphoma mimicking pyoderma gangrenosum: A case report

Primary cutaneous gamma delta T-cell lymphoma with a predominantly epidermotropic pattern

Primary cutaneous gamma delta T-cell lymphoma with a predominantly epidermotropic pattern

Rare but deadly: A case of primary cutaneous gamma-delta T-cell lymphoma

Rare but deadly: A case of primary cutaneous gamma-delta T-cell lymphoma

CD8陽性末梢性T細胞リンパ腫と鑑別を要した皮膚原発節外性NK/T細胞リンパ腫，鼻型の1例

62歳，男性。初診の6ヵ月前から躯幹・四肢に環状の紅斑を生じた。皮膚生検でCD8陽性異型リンパ球が血管周囲および表皮内へ密に浸潤する像を認め，CD8陽性皮膚T細胞性リンパ腫が疑われ当科へ紹介された。初診時，全身に多発する環状の紅斑と紅色腫瘤を認めた。末梢血に異型細胞はなく，PET－CTを含む画像検査では，皮膚に多発する腫瘤への集積像を認めたが，他に異常集積は認めなかった。受診後より皮疹が急速に増悪し，末梢性T細胞リンパ腫，非特定としてCHOP療法を開始した。その後，追加の精査にて浸潤細胞はCD56陽性，EBER－ISH陽性であり，Southern blot hybridization法でTCR遺伝子再構成検査も陰性であることが判明し，皮膚原発節外性NK/T細胞リンパ腫と診断した。CHOP療法は無効でSMILE療法に変更し，2コースで完全寛解を得た。CD8陽性末梢性T細胞リンパ腫との鑑別が重要と考え報告した。

Primary Cutaneous γδ T-Cell Lymphoma and Hemophagocytic Syndrome.

Primary cutaneous γδ T-cell lymphoma and hemophagocytic syndrome (HPS) is a very rare disease process with only 41 cases of this type of lymphoma published to date. We report the case of a 56-year-old woman who developed high-grade fevers and multiple nonhealing bilateral lower extremity ulcers associated with a recent diagnosis of Ackerman syndrome. Multiple lesion biopsies yielded a differential diagnosis including sarcoidosis and lupus panniculitis. Each biopsy site developed into a poorly healing wound. After extensive rheumatologic and dermatologic workup, failed courses of hyperbaric oxygen and corticosteroids, and the development of worsening fevers, the diagnosis of γδ T-cell lymphoma was made. The objective of this article was to illustrate a very rare case of a rapidly progressing cutaneous lymphoma while stressing the importance of a surgical approach to nonhealing wounds and biopsy in chronic wounds.An aggressive multidisciplinary approach including infectious disease, hematology/oncology, nephrology, plastic surgery, and interventional pulmonology was used. Medical approaches included several courses of corticosteroids and antineoplastics. Our surgical approach included numerous excision and debridements of her nonhealing ulcers with removal of necrotic tissue. It also included placement of cadaveric epidermal graft, and bovine tendon collagen cross-linked with glycosaminoglycans with a silicone matrix bilayer (Integra, Plainsboro, NJ) combined with multiple local myocutaneous advancement flaps. This approach demonstrated clinical improvement in wound healing. Pathological examination of several lesions included immunohistochemistry staining, flow cytometry, and molecular studies.The diagnosis of primary cutaneous γδ T-cell lymphoma was made based on a pathology specimen. Subsequent imaging showed numerous pulmonary nodules later determined to be pulmonary Aspergillosis. Additionally, she developed a constellation of clinical and laboratory features defined as the HPS. Despite medical therapy and improved wound healing, she died to seizures that left her comatose, thought to be secondary to central nervous system advancement of her HPS. The patient eventually died after care was withdrawn.Cutaneous forms of lymphoma must be considered in the differential diagnosis of atypical cutaneous lesions or nonhealing wounds. There should be no delay in performing a skin biopsy to obtain a tissue diagnosis. A surgical approach to wounds clearly demonstrates improved wound healing.

Primary cutaneous extranodal natural killer/T-cell lymphoma misdiagnosed as peripheral T-cell lymphoma: the importance of EBV in situ hybridization for diagnosis

Cutaneous non-mycosis fungoides (MF) T- and NK/T-cell lymphomas are heterogeneous with different degrees of aggressiveness. Extranodal natural killer/T-cell lymphoma (ENKTL) is a predominantly extranodal lymphoma associated with EBV. In our recent Asian collaborative study on primary cutaneous ENKTL, we identified five patients initially misdiagnosed as various types of PTCL because EBER was not performed. Three patients were males and two were females, with a median age of 45 years (range 28~71 years). Based on the initial diagnoses (two as PTCL, two as primary cutaneous CD30-positive T-cell lymphoproliferative disorders and one as subcutaneous panniculitis-like T-cell lymphoma), all patients received CHOP chemotherapy and three with additional radiotherapy. All patients experienced persistent disease or relapse despite treatment (mean duration 11 months, range 1~24 months). These rare tumors often pose significant diagnostic challenges to the practicing dermatopathologists and (hemato)pathologists. We proposed to include EBER as a screening assay for primary cutaneous non-MF T-cell lymphoma, at least in East Asia where the relative frequency of such tumors is higher than in the West. As novel chemotherapy regimen SMILE (L-asparaginase, methotrexate, ifosphamide, etoposide, and dexamethasone) has been shown to be more effective than CHOP for ENKTL, a correct diagnosis of ENKTL is very important.

Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Marked Pagetoid Epidermotropism Shortly After Allogeneic Stem Cell Transplantation

Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Marked Pagetoid Epidermotropism Shortly After Allogeneic Stem Cell Transplantation

Primary Cutaneous γδ T-Cell Lymphoma Should Be Limited to Cases With TCRγδ Expression

I read with great interest the article by Magro and Wang1 on 5 cases of primary cutaneous γδ T-cell lymphoma with an indolent course. These 5 patients had localized T-cell lymphoma in the subcutis with a less aggressive clinical course, and the authors raised the point that not all patients with primary cutaneous γδ T-cell lymphoma have a poor prognosis. The major weakness …

Characteristics of primary cutaneous lymphoma according to WHO-EORTC classification in Korea

Primary cutaneous lymphoma (PCL) is an extranodal non-Hodgkin lymphoma with primary involvement of the skin. Epidemiological data on PCLs according to the World Health Organization/European Organization for Research and Treatment of Cancer classification (WHO-EORTC) has not been investigated in Korea to date. To evaluate the demographic characteristics, clinical and histological features, and survival data of patients with PCL according to the WHO-EORTC classification. In total, 93 patients with PCL were retrospectively identified from an extensive review of medical records over a 16-year period. The tumours found included primary cutaneous CD30+ lymphoproliferative disorders, extranodal natural killer/T-cell lymphoma and primary cutaneous diffuse large B-cell lymphoma. We found that 81.6% of the patients had primary cutaneous T-cell and natural killer-cell lymphoma, and 16.2% had primary cutaneous B-cell lymphoma, with 2.2% having precursor haematological neoplasms. The median age was 52 years (range 3-95) and the male : female ratio was 1: 1.16. The 5-year survival rate was 92.5%. The incidence rates of many PCL subtypes in Koreans differ from those of other countries.