Cutaneous T-cell Lymphoma Presenting Research Articles

18F-Labeled Fluoro-2-Deoxyglucose Positron Emission Tomography and Computed Tomography Findings in a Case of Atypical Presentation of Adult Cutaneous T-cell Lymphoma.

Adult cutaneous T-cell lymphoma is an uncommon malignancy with poor prognosis and is usually seen in association with human T-cell lymphotropic virus type 1. We present the case of a 25-year-old female who gave a history of extensive whole-body polypoidal cutaneous and bilateral orbital and breast swellings. Biopsy was suggestive of cutaneous T-cell lymphoma and the patient was evaluated with 18F-labeled fluoro-2-deoxyglucose positron emission tomography and computed tomography for initial staging of the disease.

Diagnostic testing of eczematous dermatitis with incomplete response to dupilumab

Diagnostic testing of eczematous dermatitis with incomplete response to dupilumab

Tre-P-19 - Autologous stem cell transplantation in atypical mycosis fungoides with central nervous system involvement: follow-up

Mycosis fungoides (MF) is a presentation of primary cutaneous T-cell lymphoma with adverse prognosis for patients with advanced stages of the disease. Refractory disease and advanced-stage disease require systemic therapy. We reported a rare case of an atypical predominantly CD8+ folliculotropic MF, a subtype of MF with poorer prognosis, in a 59-year-old woman. She was initially diagnosed with MF restricted to the skin, of T3N0M0B0/stage IIB according to the current World Health Organization–European Organisation for Research and Treatment of Cancer classification. First-line treatment with local percutaneous radiotherapy in combination with systemic interferon alfa-2a resulted in complete remission. However, 21 months later the disease progressed to T3N0M1B0/stage IVB with development of cerebral manifestation and thus very poor prognosis. Allogeneic stem cell transplantation (SCT) was not a therapeutic option due to the lack of a suitable donor. We initiated methotrexate and cytarabine chemotherapy, followed by high-dose chemotherapy with thiotepa and carmustine with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Readministration of interferon alfa-2a as a maintenance treatment after the salvage autologous SCT resulted in a complete remission during a follow-up period of 32.5 months after autologous SCT. After that, the disease relapsed with simultaneous skin and cerebral lesions. Despite immediate intrathecal and subsequently intravenous administration of high-dose methotrexate and cytarabin, we observed further progressed and the patient deceased 3.5 months after this second fulminant recurrence and in total 36 months after autologous SCT transplantation.

A clinician's guide to cutaneous T-cell lymphoma presenting as recalcitrant eczematous dermatitis in adults.

Cutaneous T-cell lymphoma (CTCL) encompasses a group of low-grade, non-Hodgkin lymphoma, including mycosis fungoides and Sézary syndrome. Diagnosis of CTCL can be challenging given the prolonged, gradual onset and shared characteristics with many benign inflammatory skin diseases. In this case series, we describe four unique cases of patients with chronic, recalcitrant eczematous dermatitis who presented for a patch-test consultation and were ultimately diagnosed with CTCL. In particular, we highlight clinical pearls to aid in distinguishing CTCL from inflammatory dermatoses and describe the diagnostic strategy to help dermatologists arrive at the diagnosis of CTCL at earlier stages of the disease.

Synergistic cytotoxic activity of cannabinoids from cannabis sativa against cutaneous T-cell lymphoma (CTCL) in-vitro and ex-vivo.

Cannabis sativa produces hundreds of phytocannabinoids and terpenes. Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL), characterized by patches, plaques and tumors. Sézary is a leukemic stage of CTCL presenting with erythroderma and the presence of neoplastic Sézary T-cells in peripheral blood. This study aimed to identify active compounds from whole cannabis extracts and their synergistic mixtures, and to assess respective cytotoxic activity against CTCL cells. Ethanol extracts of C. sativa were analyzed by high-performance liquid chromatography (HPLC) and gas chromatography/mass spectrometry (GC/MS). Cytotoxic activity was determined using the XTT assay on My-La and HuT-78 cell lines as well as peripheral blood lymphocytes from Sézary patients (SPBL). Annexin V assay and fluorescence-activated cell sorting (FACS) were used to determine apoptosis and cell cycle. RNA sequencing and quantitative PCR were used to determine gene expression. Active cannabis compounds presenting high cytotoxic activity on My-La and HuT-78 cell lines were identified in crude extract fractions designated S4 and S5, and their synergistic mixture was specified. This mixture induced cell cycle arrest and cell apoptosis; a relatively selective apoptosis was also recorded on the malignant CD4+CD26- SPBL cells. Significant cytotoxic activity of the corresponding mixture of pure phytocannabinoids further verified genuine interaction between S4 and S5. The gene expression profile was distinct in My-La and HuT-78 cells treated with the S4 and S5 synergistic mixture. We suggest that specifying formulations of synergistic active cannabis compounds and unraveling their modes of action may lead to new cannabis-based therapies.

A Unique Presentation of Cutaneous T-cell Lymphoma Involving the Gastrointestinal Tract

Introduction: Most cases of lymphoma involving the gastrointestinal (GI) tract are of B-cell lineage. T-cell lymphoma affecting the GI tract is infrequent. Cases of lymphoma with GI involvement mainly involve primary lesions and not secondary disease. Herein, is a patient with cutaneous T-cell lymphoma (CTCL) who presented with a bowel perforation and found to have secondary T-cell lymphoma of the small intestine. Case: A 74-year-old Caucasian female with primary CTCL (Figure 1) treated with conventional chemotherapy agents and radiation of large areas of her skin presented with one-month history of periumbilical abdominal pain. Admission labs revealed normocytic anemia, severe thrombocytopenia, elevated transaminases, and hyperbilirubinemia. Computed tomography of the abdomen and pelvis with oral contrast was concerning for perforated viscus in the pelvis, most likely communicating with the cecum with what appeared to be extravasation of oral contrast (Figure 2). Exploratory laparotomy revealed wide-open perforation of distal ileum, matted interloop adhesions of small bowel, with distal ileal obstruction, and fecal peritonitis. She underwent a resection of the distal ileum with reanastomosis. Immunohistochemistry and histopathology from jejunal and distal ileum biopsies were consistent with T-cell lymphoma (Figure 3). The patient had recurrent refractory T-cell lymphoma now with bowel metastasis. Given her poor prognosis, the patient transitioned to hospice. Discussion: GI lymphoma represents up to 10% of primary GI malignancies with B-cell lymphoma occurring more often than T-cell lymphoma. CTCL, a type of non-Hodgkin lymphoma, is diagnosed by the presence of malignant T-cells in chronically inflamed skin. Early presentation is mostly limited to skin patches or plaques, but in one third of patients the disease becomes progressive and spreads to blood, lymph nodes, and visceral organs. The most frequent site of GI involvement is the stomach, followed by the small intestine and ileocecal area.A study that reviewed the incidence of perforation in biopsy-proven GI involvement lymphoma found that 59% of perforations were associated diffuse large B-cell lymphoma. In this case, the patient had secondary T-cell lymphoma, which is infrequent, and was found to have a perforation which is uncommon. Patients with a history of lymphoma who present with new onset abdominal pain should be evaluated for free air in the abdomen to rule out perforation.2568_A Figure 1. Wound on the left lower extremity2568_B Figure 2. CT abdomen and pelvis with oral contrast showing extraluminal extravasation of contrast in pelvic cul-de-sac2568_C Figure 3. T-cell marker (CD3) expression in neoplastic cells from small bowel biopsy (A) and hematoxylin and eosin stain showing lymphocyte predominance from small bowel biopsy (B)

Primary Cutaneous T-Cell Lymphomas Showing Gamma-Delta (γδ) Phenotype and Predominantly Epidermotropic Pattern are Clinicopathologically Distinct From Classic Primary Cutaneous γδ T-Cell Lymphomas.

Primary cutaneous gamma-delta (γδ) T-cell lymphoma is a rare disease that typically involves the dermis and subcutis. Cases of primary cutaneous T-cell lymphomas showing γδ phenotype and predominantly epidermotropic pattern (EγδTCL) are not well defined. In this series, cases of primary cutaneous T-cell lymphomas showing γδ phenotype were reviewed and classified as predominantly epidermotropic (EγδTCL) when >75% of lymphoma cells resided in the epidermis or predominantly dermal and/or subcutaneous (DSγδTCL). Clinical, pathologic, and immunophenotypic features were compared in 27 biopsies from 13 patients of EγδTCL and 13 biopsies from 7 patients of DSγδTCL. The lymphoma cells were diffusely positive for CD3 and T-cell receptor (TCR)γ, mostly positive for granzyme B and TIA-1, variably positive for CD8, CD7, and CD30, and negative for CD4 and TCRβ. Two patients with EγδTCL had dissemination to lymph nodes and 1 to the lung; 1 patient with DSγδTCL had gastrointestinal involvement. The median survival of patients with EγδTCL was not reached, and with a median follow-up of 19.2 months, 3/13 died. In contrast, the median survival of patients with DSγδTCL was 10 months, and after a median follow-up of 15.6 months, 5/5 died (P<0.01). EγδTCL is a rare presentation of cutaneous T-cell lymphoma that can be distinguished from DSγδTCL based on the extent of epidermotropism and has a better prognosis and longer median survival than DSγδTCL. However, although EγδTCL resembles mycosis fungoides clinically and histologically, a subset of EγδTCL is more likely to behave more aggressively than typical mycosis fungoides.

Cutaneous T-cell lymphoma presenting as panniculitis with arthritis

Cutaneous T-cell lymphoma presenting as panniculitis with arthritis

Cutaneous lymphomas with a panniculitic presentation.

Subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous gamma/delta T-cell lymphoma are the two most common types of cutaneous T-cell lymphoma presenting with panniculitis-like lesions. In this article the characteristic clinical, histological and immunophenotypical features of these conditions are reviewed and criteria to differentiate these tumors from other benign and malignant lymphoproliferations presenting with panniculitis-like lesions are discussed.

Synchronous presentation of cutaneous T-cell lymphoma and chronic lymphocytic leukemia

Synchronous presentation of cutaneous T-cell lymphoma and chronic lymphocytic leukemia

Multilayered T-cell memory in human skin.

The skin represents a highly complex immunological microenvironment capable of protecting the organism from infectious pathogens. Accordingly, the human skin harbors large quantities of immune cells, including billions of memory T cells (1,2). While some of these T cells are constantly recirculating through the skin and thus are in equilibrium with their counterparts in the blood, others remain permanently resident in the skin and never return to the circulation (2). A recent study published in Science Translational Medicine by Watanabe and colleagues (3) has shed new light on the composition of these recirculating and resident T-cell subsets in human skin, and has linked the malignant transformation of individual subsets to the variable clinical presentation of cutaneous T-cell lymphoma (CTCL).

A Rare Case of Hydroa Vacciniforme–Like Cutaneous T-Cell Lymphoma Presenting Mimicking Herpes Simplex in an Adult

A Rare Case of Hydroa Vacciniforme–Like Cutaneous T-Cell Lymphoma Presenting Mimicking Herpes Simplex in an Adult

Hydroa vacciniforme–like cutaneous T-cell lymphoma: Clinicopathologic and immunohistochemical study of 12 cases

Hydroa vacciniforme (HV)-like lymphoma (HVL) is a rare and aggressive cutaneous T-cell lymphoma occurring mainly in children in Latin America and Asia. Chronic latent Epstein-Barr virus infection has been associated with both HV and HVL. We sought to evaluate the clinical presentation and histopathology of this rare cutaneous T-cell lymphoma. We reviewed the clinical, morphologic, and immunophenotypical features in 12 cases of HVL from Bolivia. All 12 patients had skin lesions in both sun-exposed and nonsun-exposed areas, including edema, blistering, ulceration, and scarring, with a slowly progressive relapsing course. All 12 patients presented with systemic symptoms and showed a characteristic swelling of the nose and lips, and periorbital edema. Eight patients died an average of 5.3 months after initial diagnosis. Four patients remained alive with persistent disease. Histopathologic examination showed an atypical lymphocytic infiltrate with angiotropism and angiocentricity. The immunophenotype showed a cytotoxic T-cell (CD8(+)) profile. All cases were associated with Epstein-Barr virus infection and differed clinically from other forms of cutaneous T-cell lymphoma. Only a limited number of cases were studied. This study confirms that HVL is a highly aggressive lymphoma, although some patients have a more indolent, chronic course.

Cutaneous γδ T-cell lymphoma presenting as facial palsy

Cutaneous γδ T-cell lymphoma presenting as facial palsy

CD20+ primary cutaneous T-cell lymphoma presenting as a solitary extensive plaque

CD20+ primary cutaneous T-cell lymphoma presenting as a solitary extensive plaque

Successful therapy of cutaneous Tcell lymphoma

Treatment objectives of cutaneous T-cell lymphoma can be achieved with a variety of modalities applied to the variable presentations of the disease. In this review, the goals of therapy are presented and each therapy is summarized in terms of its use to achieve those goals. After reviewing the modalities, the strategies are summarized in reference to each presentation of cutaneous T-cell lymphoma.

Unilesional folliculotropic/syringotropic cutaneous T-cell lymphoma presenting as an indurated plaque on the nape

International Journal of DermatologyVolume 45, Issue 10 p. 1268-1270 Unilesional folliculotropic/syringotropic cutaneous T-cell lymphoma presenting as an indurated plaque on the nape Pa-Fan Hsiao MD, MMS, Pa-Fan Hsiao MD, MMSSearch for more papers by this authorCheng-Hsiang Hsiao MD, Cheng-Hsiang Hsiao MDSearch for more papers by this authorTsen-Fang Tsai MD, Tsen-Fang Tsai MDSearch for more papers by this authorShiou-Hwa Jee MD, PhD, Shiou-Hwa Jee MD, PhDSearch for more papers by this author Pa-Fan Hsiao MD, MMS, Pa-Fan Hsiao MD, MMSSearch for more papers by this authorCheng-Hsiang Hsiao MD, Cheng-Hsiang Hsiao MDSearch for more papers by this authorTsen-Fang Tsai MD, Tsen-Fang Tsai MDSearch for more papers by this authorShiou-Hwa Jee MD, PhD, Shiou-Hwa Jee MD, PhDSearch for more papers by this author First published: 11 October 2006 https://doi.org/10.1111/j.1365-4632.2006.03080.xCitations: 12Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume45, Issue10October 2006Pages 1268-1270 RelatedInformation

Hypopigmented mycosis fungoides in a 10-year-old boy

Cutaneous T-cell lymphoma (CTCL) presenting with hypopigmented lesions is an uncommon clinical variant of the disease, usually described in dark-skinned patients. We report a case of hypopigmented CTCL in a 10-year-old boy. The disease has responded favorably to narrowband UVB therapy. This case illustrates the importance of clinical suspicion for mycosis fungoides in patients with widespread hypopigmentation.

Alemtuzumab: effective monotherapy for simultaneous B‐cell chronic lymphocytic leukaemia and Sézary syndrome

The simultaneous presentation of chronic lymphocytic leukaemia (CLL) and cutaneous T-cell lymphoma (CTCL) is a very rare occurrence where optimal treatment is unknown. We present the case of a 65-yr-old man who was successfully treated with alemtuzumab monotherapy for both disorders, but at a cost of severe infectious morbidity and prolonged pancytopenia.

Cutaneous T‐cell lymphoma presenting as disseminated, pigmented, purpura‐like eruption

A 48-year-old man with a history of psoriasis, who had received no oral pharmacologic treatment, presented in March 2000 with a progressive, cutaneous, pruritic eruption that began on his arms and legs, and became generalized. Examination revealed discrete, purpuric elements disseminated over the entire cutaneous surface, and in some areas confluent, large, erythematous, purpuric patches (1, 2). The palmoplantar surfaces and mucous membranes were spared. No lesions suggesting parapsoriasis or the usual presentation of cutaneous T-cell lymphoma (CTCL) were present. Figure 1Open in figure viewerPowerPoint Confluent, large, erythematopurpuric patches with sharply pointed purpuric elements on the buttocks Figure 2Open in figure viewerPowerPoint Similar lesions on the lower limbs Histopathologic examination of a cutaneous specimen showed numerous atypical lymphocytes in the superficial dermis, slight changes at the dermo-epidermal junction, and an intense epidermotrophism, with atypical lymphocytes in the epidermis. These intraepidermal atypical lymphocytes were present both isolated and forming aggregates of 4-7 cells (Fig. 3). Immunohistochemical studies demonstrated that these lymphocytes were T-helper cells. Figure 3Open in figure viewerPowerPoint Atypical lymphocytes in the superficial dermis, slight changes at the dermo-epidermal junction, and intense epidermotrophism, with atypical lymphocytes. Intraepidermal lymphocytes were present both isolated and forming small cellular aggregates Routine laboratory tests were normal, except for slight hypercholesterolemia. Direct examination of peripheral blood, coagulation, antinuclear antibody (ANA) immunofluorescent test, body scanner, and bone marrow study were normal. CTCL stage IB was diagnosed and psoralen plus UVA (PUVA) therapy was initiated.