Successful treatment of Sezary syndrome with extracorporeal photopheresis – The first attempt in India

Aseem K Tiwari, Dinesh Arora, Pratibha Dhiman, Sheilly Kapoor, Geet Aggarwal, Ravi C Dara, Ashok VaidAsian Journal of Transfusion Science 2019 13(1):66-69Sezary syndrome (SS) is more aggressive leukemic variant of cutaneous T-cell lymphoma in which a significant number of circulating malignant (Sezary) cells are observed in peripheral blood. Although single-agent or combination chemotherapy regimens have produced moderately high response rates in patients with advanced-stage SS, these responses are invariably not durable. Extracorporeal photopheresis (ECP) is recommended as an immunomodulator treatment, offering better life quality for patient. We would like to present the first SS case treated successfully with low-dose methotrexate and ECP in India. A 50-year-old male presented with rash and severe pruritus all over the body for 2 years. He had received various treatment regimens but without any symptomatic improvement. He underwent detailed examination and diagnosis of SS was established. Peripheral smear revealed total leukocyte count of 14900/μ l with 55% cells reported as Sezary cells. Contrast-enhanced computerized tomography revealed few insignificant (<1.5 cm) bilateral nodes in the axillary and inguinal region. The patient's disease stage was determined IVA1, and grade was T4N0M0B2. He received six cycles of CHOP, which led to a short-term remission of <3 months, and he was started on single-agent methotrexate along with skin supportive treatmen...
Source: Asian Journal of Transfusion Science - Category: Hematology Authors: Source Type: research

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AbstractThe major aim of Session 1 of the 2018 European Association of Hematopathology/Society for Hematopathology Workshop was to collect examples of cutaneous lymphomas, excluding mycosis fungoides/Sezary syndrome, as defined in the current WHO classification of tumours of the haemetopoietic and lymphoid tissues. Overall 42 cases were submitted. These were considered in four main categories: primary cutaneous B cell lymphomas (12 cases), primary cutaneous T cell lymphomas/lymphoproliferations with CD8+/cytotoxic phenotype (12 cases), primary cutaneous CD30-positive lymphoproliferative disorders (15 cases) and primary cut...
Source: Virchows Archiv - Category: Pathology Source Type: research
Contributors : Maria Wysocka ; Andrew V Kossenkov ; Bernice M Benoit ; Elisha Singer ; Andras Schaffer ; Tzvete Dentchev ; Satoshi Nagata ; Tomoko Ise ; Louise C Showe ; Alain H RookSeries Type : Expression profiling by arrayOrganism : Homo sapiensSezary syndrome (SS) represents a leukemic variant of cutaneous T cell lymphoma (CTCL) with circulating malignant CD4 T cells trafficking to the skin. The cell surface molecules present on malignant cells are also expressed on normal CD4 T cells. Therefore, we attempted to find a specific marker for malignant cells that would distinguish them from normal cells. Comprehensive micr...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by array Homo sapiens Source Type: research
Mycosis fungoides (MF) and Sezary syndrome (SS) are subtypes of extranodal T cell nonHodgkin lymphoma, and are the most common forms of cutaneous T cell lymphoma (CTCL). Although both subtypes involve the skin, SS is characterized by leukemic involvement of malignant T cells that typically match the clone found in the skin. MF has a plethora of clinicopathological variants, one of which is the pigmented purpuric dermatosis (PPD)-like MF. Although the PPD-like MF is rarely reported, it has been previously described as either preceding MF or appearing as the initial presentation of MF.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research
Conclusions Epidemiological studies have repeatedly helped identify definitive triggers for several diseases. As highlighted in this perspective report, previous studies strongly argue for the interplay between intrinsic factors and putative preventable extrinsic triggers/promoters for CTCL. Given the evidence of geographical regional clustering of CTCL patients, CTCL occurrence in unrelated family members and recent evidence implicating S. aureus in the pathogenesis/progression of CTCL, more research is needed to decipher the precise mechanism by which specific environmental exposures may be driving the pathogenesis of t...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Conclusions The major challenges in the development of adoptive cell therapy for T cell tumors, as mentioned above, remain fratricide, T cell aplasia and the potential for leukemic transduction or poor T cell function if used in the autologous setting. Approaches to overcome fratricide include the genetic modification and deletion of the T cell antigen in the case of long-term CAR-T cell persistence or regulated CAR-T expression. To ensure restoration of T cell immunity, transient CAR expression can be achieved incorporation of a CAR suicide gene, transient CAR expression using mRNA electroporation, or short-lived NK cell...
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
Abstract Advanced-stage cutaneous T-cell lymphoma (CTCL) is usually a fatal malignancy despite optimal use of currently available treatments. In this preclinical study of novel CTCL therapy, we performed in vitro and ex vivo experiments to determine the efficacy of combination treatment with a panel of BET bromodomain inhibitors (BETi) (JQ1, OTX015, CPI-0610, I-BET762) and HDAC inhibitors (HDACi) (SAHA/Vorinostat, Romidepsin). BETi/HDACi combinations were synergistic (combination index
Source: Neoplasia - Category: Cancer & Oncology Authors: Tags: Neoplasia Source Type: research
Cutaneous T-cell lymphomas (CTCL) represent up to 80% of extranodal non-Hodgkin Lymphomas, the most common being Mycosis Fungoides (MF) with or without its leukemic stage Sezary Syndrome and Primary cutaneous CD30+ T-cell lymphoproliferative disorders. Prognosis for advanced stages is poor, with 5-year survival 40-70% for patients with advanced skin stages, and 15-40% for those with extracutaneous involvement and CD30+ transformed CTCL. The disease is considered incurable, and most patients will undergo at least two different lines of therapy, and up to 36% undergo at least four different lines due to a short duration of r...
Source: Blood - Category: Hematology Authors: Tags: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma-Clinical Studies Source Type: research
Background: Sézary syndrome (SS) is a highly-morbid T cell leukemic lymphoma with no widely-effective treatments and few preclinical models. We demonstrated effective T cell lymphoma therapy with allogeneic gene-edited anti-CD7 CARTs (Cooper et al, Leukemia, 2018). However, SS T cells typically lose CD7 but maintain ubiquitous high CD2 expression. Thus, we generated CD2- and TRAC-deleted anti-CD2 universal CARTs (UCART2) and multiple SS xenograft models (PDXs) as preclinical UCART2 testing platforms. We further tested a stable homodimeric interleukin-7 molecule, the long-acting form of recombinant human interleukin-...
Source: Blood - Category: Hematology Authors: Tags: 625. Lymphoma: Pre-Clinical-Chemotherapy and Biologic Agents: Immunologic approaches Source Type: research
Conclusions: Lymphoma cells in MF/SS show marked heterogeneity of expression of PD-1 including clear subset arising from TFH. This suggests MF/SS may represent multiple biological entities. Further studies will be necessary to investigate the clinical significance of cell-of-origin of MF/SS.DisclosuresYabe: Y-mAbs Therapeutics: Consultancy. Moskowitz: ADC Therapeutics: Research Funding; Incyte: Research Funding; Bristol Myers-Squibb: Consultancy, Research Funding; Takeda: Honoraria; Merck: Research Funding; Seattle Genetics: Consultancy, Honoraria, Research Funding. Horwitz: Infinity/Verastem: Consultancy, Research Funding...
Source: Blood - Category: Hematology Authors: Tags: 624. Hodgkin Lymphoma and T/NK Cell Lymphoma-Clinical Studies: Poster I Source Type: research
Introduction: Leukemic cutaneous T cell lymphoma, better known as Sezary syndrome, represents an aggressive subtype of cutaneous T cell lymphoma, characterized by extensive skin, lymph-node and peripheral blood involvement. The prognosis is generally poor, due to the progressive nature of the disease that leads to profound immunosuppression and subsequent opportunistic infections. There is no standard initial treatment and durable responses are rarely achieved. Alemtuzumab is a humanized monoclonal antibody against CD52, a cell surface antigen carried by most leukocytes, including B and T cells.
Source: European Journal of Cancer - Category: Cancer & Oncology Authors: Source Type: research
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