Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

Olsen, Elise A.; Rook, Alain H.; Zic, John A.; Kim, Youn; Porcu, Pierluigi; Querfeld, Christiane; Wood, Gary S.; Demierre, Marie‐France; Pittelkow, Mark R.; Wilson, Lynn D.; Pinter‐Brown, Lauren; Advani, Ranjana H.; Parker, Sareeta; Kim, Ellen J.; Junkins‐Hopkins, Jacqueline M.; Foss, Francine M.; Cacchio, Patrick; Duvic, Madeleine

doi:10.1016/j.jaad.2010.08.037

Cited by 155 publications

(153 citation statements)

References 317 publications

(376 reference statements)

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“…Other treatment options include phototherapy with PUVA or UVBnb; total skin electron beam radiation; interferon α or γ; bexarotene; denileukin diftitox; methotrexate; histone deacetylase inhibitors (vorinostat and romidepsin); alemtuzumab (anti-CD52 monoclonal antibody); chlorambucil combined with prednisone; systemic chemotherapy with gemcitabine and vinorelbine; and anthracycline-based schemes. As in MF, multidrug polychemotherapy for SS has partial and transient responses, with rapid relapses and minimal impact on overall survival 48 . Allogeneic bone marrow transplantation reports show promising results concerning the sustained remission of the disease 49 .…”

Section: Sézary Syndrome (Ss)mentioning

confidence: 99%

Cutaneous lymphomas: A review

Miyashiro¹,

Sanches²

2015

Cumhuriyet Med J

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SUMMARYSkin may be affected by non-Hodgkin lymphomas, and it is the second most frequently involved extranodal organ, after the gastrointestinal tract. Cutaneous lymphomas may originate from T, B, or NK lymphocytes. Diagnosis is difficult, and knowledge of these diseases is important to ensure their detection and adequate treatment and follow-up. Clinical picture is heterogeneous, and histology is essential to confirm the diagnosis. The classification is given by the correlation between clinical findings, histology and immunophenotyping. Therapeutic options include skin-directed therapies, immunomodulatory or low-dose immunosuppressive drugs. Rare cases of aggressive disease require systemic multidrug chemotherapy. The purpose of this review is to describe the ontogeny of T, B and NK lymphocytes, and to detail the most accepted classification of cutaneous lymphomas, proposed by the World Health Organisation and European Organisation for Research and Treatment of Cancer. Treatment of cutaneous lymphomas will be briefly discussed.

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Section: Sézary Syndrome (Ss)mentioning

confidence: 99%

Cutaneous lymphomas: A review

Miyashiro¹,

Sanches²

2015

Cumhuriyet Med J

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“…Limitados parches, pápulas y/ o placas que cubren menos de 10% de la superficie corporal, se podría dividir en T1a (sólo parches) o T1b ( placas + parches) T 2 Parches, pápulas y/ o placas que cubren más del 10% de la superficie corporal, se podría dividir en T2a (sólo parches) o T2b ( placas + parches) T 3 Uno o más tumores (mayor a 1 cm de diámetro) T 4 Confluencia de eritema cubriendo mayor o igual al 80% de la superficie corporal …”

Section: Compromiso De Piel (T) Tunclassified

“…En la mayoría de los casos de SS no se puede determinar la causa, pero algunos casos han sido asociados a virus linfotrópicos tipo 1 y 2 (HTLAV1/2) 3 .…”

Section: Enfermedad Recurrente O Refractariaunclassified

Fotoféresis en el tratamiento de Síndrome de Sézary: Caso clínico

et al. 2015

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“…The aetiology of the disease is still largely unknown. Except for a small number of therapeutic agents (interferons), there are no reliable efficacy data on agents used to treat SS patients [6]. A better understanding of the immunopathogenesis of this disease could aid in finding a more effective therapy.…”

Section: Introductionmentioning

confidence: 99%

“…In general, a shift in cytokine response from Th1 to Th2 is prominent in this disease, with concurrent down-regulation of IFN- and up-regulation of IL-10, IL-4 and IL-5. The latter are associated with defective neutrophil function and a reduction in cutaneous dendritic cells, as a result of the abnormal cytokine milieu [6]. Control of lymphocytic function and proliferation is of the utmost importance in the physiological balance of the immune system; alteration of this homeostasis may be important in the development of cancer or autoimmunity.…”

Section: Introductionmentioning

confidence: 99%

Thymopentin down-regulates both activity and expression of iNOS in blood cells of Sézary syndrome patients

et al. 2012

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While thymopentin has been used for many years in the experimental treatment of Sézary syndrome (SS), a rare and very aggressive lymphoma, its mechanism of action is still not known.Herein we show that this peptide acts as an inhibitor of isolated iNOS and nNOS isoforms, and reduces iNOS protein/mRNA levels and iNOS activity in blood cells obtained from both healthy donors and SS patients. Similar results were obtained with TPN-2, the N  -nitro analogue of the Arg-Lys motif present in thymopentin. Additional investigations are necessary to confirm the role and the relative importance of the two mechanisms of iNOS down-regulation in the therapeutic action of these peptides against SS.

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Sézary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

Cited by 155 publications

References 317 publications

Cutaneous lymphomas: A review

Cutaneous lymphomas: A review

Fotoféresis en el tratamiento de Síndrome de Sézary: Caso clínico

Thymopentin down-regulates both activity and expression of iNOS in blood cells of Sézary syndrome patients

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