Insuficiencia respiratoria en una paciente con síndrome de Sézary / Respiratory failure in a patient with Sézary syndrome

Paciente de 44 años de sexo femenino, sin ninguna enfermedad de base preexistente, con una historia de aproximadamente diez meses de presentar lesiones eritemato-descamativas pruriginosas inicialmente localizadas en extremidades inferiores y que luego se generalizaron en todo el cuerpo, asociándose a la pérdida de peso de aproximadamente 15 kg. El manejo inicial consistió en corticoides tópicos y antihistamínicos orales con poca respuesta clínica. Se inició el estudio por dermatología y se confirmó el diagnóstico inicial de neoplasia cutánea maligna de células T. Luego se realizó el frotis de médula ósea, en el que se identificaron células «cerebriformes¼ que confirmaron el diagnóstico de síndrome de Sézary. La paciente recibió esquema de quimioterapia ciclofosfamida, doxorrubicina, vincristina, etopósido y prednisona. La respuesta inicial fue favorable, con alta hospitalaria y seguimiento en la consulta externa. Transcurridos tres meses de tratamiento, la paciente consultó por episodio febril, tos productiva más distrés respiratorio asociado a estertores basales bilaterales, presentó insuficiencia respiratoria y durante la inducción a la ventilación mecánica sufrió un paro cardiorrespiratorio y falleció

44-year-old female patient, with no preexisting underlying disease, with a history of approximately ten months of presenting pruritic erythematous-desquamative lesions initially localized in the lower extremities and later generalized throughout the body, associated with weight loss of 15 kg. Treatment. Initial management consisted of topical corticosteroids and oral antihistamines with little clinical response. A dermatology wok-up was initiated, and the initial diagnosis of malignant T-cell neoplasm was confirmed. A bone marrow smear was performed, in which "cerebriform" cells were identified, confirming the diagnosis of Sézary syndrome. The patient received cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone chemotherapy. Outcome. The initial response was favorable, with hospital discharge and outpatient follow-up. After three months of treatment, the patient consulted for a febrile episode, productive cough plus respiratory distress associated with bilateral basal rales, presented respiratory failure, and during induction of mechanical ventilation suffered cardiorespiratory arrest and died.

Concept of Cutaneous T Cell Lymphoma in Ayurveda Perspective and its Management by Sodhana, Samana and Rasayana: A Review Article

Cutaneous T cell lymphoma (CTCL) are a rare group of diseases caused by uncontrolled proliferation of T cells which belongs to mature T cell lymphoma having indolent nature. Two thirds of the CTCL are comprised of Mycosis Fungoides (MF) and Sezary Syndrome (SS). They are characterized by macules and patches which on later progresses to tumors or nodules with adenopathy and other organ infiltration. If left untreated the risk of developing infection increases with visceral involvement of skin, GI tract, lungs and adrenals. Diagnosis is done by histopathological appearance, cytogenetic analysis, etiology and the functional biology of neoplastic cells. Imaging techniques (MRI and CT) are widely done to assess the staging of disease and other tissue involvement. Radiotherapy, chemotherapy and retinoids have been in use since long time, but possess many side effects. According to Ayurveda, CTCL can be caused by Ahara like Virudha, Agantuja bhavas, Beeja-beejabhaga-beejabhagavayava dushti and Ojas/bala hani. The clinical features can be related with Kushta and in later stage simulates Dhatugata kushta and Granthi-arbuda. The etiopathogenesis of CTCL can be considered as formation of Ama, Agnimandhya, Srothovaigunya, and Balahani. Management will be preventive, curative and palliative with Sodhana, Samana and Rasayana therapies

Mycosis fungoides and Sézary syndrome: focus on the current treatment scenario

Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

Role of flow cytometric analysis of peripheral blood in the diagnosis of lymphoma-associated erythroderma / 中华皮肤科杂志

Objective:To investigate the value of flow cytometric analysis of peripheral blood in the diagnosis of erythroderma.Methods:A total of 29 patients with erythroderma were collected from Hospital of Dermatology, Chinese Academy of Medical Sciences from September 2017 to December 2020, including 6 with erythrodermic mycosis fungoides (EMF) , 5 with Sézary syndrome (SS) , 18 with inflammatory erythroderma (IE) with different etiologies. Four healthy volunteers served as healthy controls. Flow cytometry was performed to detect peripheral blood lymphocyte subsets, immunophenotypes and clonality, and their differences were analyzed between inflammatory erythroderma and lymphoma-related erythroderma. One-way analysis of variance and least significant difference- t test were used for comparisons between groups. Results:The proportions of T cells, B cells, NK cells and CD4 -CD8 - cells significantly differed among the EMF group, SS group, IE group and control group (all P < 0.001) . The proportion of T cells was significantly higher in the SS group (93.8% ± 3.4%) than in the EMF group (42.7% ± 6.4%) and IE group (46.0% ± 6.8%, t = 12.8, 14.4, respectively, both P < 0.001) , and the proportion of CD4 -CD8 - cells was significantly lower in the IE group (0.37% ± 0.40%) than in the EMF group (2.93% ± 0.84%) and SS group (2.38% ± 0.74%, t = 9.2, 6.7, respectively, both P < 0.05) . The expression of clonal T-cell receptor β-chain variable region (TCR-vβ) was not detected in healthy controls or IE patients; the T cell subsets expressing clonal TCR-vβ were detected in 3 cases of EMF and all cases of SS, and they were all identified to be cells with a CD4 +CD7 -CD26 - phenotype. There were significant differences among the above 4 groups of subjects in the proportions of CD4 + T lymphocytes expressing chemokine receptors CCR4, CXCR3, CCR5, cutaneous lymphocyte antigen (CLA) or programmed death receptor-1 (PD-1) on the cell surface (all P < 0.001) . Compared with the SS group and EMF group, the IE group showed significant decreased proportions of CD4 + T lymphocytes expressing CCR4, CLA or PD-1 (all P < 0.001) , but significantly increased proportions of CD4 + T lymphocytes expressing CXCR3 or CCR5 (all P < 0.001) . Conclusion:Flow cytometric analysis of peripheral blood lymphocyte subsets, immunophenotypes and clonality can provide a reference for the etiological diagnosis of erythroderma, and is helpful for the differential diagnosis between lymphoma-associated erythroderma and inflammatory erythroderma.

Síndrome de Sézary. Etiopatogenia, epidemiología, clínica, diagnóstico y pronóstico / Sézary syndrome Pathogenesis, epidemiology, clinical features, diagnosis and prognosis

El síndrome de Sézary (SS) es una rara y agresiva variante leucémica del linfoma cutáneo de células T, de pronóstico ominoso. Se caracteriza por presentar la tríada eritrodermia, linfadenopatías y linfocitos T neoplásicos circulantes. El diagnóstico está dado por la clínica, el estudio histopatológico, la citometría de flujo y el reordenamiento genético del receptor del linfocito T. En esta revisión se analizan la presentación clínica, la histopatología, el diagnóstico y el pronóstico de este síndrome. (AU)

Sézary syndrome (SS) is a rare and aggressive leukemic cutaneous T-cell lymphoma with poor prognosis. Is characterized by a triad of erythroderma, lymphadenopathy and circulating neoplastic T cells. Diagnosis is made by clinical features, histopathology, flow cytometry and T-cell receptor gene rearrangements. In this review we will analyze clinical presentation, histopathology, diagnosis and prognosis of SS. (AU)

Síndrome de Sézary. Presentación de un caso / Sézary síndrome. Case presentation

RESUMEN El síndrome de Sézary constituye la fase leucémica de la micosis fungoide caracterizado por eritrodermia, adenopatías superficiales y células atípicas en sangre. Predomina en los hombres con una proporción 2/1 respecto a las mujeres, y en las edades entre los 60 y 70 años de edad. La enfermedad es de difícil tratamiento, con un pronóstico reservado por su baja supervivencia. Por ser infrecuente y su posible similitud con otras dermatosis, se presenta un caso con antecedentes de psoriasis vulgar con 5 años de evolución, que hacía aproximadamente 6 meses, se encontraba sin mejoría en brote de agudización a pesar de los tratamientos indicados (AU).

ABSTRACT Sezary syndrome is the leukemic part of the fungoid mycosis, characterized by erythroderma, surface adenopathies and atypical cells in blood. It predominates in men with a 2/1 proportion in respect to women, and in ages ranging from 60 to 70 years. It is a difficult treated disease, with a reserved prognosis because of the low survival. Due to its infrequency and possible similarity to other dermatosis, it is presented a case with antecedents of vulgar psoriasis of 5 years evolution, without improvement for around 6 months, in acute outbreak in spite of the indicated treatments (AU).

Performance Evaluation of Alarms and Parameters Generated by Hematology Analyzer during Detection of Circulating Sezary Cells of Peripheral Blood Smears / 现代检验医学杂志

Objective To evaluate the performance alarms and parameters generated by hematology analyzer during detection of circulating sezary cells of peripheral blood smears.Methods Blood samples from patients with Sezary syndrome (n=21) were studied with Sysmex XN-9000 analyzer,and compared to patients with benign or tumoral skin lesions (n=48) and patients with chronic lymphoproliferative B-cell diseases (n=55) and normal person (n=45) used as control.Results In present series,the value of structural lymphoid parameters (ly-x and ly-z) and the alarm Blast/Abn Lympho were statistically higher in Sezary cases than in control cases (U=37,40,44,55,38,41,all P＜0.05).Screening Sezary cells critical values of ly-x and ly-z determined by ROC curve were greater than or equal to 86 and was more than or equal to 68.The combination of above three indexes up (i.e.alarm Blast/Abn Lympho＞ 100,ly-x=86 and ly-z＞68) sensitivity and specificity respectively for 100 ％ and 92.1 ％,in order to get the best screening Sezary cells diagnostic efficiency.In addition,the value of ly-x was associated to the count of circulating Sezary cells and value of ly-z to the presence of large Sezary cells,both parameters described as prognostic factors.Conclusion The combination of alarm Blast/Abn Lympho and structural parameters (ly-x/ ly-z/ly-y) may allow to define rule of blood slide review to screen circulating Sezary cells.

Expression of growth factor independence 1 in peripheral blood sézary cells of patients with sézary syndrome / 第二军医大学学报

Objective To detect the expression of growth factor independence 1 (GFI-1) in peripheral blood of patients with Sézary syndrome and normal persons, so as to provide a theoretical basis for developing GFI-1 gene target therapy. Methods CD4+ CD7- Sézary cells (SS cells) were separated and purified from peripheral blood of 7 patients with Sézary syndrome by flow cytometry, CD4+ T cells from peripheral blood of 10 normal persons, Sézary syndrome-derived cell line Hut78 and human acute T cell leukemia cell line Jurkat as controls. The mRNA and protein expressions of GF-1were detected by qPCR and Western blotting, respectively. Then after interferon-a-2b (IFN-a2b) was used to induce Hut78 cell apoptosis, the cell proliferation wasmeasured by MTS, themRNA expression of GFI-1, cell cycle-dependent protein kinase inhibitor P21, tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) and Caspasi-3 was detected by qPCR, and the cell apoptosis was detected by flow cytometry. Results The expression of GFI-1 mRNA in the SS cells was significantly higher than that in the Jurkat and CE4+ T cells (all P<0.05). The expression of GF-1 protein in the SS cells and Hut78 cells was significantly higher than that in the Jurkat and CD4+ T cells (all P<0.05). IFN-a2b significantly mhibited the proliferation of Hut78 cells, and the effect was concentration-dependent and time-dependent The mRNA expression of GFI-1 ln Hut78 cells was significantly decreased in a time-dependent manner at 12 h and 24 h treated with IFN-a2b, while the mRNA expressions of P21, TRAIL and Caspase-3 were significantly increased (P〈0. 05). The apoptosis of Hut78 cells was significantly increased at 12 h and 24 h treated with IFN-α2b (P〈0. 05). Conclusion The expression of GFI-1 gene in peripheral blood SS cells of patients with Sézary syndrome is increased and can be inhibited by IFN-a2b, indicating that GFI-1 gene may play an important regulatory role in tumor proliferation of SS cells in patients with Sézary syndrome.

Sezary syndrome – A case report

Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of lymphoproliferative disorders characterised by monoclonal expansion of malignant T cells, primarily helper T (CD4) cells. Mycosis fungoides (MF) and its erythrodermic and leukemic variant, the Sezary syndrome (SS) are the most common clinical types of CTCL. A 48 year old female presented to medical outpatient department with complaints of increasing breathlessness and cough with expectoration of six months duration. She was a known treated case of pulmonary tuberculosis. On examination she had multiple papules and rashes all over the face neck and trunk and generalized lymphadenopathy involving bilateral axillary, cervical and inguinal nodes. Due to the presence of the characteristic triad of erythroderma, lymphadenopathy and circulating atypical lymphoid cells (Sezary cells) and immunophenotypic positivity for T helper subtype, a diagnosis of Sezary syndrome was done.

Alemtuzumab in refractory Sézary syndrome

Abstract: Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response. The treatment was discontinued after 4 months due to adverse effects, with the patient showing a sustained response without disease progression after 13 months of follow-up.

Fotoféresis en el tratamiento de Síndrome de Sézary: Caso clínico / Sézary syndrome treated with extracorporeal photopheresis: Report of one case

Sézary syndrome (SS) is an unusually aggressive T- cell lymphoma characterized by the triad of erythroderma, the presence of more than 1,000 Sézary cells in peripheral blood and lymphadenopathies. It is accompanied by generalized pruritus and poor quality of life. The management of SS depends on its stage, patient comorbidities, and treatment availability. Extracorporeal photopheresis (ECP) is the first line of treatment for patients with T-cell lymphomas in stage IVA1, IVA2 or SS. This treatment comprises three phases: leukapheresis, photoactivation and subsequent reinfusion of lymphocytes. As it is an immunomodulatory therapy it does not produce generalized immunosuppression. We report a 76 year-old male with SS stage IIIb initially treated with 12 sessions of ultraviolet phototherapy without response. After 10 well-tolerated sessions of ECP, itching and skin lesions eventually disappeared.

Actualización en diagnóstico y manejo de micosis fungoide y síndrome de Sézary / Update on diagnosis and management of mycosis fungoides and Sézary's syndrome

Los linfomas cutáneos primarios consisten en una proliferación anormal de linfocitos T o B que muestran tropismo por la piel, sin evidenciarse compromiso extra cutáneo al momento del diagnóstico. Se dividen en linfomas de células T (75 por ciento-80 por ciento) y linfomas de células B (20 por ciento-25 por ciento). La micosis fungoide es una neoplasia de estirpe T y constituye el linfoma cutáneo primario más frecuente. Su presentación clínica clásica consiste en 3 etapas: parche, placa y tumor. Sin embargo, tiene múltiples variantes y un amplio diagnóstico diferencial, por lo que para su diagnóstico se requiere una estricta correlación entre la clínica y la histopatología. El síndrome de Sézary, por su parte, es considerado la variante leucémica de los linfomas cutáneos primarios y forma parte del diagnóstico diferencial de las eritrodermias. En esta revisión profundizaremos en los principales aspectos de la clínica, histopatología, criterios diagnósticos y tratamiento de la micosis fungoide y el síndrome de Sézary.

Primary cutaneous lymphomas represent an abnormal proliferation of T or B-cells with skin-homing ability, with no evidence of extra cutaneous disease at the time of diagnosis. They are divided in T-cell lymphomas (75 percent-80 percent) and B-cell lymphomas (20 percent-25percent). Mycosis fungoides (MF) is a T-cell malignancy, being the most common lymphoma. Classic MF presents 3 clinical phases: patch, plaque and tumor stage. However, it has numerous variants and a wide range of differential diagnosis, so that precise clinicopathologic correlation is necessary for make a correct diagnosis. Sézary syndrome is an aggressive leukemic primary cutaneous T-cell lymphoma variant and it is part of the spectrum of erythroderma. In this review we will analyze the main aspects about clinical presentation, histopathology, diagnosis and treatment of mycosis fungoides and Sézary syndrome.

Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update / Micose fungóide e síndrome de Sézary: revisão e atualização clínica, histopatológica e imuno-histoquímica

This paper reviews the diagnostic and classificatory concepts of mycosis fungoides and Sézary syndrome in light of the latest normative publications. It describes the great variability of the clinical expression of mycosis fungoides in its early stages as well as the histopathological and immunohistochemical aspects that help with diagnosis. The diagnostic criteria required for characterizing Sézary syndrome and the staging system used for both mycosis fungoides and Sézary syndrome are described.

O artigo revisa os conceitos diagnósticos e de classificação da micose fungóide e da síndrome de Sézary a luz das publicações normativas mais recentes. Descreve a grande variabilidade de expressão clinica da micose fungóide em seus estágios iniciais assim como os aspectos histopatológicos e imuno-histoquímicos auxiliares ao diagnóstico. São descritos os critérios de diagnósticos exigidos para que se caracterize a síndrome de Sézary e o sistema de estadiamento, utilizado para ambas, micose fungóide e síndrome de Sézary.

Linfoma cutáneo de células T Revisión del tema con énfasis en la inmunopatogénesis / Cutaneous T-cell lymphoma: Review with emphasis on immunophatogenesis

Bajo el término linfoma cutáneo de células T (LCCT) se agrupa una serie de enfermedades que tienen en común la presencia de un clon maligno de células T y la afectación de la piel. La micosis fungoide (MF) es la forma más común de LCCT, seguida por el síndrome de Sèzary (SS). La causa definitiva de la MF es desconocida. El inmunofenotipo de la mayoría de los casos de MF es de células T de memoria CD3+CD4+CD45RO+CLA+CD8-. Los linfocitos T CD4+ malignos migran hasta la epidermis y se localizan alrededor de las células de Langerhans (LC). En el SS las células pierden el epidermotropismo e infiltran extensamente la dermis, la sangre y otros tejidos; estas células presentan un inmunofenotipo predominante de células T neoplásicas CD3+CD4+CD8-CD7-CD26- CD158k+CD94-. El perfil Th2 y la supresión de respuestas Th1 son dos factores críticos en la progresión de la enfermedad. Se han evaluado muy poco las alteraciones en la regulación inmune en el LCCT. Un mejor entendimiento de la función de las células T y de la inmunobiología de los LCCT permitiría mejorar el pronóstico, el tratamiento y el seguimiento de los pacientes con estas neoplasias.

The term cutaneous T-cell lymphoma (CTCL) refers to several diseases that have in common the presence of a malignant T-cell clone with involvement of the skin. Mycosis fungoides (MF) is the most common form of CTCL, followed by the Sèzary syndrome (SS). The cause of mycosis fungoides is unknown. Immunophenotype of most cases of MF is made up by memory T cells CD3+CD4+CD45RO+CLA+CD8-. Malignant T CD4+ lymphocytes migrate to the epidermis and localize around Langerhans cells. In SS, cells loose their epidermotropism and extensively infiltrate the dermis, blood and other tissues. These cells present a predominant immunophenotype of neoplasic T cells CD3+CD4+CD8-CD7-CD26-CD158k+CD94-. The Th2 profile and the supression of Th1 responses are two critical factors in the progression of the disease. Alterations in the immune regulation in CTCL have not been thoroughly evaluated. A better understanding of T-cell function and of the immunobiology of CTCL would allow to improve the prognosis, therapy and follow-up of patients with these neoplasias.

Generalized Erythroderma as a Manifestation of Sezary Syndrome / 대한피부과학회지

Sezary syndrome is a variant of cutaneous T-cell lymphoma and likely represents the leukemic phase of mycosis fungoides. Sezary syndrome is characterized by generalized erythroderma, pruritus, generalized lymphadenopathy, and circulating atypical cells with cerebriform nuclei. Histopathological features are variable, as this is a later stage in the development of mycosis fungoides. Atypical lymphocytes in the dermis and Pautrier's microabscesses may be present on a histopathological examination but up to one-third of cases may have non-specific findings. Immunological findings supporting a diagnosis of Sezary syndrome include a predominance of CD4+ lymphocytes in both skin biopsy specimens and peripheral blood. In our case, a 48-year-old male was referred for evaluation of generalized hyperpigmentation with exfoliation on his entire body. We suspected that the clinical features represented Sezary syndrome. We obtained a lymph node biopsy, immunological cell typing, and a peripheral blood smear and diagnosed Sezary syndrome.

Es posible curar a un paciente con micosis fungoides? Informe de un caso / Is it possible to cure a patient with mycosis fungoides? A case report

La micosis fungoides y el síndrome de Sezary están constituidos por un grupo de linfomas no Hodgkin indolentes, extranodales, con origen en linfocitos T; afectan la piel de manera primaria; son generalmente incurables y los pacientes tienen mal pronóstico porque la enfermedad es habitualmente refractaria a diversas modalidades terapéuticas. El trasplante de médula ósea alogénico es una opción terapéutica más. La alta mortalidad del acondicionamiento pretrasplante convencional ha hecho que se considere el trasplante alogénico con acondicionamiento de intensidad reducida como una opción viable para esta enfermedad. Se presenta el caso de una paciente con micosis fungoides a quien se hizo un trasplante hematopoyético con un esquema no mieloablativo y en quien se logró remisión completa sostenida de la enfermedad.

Sezary syndrome (SS) and mycosis fungoides (MF) are a group of non Hodgkin lymphomas that originate from T-lymphocytes and involve mostly the skin. These entities are generally non treatable and patient prognosis remains poor even with the advent of current treatment schedules. Complete remissions are seldom observed. For this reason, bone marrow transplant has been used as a treatment option. The high mortality associated with this procedure has turned reduced intensity conditioning stem cell transplant into a treatment option. This case study illustrates how stem cell transplant offers complete remission of this type of lymphomas.

A case report of a patient with mycosis fungoides that affected supra auricular skin and scalp

A Case of Sezary Syndrome / 대한혈액학회지

Sezary syndrome (SS), one of the cutaneous T-cell lymphomas (CTCLs), is an erythrodermic variant of mycosis fungoides (MF). SS is characterized by a pruritic exfoliation or infiltrated erythroderma accompanied by circulating tumor cells with convoluted nuclei in the peripheral blood. The malignant cells in the SS usually have a mature CD4+ T-helper cell phenotype. The reported cases of SS are rare in Korea. We report a case of SS who had intractable erythroderma for 2 years and who subsequently developed generalized lymphadenopathy. The laboratory data revealed a white blood cell count of 61x109/L with 78% of lymphocytes, the majority having a convoluted or cerebriform nucleus. The skin biopsy showed Pautrier's microabscesses with dermal infiltrate of mononuclear cells containing convoluted nuclei. The lymph node biopsy demonstrated paracortical infiltration by convoluted lymphocytes. Consistent with his diagnosis, 92% of peripheral mononuclear cells expressed CD4+.

A Case of Sezary Syndrome / 대한피부과학회지

Sezary syndrome is a variant of cutaneous T-cell lymphoma (CTCL) featuring erythroderma, pruritus, adenopathy and circulating atypical T-lymphocytes. Recent studies suggest that patients with prolonged idiopathic erythroderma, possibility of Sezary syndrome should be considered because in a certain proportion of these patients, they may develop CTCL. We report a case of a 35-year-old man with a prolonged erythroderma for three years. On physical examination, multiple swellings of lymph nodes were noted. Presence of Sezary cells in the peripheral blood, skin and lymph nodes supported the diagnosis of Sezary syndrome.

A Case of Sezary Syndrome / 대한피부과학회지

Patients with idiopathic erythroderma have often been regarded to have a pre-Sezary syndrome because some of them have developed a cutaneous T-cell lymphoma during follow-up. Sezary syndrome is a form of leukemia-lymphoma characterized clinically by erythroderma, pruritus, adenopathy, and circulating atypical cells with cerebriform nuclei. We describe a case of Sezary syndrome in a 40-year-old man, who suffered from idiopathic erythroderma for 3 years. We suggest that close and long-term follow-up should be performed on patients with idiopathic erythroderma.