Here, we report a case of a 49-year-old Japanese man with SS who did not present with erythroderma initially, but exhibited erythematous itchy papules symmetrically located on the legs and arms. [ncbi.nlm.nih.gov]

The clinical presentation, diagnosis, and staging of SS are presented separately, as is the diagnosis and treatment of MF. [uptodate.com]

• Lymphadenopathy

  Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. [ncbi.nlm.nih.gov]

  Definition / general Sézary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and the presence of clonally related neoplastic T cells with cerebriform nuclei (Sézary cells) in skin, lymph nodes and peripheral blood In [pathologyoutlines.com]

  A 66 year old man presented with asymptomatic multiple papules, plaques and nodules and with generalized lymphadenopathy. Peripheral smear showed more than 60% of Sezary cells. [ijdvl.com]

• Fever

  The most commonly reported serious reactions are pneumonia (infection of the lungs), fever, infusion-related reactions and cellulitis (inflammation of the deep skin tissue). [ema.europa.eu]

  Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; outward turning of the eyelids (ectropion); palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly. [diseaseinfosearch.org]

  He had no history of fever, night sweats, weight loss, jaundice, cough, joint pain, previous allergy or any other systemic symptoms. [omicsgroup.org]

  In contrast, Lundin et al. 20 reported grade 3 rigors in 18% of patients, grade 1–2 fever in 68%, grade 3 fever in 5%, and grade 3 fatigue in 9% of patients. [haematologica.org]

  Non-Hodgkin lymphomas can occur at any age and are often marked by lymph nodes that are larger than normal, fever, and weight loss. There are many different types of non-Hodgkin lymphoma. [rarecancers.org.au]

• Short Arm

  Loss of material from the short arms of chromosomes 10 and 8 and from the short and long arms of chromosome 17 are also observations that require detailed analysis at the molecular level. [bloodjournal.org]

• Long Arm

  Partial monosomy for the long arm of chromosome 6 in two patients in the current series is of interest because the presence of at least three tumor-suppressor genes on the long arm of chromosome 6 has been suggested based on three regions of minimal molecular [bloodjournal.org]

• Pruritus

  Serum samples from the peripheral blood of 23 patients and 17 controls were analyzed for IL-31 abundance and correlated with disease stage and pruritus. [ncbi.nlm.nih.gov]

  The patient experiences pruritus, alopecia, edema, and nail and pigment changes. Sézary syndrome Sezary syndrome Mycosis fungoides, see there. [medical-dictionary.thefreedictionary.com]

• Alopecia

  The patient experiences pruritus, alopecia, edema, and nail and pigment changes. Sézary syndrome Sezary syndrome Mycosis fungoides, see there. [medical-dictionary.thefreedictionary.com]

  The skin manifestations at diagnosis varied from patches to tumors often seen in mycosis fungoides, and other rarer findings such as excoriation, palmoplantar keratoderma and alopecia. [ncbi.nlm.nih.gov]

  Alopecia and ectropion are common, exfoliation and heat loss thru the skin are worrisome and infiltration of the lymph nodes occurs. Many of these patients have generalized pruritus and shaking chills at presentation. [dermnet.com]

  Mycosis fungoides-associated follicular mucinosis : must be distinguished from benign follicular mucinosis / "alopecia mucinosa". [e-immunohistochemistry.info]

• Plantar Hyperkeratosis

  hyperkeratosis, onychodystrophy, leonine facies (resembles a lion) Associated with monoclonal gammopathy and secondary neoplasms (carcinoma, other non-Hodgkin lymphomas), attributed to the loss of normal circulating CD4+ cells Skin lesions rarely become [pathologyoutlines.com]

• Follicular Plugging

  A 40-year-old man presented with pruritic plaques affecting his head and trunk, characterized by follicular plugging. The histology was consistent with folliculotropic MF and T-cell gene analysis studies revealed a T-cell clone in the skin only. [ncbi.nlm.nih.gov]

• Peripheral Neuropathy

  We found that TNSc scores significantly correlated with CTCAE grade, with Spearman correlation coefficient 0.68 (p KEYWORDS: Brentuximab vedotin; Chemotherapy-induced peripheral neuropathy; Mycosis fungoides; Neuropathy; Sézary syndrome; Total neuropathy [ncbi.nlm.nih.gov]

  The diagnosis of axonal neuropathy secondary to a neural infiltration by Sézary cells was established. Figure 1 Right peripheral facial paralysis with Bell palsy sign. A, right peripheral facial paralysis; B, Bell palsy sign. [jamanetwork.com]

  neuropathy 0009830 Skeletal muscle atrophy Muscle degeneration Muscle wasting [ more ] 0003202 Tremor 0001337 Showing of 24 | Last updated: 3/1/2020 Although a small percentage of cases of Sezary syndrome are associated with human T-lymphotropic viruses [rarediseases.info.nih.gov]

Currently, the disappearance of markers such as CD26, CD27 and CD7 from the cell surface of malignant T lymphocytes remains the most consistent feature of CTCL and is routinely used in the diagnostic workup. [atlasofscience.org]

(See Pathophysiology, Etiology, Presentation, and Workup.) Patch-stage mycosis fungoides. Plaque-stage mycosis fungoides. Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD. [emedicine.medscape.com]

Lymphomas/European Organization of Research and Treatment of Cancer (ISCL/EORTC) and the American Joint Committee on Cancer recommend assessment of T-cell immunophenotype, including CD26 and CD7, by peripheral blood flow cytometry during the staging workup [ajcp.oxfordjournals.org]

Abstract In order to provide a common standard for the treatment of mycosis fungoides (MF) and Sézary syndrome (SS), the European Organisation for Research and Treatment of Cancer-Cutaneous Lymphoma Task Force (EORTC-CLTF) published in 2006 its consensus [ncbi.nlm.nih.gov]

Treatment Options by Stage For information about the treatments listed below, see the Treatment Option Overview section. [cancer.gov]

Many other clinical, biological and pathological factors may help to distinguish groups at risk and predict prognosis more accurately. [ncbi.nlm.nih.gov]

It presents suddenly and is associated with a poor prognosis. Clinical recognition is crucial for the diagnostic process and initiation of appropriate treatment. [doi.org]

Etiology SS has been linked to a wide range of chromosomal anomalies, in particular rearrangements in the 6q23-27 region leading to alterations in the MYB proto-oncogene and the interleukin-22 receptor subunit alpha-2 gene ( IL22RA2 ), but its etiology [orpha.net]

Further studies are necessary to elucidate the etiology of this entity. [ncbi.nlm.nih.gov]

In this review, we discuss the epidemiology, clinicopathogenetic features and therapeutic approaches in mycosis fungoides and Sézary syndrome. © 2015 Japanese Dermatological Association. [ncbi.nlm.nih.gov]

Macit Ilkit and Murat Durdu, Tinea pedis: The etiology and global epidemiology of a common fungal infection, Critical Reviews in Microbiology, 41, 3, (374), (2015). M. Duvic, L. C. Pinter-Brown, F. M. Foss, L. Sokol, J. L. Jorgensen, P. [doi.org]

Case Name (click to open) Comments Size Case 28 Mycosis Fungoides / Seazary Syndrome 702 kB Epidemiology It is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of [wiki.clinicalflow.com]

The pathophysiologic mechanisms that cause and result in different behaviors of the skin-homing-malignant T cells in different stages of cutaneous T-cell lymphoma (CTCL) are still unknown. [ncbi.nlm.nih.gov]

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Sezary syndrome is a leukemic form of cutaneous T-cell lymphoma (CTCL). [mayomedicallaboratories.com]

(See Pathophysiology, Etiology, Presentation, and Workup.) Patch-stage mycosis fungoides. Plaque-stage mycosis fungoides. Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD. [emedicine.medscape.com]

Despite its utility and duration of use, the current practice of using UVL guidelines for psoriasis to treat patients with MF/SS is problematic because the goals of prolonging survival and preventing disease progression are unique to CTCL compared to [ncbi.nlm.nih.gov]

Radiation therapy – A treatment that aims to kill cancer cells or prevent them from growing through high-energy x-rays. Chemotherapy – The use of drugs to prevent the cancer cells from growing by killing them or stop them from dividing. [syndromespedia.com]

How can Sézary Syndrome be Prevented? Currently, the cause of Sézary Syndrome is unknown. Hence, there are no known methods to prevent the tumor occurrence. Eliminating the modifiable risk factors may help in preventing the condition. [dovemed.com]

[…] comprehensive body of resources on Sezary syndrome. http://rarediseases.info.nih.gov/GARD/Condition/7629/Sezary_syndrome.aspx RD Sezary syndrome information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention [signs-and-symptoms.org]