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Sézary Syndrome

Sezary Syndrome


Presentation

  • Herein, we present a case report of a patient whose initial presentation involved an intramammary lymph node prior to the onset of more characteristic skin disease. Sezary syndrome was confirmed by cythopathologic findings.[ncbi.nlm.nih.gov]
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]
Fever
  • 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]
  • 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.[rarediseases.info.nih.gov]
  • 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]
Ascitic Fluid
Short Arm
  • In an additional patient (no. 10), a reciprocal translocation involved the short arm of chromosome 1 at 1p33, t(1; 4)(p33; p16) (Fig 3 ). In five patients, rearrangements resulted in complete or partial monosomy for the short arm of chromosome 8.[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]
  • We describe a case of a delayed diagnosis of Sezary syndrome in a patient with longstanding generalised pruritus, erythroderma, alopecia and nail dystrophy.[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]
Excoriation
  • 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]
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
  • Abstract Chemotherapy-induced peripheral neuropathy (CIPN) is common, frequently limits chemotherapy dosing, and negatively impacts quality of life.[ncbi.nlm.nih.gov]
  • Comola MNemni RComi G Peripheral neuropathy associated with mycosis fungoides. J Neurol Neurosurg Psychiatry 1989;52 (4) 536- 538 PubMed Google Scholar Crossref 8.[jamanetwork.com]

Workup

  • 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.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]

Treatment

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

Prognosis

  • 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

  • We then discuss updates in the etiology of this elusive disease and the genetic and molecular landscapes that define it.[ncbi.nlm.nih.gov]
  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • (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.com]

Prevention

  • 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]
  • RD Sezary syndrome information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. DermNet NZ Facts about Sezary Syndrome from DermNet NZ.[signs-and-symptoms.org]
  • 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]

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