Non-Classic Signs of Sézary Syndrome: A Review

Morris, Lisa; Tran, Jessica; Duvic, Madeleine

doi:10.1007/s40257-020-00501-7

Cited by 15 publications

(16 citation statements)

References 57 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Mycosis fungoides presents with various clinical stages. The clinical presentation of SS is characterized by the triad of erythroderma (≥ 80 % of the body surface area affected) (Figure 9), lymphadenopathy, and Sézary cells (atypical T cells with cerebriform nuclei) in the skin, lymph nodes, and peripheral blood [61,62]. A recent study reports the following additional clinical features of SS: palmoplantar hyperkeratoses (37.6 %), onychodystrophy (15.6 %), alopecia (10.9 %), leonine facies (3.6 %), and ectropion (3.4 %) [61].…”

Section: Clinical Presentation: Mycosis Fungoides Versus Sézary Syndromementioning

confidence: 99%

Mycosis fungoides and Sézary syndrome

Jonak

Tittes

Brunner

et al. 2021

J Deutsche Derma Gesell

View full text Add to dashboard Cite

Mycosis fungoides (MF) and Sézary syndrome (SS) are primary cutaneous T-cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor-cell burden (Sézary cells) in the peripheral blood consistent with advanced-stage disease and a poor prognosis. In early-stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early-stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin-directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets.

show abstract

Section: Clinical Presentation: Mycosis Fungoides Versus Sézary Syndromementioning

confidence: 99%

Mycosis fungoides and Sézary syndrome

Jonak

Tittes

Brunner

et al. 2021

J Deutsche Derma Gesell

View full text Add to dashboard Cite

show abstract

“…Neoplastische Erkrankungen Morbus Darier [13] Leukämie AML [4] Rhinophym/ Rosazea/Talgdrüsenhyperplasie [14,15] CCL [5,6] Stoffwechselstörungen Sézary-Syndrom [7] Lichen myxoedematosus [16] Mycosis fungoides [8] Granulomatöse Erkrankungen…”

Section: Infektionen Photodermatosenunclassified

Facies leontina bei chronischer lymphatischer Leukämie

Kuske

Hering

Sergon

et al. 2020

Aktuelle Dermatologie

View full text Add to dashboard Cite

ZusammenfassungWir berichten über die seltene Entwicklung einer Leukaemia cutis, die sich als Facies leontina bei einem Patienten mit seit 4 Jahren bekannter chronischer lymphatischer Leukämie manifestierte. In Zusammenschau der Histomorphologie und des immunhistochemischen Phänotyps zeigte sich in der Dermis ein monomorphes Infiltrat von kleinen lymphatischen Zellen mit runden chromatindichten Zellkernen sowie Koexpression von CD20, CD5 und CD23. Dazwischen eingestreut waren einzelne CD3-positive T-Zellen. Obwohl die Facies leontina am häufigsten mit der lepromatösen Lepra oder einem kutanen T-Zell-Lymphom assoziiert ist, sollte insbesondere bei älteren Menschen auch an eine zugrundeliegende Leukämie gedacht werden.

show abstract

“…These lymphocytes are usually CD3 + , CD4 + , CD7 − , and CD26 − cells. 1,2 Treatment is unsatisfactory. alemtuzumab (anti-CD52), and mogamulizumab (anti-CCR4).…”

Section: Casementioning

confidence: 99%

“…Diagnosis of Sezary syndrome is based on the presence of absolute Sezary cell count of more than 1000 cells/mm 3 . These lymphocytes are usually CD3 + , CD4 + , CD7 − , and CD26 − cells 1,2 …”

Section: Casementioning

confidence: 99%

Rare etiology of generalized pruritus: Two cases of Sezary syndrome with review of therapy

Neema

Das

2020

Dermatologic Therapy

View full text Add to dashboard Cite

Generalized pruritus is a common complaint in elderly, rarely due to Sezary syndrome. We hereby present two cases of elderly gentlemen presenting with generalized pruritus of several years duration and were diagnosed to be suffering from Sezary syndrome after extensive evaluation at our centers. Peripheral blood smear Atypical cellspresent Atypical cellspresent CXR PA (Chest X ray, postero-anterior) view Normal Normal Ultrasound abdomen Normal Normal Contrast enhanced CT scan-Chest Normal Mediastinal lymphadenopathy

show abstract

Non-Classic Signs of Sézary Syndrome: A Review

Cited by 15 publications

References 57 publications

Mycosis fungoides and Sézary syndrome

Mycosis fungoides and Sézary syndrome

Facies leontina bei chronischer lymphatischer Leukämie

Rare etiology of generalized pruritus: Two cases of Sezary syndrome with review of therapy

Contact Info

Product

Resources

About