mycosis fungoides
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Author(s):  
Yavuz Semiz ◽  
Ezgi Aktaş ◽  
Mine İlayda Şengör Aygün ◽  
Özben Yalçın

2022 ◽  
pp. 1-6
Author(s):  
Werner Kempf ◽  
Christina Mitteldorf

Kutane T-Zell-Lymphome (CTCL) machen den Großteil aller primären kutanen Lymphome (CL) aus. 80 % aller Fälle von CTCL wiederum entfallen auf Mycosis fungoides (MF) und CD30-positive lymphoproliferative Erkrankungen der Haut. Das klinische Bild der verschiedenen Formen von CTCL zeigt Überschneidungen. Darum ist die klinisch-pathologische Korrelation von großer Bedeutung für die finale Diagnosestellung. Die MF zeigt einen charakteristischen Verlauf mit makuläre Läsionen (Patches), infiltrierte Plaques und, bei einem Teil der Patienten (10–20 %), Tumoren. Die Behandlung erfolgt stadienorientiert mit auf die Haut gerichteten Therapien wie UV-Lichttherapien und Kortikosteroiden in frühen Stadien der Erkrankung (Patch- und begrenztes Plaque-Stadium) und systemischen Therapien (Retinoide bzw. Rexinoide, Interferon, Monochemotherapie, zielgerichtete Therapie) und/oder Strahlentherapie (lokal oder Ganzkörperbestrahlung mit Elektronen) in fortgeschrittenen Stadien. Neuartige Ansätze umfassen zielgerichtete Therapien wie Mogamulizumab (Antikörper gegen CCR4) oder Brentuximab Vedotin (Antikörper gegen CD30) sowie Histon-Deacetylase-Inhibitoren. Angesichts des Impacts von zielgerichteten Therapien sind Biomarker wie CD30 nicht nur für die Diagnosestellung und korrekte Klassifikation eines Lymphoms im Einzelfall von großer Bedeutung, sondern als potenzielle Wirkstoff-Zielmoleküle auch für die Therapie. In der kürzlich überarbeiteten WHO-Klassifikation von 2017 und der aktualisierten WHO-EORTC-Klassifikation der CL von 2018 ist erstmals das CD8-positive akrale T-Zell-Lymphom als eigene, noch provisorische Entität aufgeführt. Diese Form zeigt charakteristische klinische, histologische und phänotypische Merkmale und eine hervorragende Prognose. Zu den seltenen, aber aggressiven CTCL zählen das primär kutane aggressive epidermotrope CD8-positive T-Zell-Lymphom und das kutane Gamma/Delta-T-Zell-Lymphom, die durch rasches Auftreten nekrotischer oder ulzerierender Plaques und Tumoren gekennzeichnet sind. Da bei diesen Formen die Prognose ungünstig ist, umfasst die Behandlung Polychemotherapien und hämatopoetische Stammzelltransplantationen.


2022 ◽  
pp. 1-3
Author(s):  
Monira Abdullah Alnasser ◽  
Nour Marwan AlKhawajah ◽  
Nada Al-Qadri ◽  
Asem Mustafa Shadid ◽  
Fahad M. Alsaif 
Keyword(s):  

Unter dem Begriff der kutanen T-Zell-Lymphome (CTCL) wird eine Gruppe lymphoproliferativer Erkrankungen zusammengefasst, die durch Lokalisation neoplastischer T-Lymphozyten in der Haut gekennzeichnet sind. Die Mycosis fungoides (MF) ist die häufigste Form von CTCL; sie macht ∼60 % aller primären kutanen Lymphome aus. Neben der klassischen Form der MF sind zahlreiche klinische und histopathologische Varianten beschrieben worden. Die malignen Lymphozyten bei MF sind meist positiv für CD3, CD4 und CD45RO und negativ für CD8. Ein ungewöhnlicheres immunhistochemisches Profil eines CD4-negativen und CD8-positiven reifen T-Zell-Phänotyps wird bei einer Minderheit der Patienten beschrieben; in bis zu 20 % der Fälle von MF im Frühstadium liegt ein CD8-positiver Phänotyp vor. Über einen für CD4 und CD8 doppelt-negativen MF-Phänotyp gibt es nur einige wenige Berichte in der Literatur. Wir stellen hier den Fall eines 60-jährigen Mannes mit MF mit CD4/CD8-doppelt-negativem Phänotyp vor.


Author(s):  
Laya Ohadi ◽  
Fatemeh Hosseinzadeh ◽  
Sahar Dadkhahfar ◽  
Soheila Nasiri

The most common variant of cutaneous T-cell lymphomas (CTCL) is mycosis fungoides (MF).The spontaneous regression (SR) of MF is rare. Here, we are reporting an interesting case of refractory MF after COVID-19. The SARS-CoV-2 could be an essential component in the improvement of clinical features related to MF.


2022 ◽  
Author(s):  
Gabriele Roccuzzo ◽  
Francesco Cavallo ◽  
Gianluca Avallone ◽  
Paolo Fava ◽  
Luca Conti ◽  
...  

Der Hautarzt ◽  
2022 ◽  
Author(s):  
Jana Dorothea Albrecht ◽  
Jan Peter Nicolay
Keyword(s):  

2022 ◽  
Vol 13 (1) ◽  
pp. 111-113
Author(s):  
Linda Manaa ◽  
Yosra Soua ◽  
Marwa Thabouti ◽  
Laila Njim ◽  
Monia Youssef ◽  
...  

Sir, Folliculotropic mycosis fungoides (FMF) represents 5% of cutaneous lymphomas. It is a rare variant of mycosis fungoides that differs not only by its clinical and histological presentation but also by its prognosis. It is characterized by an infiltrate of atypical lymphocytes in the perifollicular dermis and hair follicles, with or without mucinosis, while epidermotropism may be completely absent. Dermoscopic and trichoscopic features in FMF are variable and not well defined. Herein, we present a unique case of FMF in a female patient with scalp alopecia, which evolved well under topical treatment. We review its trichoscopic findings. A 64-year-old female presented to our dermatology department with a seven-month history of an alopecic plaque on the scalp. No complaints of itching or burning were made. There was a history of arterial hypertension and dyslipidemia, which has been treated by oral medication. A physical examination revealed an erythematous, non-infiltrated, circumscribed alopecia in the frontal region of the scalp 8 cm in size associated with alopecia of the eyebrows (Fig. 1a and 1b). No other cutaneous lesions were evident. There were no other alopecic or infiltrated plaques, no hyperkeratosis or follicular papules, no acneiform lesions. A histopathological examination of a biopsy specimen revealed an epidermis covered with focally parakeratotic hyperkeratosis. The dermis contained a lymphocytic infiltrate in the follicles and the perifollicular areas. The hair follicles were dissociated by Alcian blue-positive edema. Folliculotropic infiltrate showed positive staining for CD3 and CD4. Some lymphocytes were CD20+ (Fig. 3a – 3c). The clinical, histological, and immunohistochemical appearance was consistent with the diagnosis of folliculotropic MF. Further examination showed no extracutaneous involvement. A full blood count and liver and kidney parameters were found to be in the normal range. A thoraco-abdomino-pelvic CT scan was without abnormality. Referring to the WHO/EORTC classification, the patient’s disease was stage IA. We initiated treatment with a high-potency topical corticosteroid with close monitoring. After two months of treatment, improvement was observed (Fig. 1c). No other cutaneous lesions were evident. There were no other alopecic or infiltrated plaques, no hyperkeratosis or follicular papules, no acneiform lesions. A histopathological examination of a biopsy specimen revealed an epidermis covered with focally parakeratotic hyperkeratosis. The dermis contained a lymphocytic infiltrate in the follicles and the perifollicular areas. The hair follicles were dissociated by Alcian blue-positive edema. Folliculotropic infiltrate showed positive staining for CD3 and CD4. Some lymphocytes were CD20+ (Fig. 3a – 3c). The clinical, histological, and immunohistochemical appearance was consistent with the diagnosis of folliculotropic MF. Further examination showed no extracutaneous involvement. A full blood count and liver and kidney parameters were found to be in the normal range. A thoraco-abdomino-pelvic CT scan was without abnormality. Referring to the WHO/EORTC classification, the patient’s disease was stage IA. We initiated treatment with a high-potency topical corticosteroid with close monitoring. After two months of treatment, improvement was observed (Fig. 1c).


2022 ◽  
Vol 13 (1) ◽  
pp. 114-115
Author(s):  
Asmae Abdelmouttalib ◽  
Fatima Zahra Elghtaibi ◽  
Sanae Sialiti ◽  
Karima Senouci

Sir, Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common malignancies of cutaneous T-cell lymphoma [1]. Herein, we report a case of SS complicated by tumor lysis syndrome and macrophage activation syndrome. A 54-year-old patient, followed since October 2017 for mycosis fungoides and undergoing various treatments (PUVA therapy, methotrexate, chlorambucil + prednisone), presented with an aggravation of lesions toward extensive and intensely pruritic. A clinical examination revealed dry erythroderma, scratch marks, wart plaques, an accentuation of frontal wrinkles and nasolabial folds (leonine facies), palmoplantar fissuring keratoderma, xanthopachyonychia of all nails, and a carapace-like appearance of the scalp (Figs. 1 – 3). Generalized lymphadenopathy, hepatomegaly, and a state of anasarca-type edema caused by hypoalbuminemia were also found. A skin biopsy revealed lymphoproliferation of CD4+ T-cells and an aberrant loss of pan-T antigens. The CD4-to-CD8 ratio was at 48.5% and Sézary cells were 6960 (absolute value). A lymph node biopsy showed a dense infiltration of Sézary cells. A PET scan revealed hypermetabolism in the entire skin and at the lymph node level. Tumor lysis syndrome was evident, with high levels of blood uric acid (at 182 mg/L), elevated LDH (at 924 U/L), and functional kidney failure. Macrophage activation syndrome was also present, with fever, anemia and thrombocytopenia, liver cytolysis, hypertriglyceridemia, and hyperferritinemia. The patient received an albumin infusion, oral corticosteroid therapy to treat the syndrome, and rasburicase for hyperuremia. Despite this, the patient died before multiagent chemotherapy could have been started. On rare occasions, SS may be preceded by a prior history of classic MF. The International Society for Cutaneous Lymphomas (ISCL) recommends that such cases be designated “SS preceded by MF” [2]. Traditionally, SS is defined as a leukemic form of CTCL associated with erythroderma. Sézary cells are a population of large lymphocytes in the peripheral blood, with grooved and lobulated nuclei, in the case of SS, numbering 1000 cells/mL or more [2]. The histopathologic findings in the skin often resemble those observed in MF, with less prominent epidermotropism. As in MF, immunohistochemical studies showing a CD4 predominance and loss of pan-T-cell markers may be helpful. Lymph node involvement is characterized by the complete effacement of the nodal architecture by the infiltrating Sézary cells (2). The poor prognostic factors in Sézary syndrome include an advanced stage of the disease, an older age at onset, and large cell transformation [3]. While high response rates may be achieved with systemic chemotherapy, they are frequently short-lived and associated with significant toxicities [2]. The management of SS is complicated and requires multidisciplinary collaboration between dermatologists, hematologists, biologists, and reanimators.


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