Childhood mycosis fungoides with a <scp>CD8</scp>+ <scp>CD56</scp>+ cytotoxic immunophenotype

Poppe, H; Kerstan, Andreas; Böckers, Martin; Goebeler, Matthias; Geissinger, Eva; Rosenwald, Andreas; Hamm, Henning

doi:10.1111/cup.12452

Cited by 16 publications

(15 citation statements)

References 33 publications

(91 reference statements)

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“…Types A, B, C and E all show a CD4+ predominant but mixed T-cell population while Type D is characteristically CD8 positive. Cytotoxic markers can be positive, including CD56 [50]. TCR beta or gamma [39] can be found.…”

Section: Clinical Presentationmentioning

confidence: 99%

Cutaneous T-cell Lymphoma

Pulitzer

2017

Clinics in Laboratory Medicine

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Synopsis Cutaneous T-cell lymphomas comprise a heterogeneous group of diseases characterized by monoclonal proliferations of T-lymphocytes primarily involving skin, modified skin appendages and some mucosal sites. This review addresses the basic clinical, histologic and immunohistochemical characteristics of this group of diseases, with additional attention to evolving literature on dermoscopy, reflectance confocal microscopy, flow cytometry and molecular data that may increasingly be applied to diagnostic and therapeutic algorithms in these diseases. Select unusual phenotypes, or diagnostic examples of classic phenotypes are demonstrated, and flags for consideration while making a pathologic diagnosis of CTCL are suggested.

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Section: Clinical Presentationmentioning

confidence: 99%

Cutaneous T-cell Lymphoma

Pulitzer

2017

Clinics in Laboratory Medicine

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“…17 Expression of CD56 has been documented only in sporadic case reports. [18][19][20][21][22][23][24][25] Interestingly, also on the molecular level, variability in TCRg gene rearrangement has been described in concurrent biopsies of MF from the same patient. 26 Our findings suggest that in a single patient, there may be substantial phenotypic variation between lesions of MF present at the same time.…”

Section: Discussionmentioning

confidence: 98%

Phenotypic Variation in Different Lesions of Mycosis Fungoides Biopsied Within a Short Period of Time From the Same Patient

Kash

Massone

Fink‐Puches

et al. 2016

The American Journal of Dermatopathology

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Phenotypic variants of mycosis fungoides (MF) include mainly the expression of cytotoxic markers by neoplastic cells (either α/β or γ/δ cytotoxic). To manage the patient properly, distinction from other cutaneous cytotoxic natural killer/T-cell lymphomas is paramount. Particularly for cutaneous γ/δ T-cell lymphoma, distinction is often based on clinicopathologic correlation (presence of tumors at first diagnosis as opposed to patches only in MF). The authors report a case of cytotoxic MF characterized by expression of TCRγ in two of three biopsies performed within a time frame of 1 week. The patient presented with patches, plaques, and 1 tumor at the time of first diagnosis; thus, distinction from cutaneous γ/δ T-cell lymphoma was not possible on clinical grounds alone. The diagnosis of MF was established, thanks to the phenotypic variations revealed by the three biopsies, with 1 lacking expression of cytotoxic proteins (TIA-1 and granzyme B) and of TCRγ. This case shows the importance to perform several biopsies in cases of cutaneous lymphoma, as morphologic and phenotypic features are variable and information gathered from a single biopsy may result in a wrong diagnosis.

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“…From the histopathological viewpoint, this MF variant requires the differential diagnosis with other cytotoxic and natural killer lymphomas including primary cutaneous aggressive CD8+ T‐cell lymphoma, CD8+ pagetoid reticulosis, lymphomatoid papulosis type D, and in rare cases, epidermotropic CD8+ y/δ+T‐cell lymphoma . Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T‐cell lymphoma is defined clinically by the abrupt presentation of extensive annular plaques with erosions and ulcerations, and by an aggressive course.…”

Section: Discussionmentioning

confidence: 99%

Pediatric CD8+/CD56+ mycosis fungoides with cytotoxic marker expression: A variant with indolent course

2018

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Mycosis fungoides is predominantly a disease of older patients, but occasionally occurs in children. We report a rare case of CD8+/CD56+ mycosis fungoides with cytotoxic marker (perforin, TIA-1, and granzyme B) expression in a 10-year-old boy. Disease presented with three asymptomatic, slowly progressive erythematous and scaling plaques, surrounded by hypochromic alone in the left tight and lower trunk. UVB narrow band associated with topical corticosteroids resulted in complete remission in about 2 months, and no recurrence at 2-year follow-up. Three similar cases have been retrieved in children through PubMed search, showing similar clinical presentation with erythematous scaling lesions, good response to skin-directed treatments and a favorable prognosis.

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Childhood mycosis fungoides with a CD8+ CD56+ cytotoxic immunophenotype

Cited by 16 publications

References 33 publications

Cutaneous T-cell Lymphoma

Cutaneous T-cell Lymphoma

Phenotypic Variation in Different Lesions of Mycosis Fungoides Biopsied Within a Short Period of Time From the Same Patient

Pediatric CD8+/CD56+ mycosis fungoides with cytotoxic marker expression: A variant with indolent course

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