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1.
An Bras Dermatol. 2018 Sep-Oct;93(5):680-685. doi: 10.1590/abd1806-4841.20187134.

Evaluation of the Cutaneous Lymphoma International Prognostic Index in patients with early stage mycosis fungoides.

Author information

1
Program of Post-graduation in Anatomical Pathology, Hospital Universitário Clementino Fraga Filho, Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.
2
Outpatient clinic of Dermatology, Faculdade de Medicina, Universidade do Sul de Santa Catarina, Palhoça (SC), Brazil.
3
Dermatology Division, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.
4
Pathology Division, Hospital Federal de Bonsucesso, Rio de Janeiro (RJ), Brazil.
5
Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.
6
Instituto Nacional de Infectologia Evandro Chagas, Fundação Oswaldo Cruz, Rio de Janeiro (RJ), Brazil.
7
Discipline of Dermatology, Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

Abstract

BACKGROUND:

Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma. TNMB system is the staging method used in MF, and it not only guides therapeutic management, but represents the main prognostic factor. In order to improve the prognostic evaluation, the Cutaneous Lymphoma International Prognostic Index (CLIPi) was proposed.

OBJECTIVE:

To evaluate the performance of CLIPi score for prognostic analysis in patients with early stage MF.

METHODS:

This is a retrospective cross-sectional observational study, with exploratory analysis. The outcome variables were disease progression and related death.

RESULTS:

One hundred and two patients were stratified according to CLIPi score, being the majority classified as low risk. Patients with intermediate or high risk presented disease progression more frequently than those with low risk (PR: 1.2 / p = 0.004 / 95%CI: 1.0 - 1.6). The same did not occur with the variable related death. In addition, survival rates were not consistent with risk stratification.

STUDY LIMITATIONS:

Small sample and its retrospective analysis.

CONCLUSIONS:

Since CLIPi score was proposed, four other studies that we could consult showed conflicting results, similar to the present study. Further studies are necessary for a recommendation of its use.

PMID:
30156617
PMCID:
PMC6106654
DOI:
10.1590/abd1806-4841.20187134
[Indexed for MEDLINE]
Free PMC Article
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2.
An Bras Dermatol. 2018 Jul-Aug;93(4):546-552. doi: 10.1590/abd1806-4841.20187106.

Clinical and epidemiological profile of patients with early stage mycosis fungoides.

Author information

1
Master's student, Postgraduate Program in Anatomical Pathology, Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Hospital Universitário Clementino Fraga Filho, Rio de Janeiro (RJ), Brazil.
2
Dermatology Outpatient Clinic, Faculdade de Medicina, Universidade do Sul de Santa Catarina, Palhoça (SC), Brazil.
3
Dermatology Service, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.
4
Pathology Service, Hospital Federal de Bonsucesso, Rio de Janeiro (RJ), Brazil.
5
Department of Pathology, Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.
6
Instituto Nacional de Infectologia Evandro Chagas - Fundação Oswaldo Cruz, Rio de Janeiro (RJ), Brazil.
7
Discipline of Dermatology, Faculdade de Medicina da Universidade Federal do Rio de Janeiro, Rio de Janeiro (RJ), Brazil.

Abstract

BACKGROUND:

Mycosis fungoides is the most common form of primary cutaneous lymphoma, with an indolent, slowly progressive course and 88% five-year survival rate. The diagnosis is challenging, especially in the early stages, and usually relies on a good clinical-histopathological correlation.

OBJECTIVE:

The aim was to establish the clinical and epidemiological profile of patients with early-stage mycosis fungoides.

METHODS:

This was a retrospective cross-sectional observational study with an exploratory analysis. Outcome variables were disease progression and mycosis fungoides-related death.

RESULTS:

One hundred and two patients were included. The majority were white males, with a mean age of 55.6 years. Mean time from onset of lesions to diagnosis was 51.08 months. The majority of patients were classified as IB stage according to TNMB. Mean follow-up time was 7.85 years. Disease progression was seen in 29.4% of the patients. Death related to the disease occurred in 7.9% of patients. Plaque lesions, involvement of more than 10% of the body surface, altered lactate dehydrogenase and beta-2-microglobulin, and stage IB were significantly associated with disease progression, and altered lactate dehydrogenase and beta-2-microglobulin also correlated with higher frequency of deaths.

STUDY LIMITATIONS:

Small sample and retrospective design.

CONCLUSIONS:

The clinical and epidemiological profile of patients with early-stage mycosis fungoides in our sample corroborates reports in the literature. Diagnostic delay in our series is also consistent with previous findings, but the rate of disease progression, despite treatment, was higher than reported in the literature.

PMID:
30066762
PMCID:
PMC6063099
DOI:
10.1590/abd1806-4841.20187106
[Indexed for MEDLINE]
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3.
Clin Nucl Med. 2018 Aug;43(8):606-608. doi: 10.1097/RLU.0000000000002166.

Imaging of Chemokine Receptor CXCR4 in Mycosis Fungoides Using 68Ga-Pentixafor PET/CT.

Author information

1
From the Department of Nuclear Medicine, Istanbul Medical Faculty, and.
2
Faculty of Health Sciences, Istanbul University, Istanbul, Turkey.

Abstract

A 52-year-old woman diagnosed with mycosis fungoides was referred for F-FDG PET/CT before treatment for the evaluation of disease severity. The patient also underwent Ga-pentixafor PET/CT for further evaluation. FDG uptake was observed in cervical, axillary, and pelvic lymph nodes and multiple widespread skin lesions throughout the body, suggestive of extensive involvement of mycosis fungoides. All lesions were visually identifiable with high target-to-background ratio on Ga-pentixafor PET/CT which demonstrated marked CXCR4 expression in all lesions that were detected using F-FDG PET/CT.

PMID:
29916918
DOI:
10.1097/RLU.0000000000002166
[Indexed for MEDLINE]
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4.
Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2017 Dec 5;31(23):1824-1827;1832. doi: 10.13201/j.issn.1001-1781.2017.23.011.

[Imaging charateristics of bony erosion and fungal culture analysis in allergic fungal rhinosinusitis].

[Article in Chinese]

Author information

1
Department of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, 100073, Beijing, China.
2
Department of Otorhinolaryngology, the First Hospital of Harbin.

Abstract

Objective:To investigate the imaging characteristics of bony erosion, fungal culture and related factors in allergic fungal rhinosinusitis (AFRS). Method:Sixty cases of AFRS were reviewed in this study. The characteristics of Computed Tomography and Magnetic Resonance Imaging including positive rate of bony erosion, eroded sites, disease extension and sinus expansion were summarized. Fungal culture and identification of nasal secretion were done. The correlation between the degree of sinus expansion, species of fungi and bony erosion was evaluated by statistical analysis. Result:Of the 60 patients, 18 (30%) had bony erosion. There was a significant difference in the proportion of bone erosion among the sinuses (P<0.05). The most commonly eroded site was the ethmoid sinus. The orbit were the most common adjacent anatomic spaces to exhibit disease extension, and anterior cranial fossa is the second most common. Statistical analysis revealed a significant association(P<0.05)of bone erosion with sinus expansion. The positive rate of fungal culture was 51.3%, among which the most common is Aspergillus. Statistical analysis shows no association(P>0.05)of bone erosion with specific fungi. Conclusion:Bone erosion is an important imaging feature of AFRS. Bone erosion may cause by sinus expansion. Extension of disease into the orbit or intracranial cavity results from a natural progression of disease after erosion occurs. Specific fungal species that can cause bone erosion was found.

KEYWORDS:

bony erosion; computed tomography; fungi; magnetic resonance imaging; sinusitis

[Indexed for MEDLINE]
5.
Medicine (Baltimore). 2018 May;97(21):e10871. doi: 10.1097/MD.0000000000010871.

Oncogenomic analysis identifies novel biomarkers for tumor stage mycosis fungoides.

Author information

1
Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College.
2
Department of Dermatology, Zhongda Hospital, Southeast University, Nanjing, Jiangsu.
3
Department of Dermatology, The Affiliated Qingdao Municipal Hospital of Qingdao University, Qingdao, China.

Abstract

Patients with mycosis fungoides (MF) developing tumors or extracutaneous lesions usually have a poor prognosis with no cure has so far been available. To identify potential novel biomarkers for MF at the tumor stage, a genomic mapping of 41 cutaneous lymphoma biopsies was used to explore for significant genes.The gene expression profiling datasets of MF were obtained from Gene Expression Omnibus database (GEO). Gene modules were simulated using Weighted Gene Co-expression Network Analysis (WGCNA) and the top soft-connected genes (hub genes) were filtrated with a threshold (0.5). Subsequently, module eigengenes were calculated and significant biological pathways were enriched based on the KEGG database.Four genetic modules were simulated with 3263 genes collected from the whole genomic profile based on cutoff values. Significant diseases genetic terminologies associated with tumor stage MF were found in black module. Subsequently, 13 hub genes including CFLAR, GCNT2, IFNG, IL17A, IL22, MIP, PLCG1, PTH, PTPN6, REG1A, SNAP25, SUPT7L, and TP63 were shown to be related to cutaneous T-cell lymphoma (CTCL) and adult T-cell lymphoma/leukemia (ATLL).In summary, in addition to the reported genes (IL17F, PLCG1, IFNG, and PTH) in CTCL/ATLL, the other high instable genes may serve as novel biomarkers for the regulation of the biological processes and molecular mechanisms of CTLT (MF/SS).

PMID:
29794791
DOI:
10.1097/MD.0000000000010871
[Indexed for MEDLINE]
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6.
In Vivo. 2018 May-Jun;32(3):517-522.

Total Skin Electron Beam Therapy with Rotary Dual Technique as Palliative Treatment for Mycosis Fungoides.

Author information

1
Department of Electroradiology, Poznan University of Medical Sciences, Poznan, Poland tomasz.piotrowski@wco.pl.
2
Department of Medical Physics, Greater Poland Cancer Centre, Poznan, Poland.
3
1st Radiotherapy Ward, Greater Poland Cancer Centre, Poznan, Poland.
4
Department of Geriatrics and Gerontology, Poznan University of Medical Sciences, Poznan, Poland.

Abstract

BACKGROUND/AIM:

The aim of the study was to retrospectively assess the efficacy and toxicity of total skin electron beam therapy (TSEBT) in patients with primary cutaneous T-cell lymphoma (MF, mycosis fungoides) at various stages of development.

PATIENTS AND METHODS:

Treatment results of 40 patients with MF stage IB-III, treated between 2001 and 2015, were reviewed. Median total dose was 32 Gy, delivered to the entire skin surface. Median follow-up was 60 months.

RESULTS:

Clinical complete response was documented in 29 and partial response in 11 patients. The clinical response significantly influenced overall survival (OS) (p=0.002) and progression-free survival (PFS) (p<0.001). Mean OS was 76 months. Mean PFS was 48.9 months and current one- and two-year PFS were 67.5% and 55%, respectively. A statistically significant correlation was found between partial and total remission time and stages of the lymphoma (p=0.015).

CONCLUSION:

TSEBT is an efficient and well-tolerated palliative treatment for symptomatic primary cutaneous T-cell lymphoma.

KEYWORDS:

Mycosis fungoides; overall survival; progression free survival; radiotherapy; toxicity

PMID:
29695554
PMCID:
PMC6000802
DOI:
10.21873/invivo.11269
[Indexed for MEDLINE]
Free PMC Article
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7.
Med Phys. 2018 Jun;45(6):2639-2646. doi: 10.1002/mp.12917. Epub 2018 May 6.

Improving treatment geometries in total skin electron therapy: Experimental investigation of linac angles and floor scatter dose contributions using Cherenkov imaging.

Author information

1
Thayer School of Engineering, Dartmouth College, Hanover, NH, 03755, USA.
2
Department of Radiation Oncology, Thomas Jefferson University, Philadelphia, PA, 19107, USA.
3
Norris Cotton Cancer Center, Dartmouth-Hitchcock Medical Center, Lebanon, NH, 03766, USA.
4
Geisel School of Medicine, Dartmouth College, Hanover, NH, 03755, USA.
5
Department of Radiation Oncology, Washington University School of Medicine, St. Louis, MO, 63110, USA.
6
Thayer School of Engineering and Department of Physics and Astronomy, Dartmouth College, Hanover, NH, 03755, USA.

Abstract

PURPOSE:

The purpose of this study was to identify the optimal treatment geometry for total skin electron therapy (TSET) using a new optimization metric from Cherenkov image analysis, and to investigate the sensitivity of the Cherenkov imaging method to floor scatter effects in this unique treatment setup.

METHODS:

Cherenkov imaging using an intensified charge coupled device (ICCD) was employed to measure the relative surface dose distribution as a 2D image in the total skin electron treatment plane. A 1.2 m × 2.2 m × 1 cm white polyethylene sheet was placed vertically at a source to surface distance (SSD) of 300 cm, and irradiated with 6 MeV high dose rate TSET beams. The linear accelerator coordinate system used stipulates 0° is the bottom of the gantry arc, and progresses counterclockwise so that gantry angle 270° produces a horizontal beam orthogonal to the treatment plane. First, all unique pairs of treatment beams were analyzed to determine the performance of the currently recommended symmetric treatment angles (±20° from the horizontal), compared to treatment geometries unconstrained to upholding gantry angle symmetry. This was performed on two medical linear accelerators (linacs). Second, the extent of the floor scatter contributions to measured surface dose at the extended SSD required for TSET were imaged using three gantry angles of incidence: 270° (horizontal), 253° (-17°), and 240° (-30°). Images of the surface dose profile at each angle were compared to the standard concrete floor when steel plates, polyvinyl chloride (PVC), and solid water were placed on the ground at the base of the treatment plane. Postprocessing of these images allowed for comparison of floor material-based scatter profiles with previously published simulation results.

RESULTS:

Analysis of the symmetric treatment geometry (270 ± 20°) and the identified optimal treatment geometry (270 + 23° and 270 - 17°) showed a 16% increase in the 90% isodose area for the latter field pair on the first linac. The optimal asymmetric pair for the second linac (270 + 25° and 270 - 17°) provided a 52% increase in the 90% isodose area when compared to the symmetric geometry. Difference images between Cherenkov images captured with test materials (steel, PVC, and solid water) and the control (concrete floor) demonstrated relative changes in the two-dimensional (2D) dose profile over a 1 × 1.9 m region of interest (ROI) that were consistent with published simulation data. Qualitative observation of the residual images demonstrates localized increases and decreases with respect to the change in floor material and gantry angle. The most significant changes occurred when the beam was most directly impinging the floor (gantry angle 240°, horizontal -30°), where the PVC floor material decreased scatter dose by 1-3% in 7.2% of the total ROI area, and the steel plate increased scatter dose by 1-3% in 7.0% of the total ROI area.

CONCLUSIONS:

An updated Cherenkov imaging method identified asymmetric, machine-dependent TSET field angle pairs that provided much larger 90% isodose areas than the commonly adopted symmetric geometry suggested by Task Group 30 Report 23. A novel demonstration of scatter dose Cherenkov imaging in the TSET field was established.

KEYWORDS:

Cerenkov; commissioning; optimization; total skin irradiation

PMID:
29663425
DOI:
10.1002/mp.12917
[Indexed for MEDLINE]
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8.
Radiat Oncol. 2018 Apr 13;13(1):67. doi: 10.1186/s13014-018-1013-2.

Bone marrow suppression as a complication of total skin helical tomotherapy in the treatment of mycosis fungoides.

Author information

1
Michigan State University College of Human Medicine, 418 W. Magnetic Street, Marquette, MI, 49855, USA.
2
Department of Human Oncology, University of Wisconsin, Madison, WI, USA.
3
Department of Human Oncology, University of Wisconsin, Madison, WI, USA. kabradley@humonc.wisc.edu.

Abstract

BACKGROUND:

Total skin electron beam therapy (TSEBT) is an effective treatment in mycosis fungoides. Total skin helical tomotherapy (TSHT) may be an alternative to TSEBT and may offer several dosimetric and treatment advantages. There are currently very few published treatment results using TSHT in place of TSEBT for treatment of mycosis fungoides.

CASE PRESENTATION:

Two patients with mycosis fungoides were treated at our institution using TSHT. The first patient was a 69-year-old Caucasian female with stage IVA2 (T2 N3 M0 B2) disease who was treated to a dose of 12 Gy in 8 fractions, with a bone marrow mean dose of 1.66 Gy and V10 = 0.41%. Two weeks after ending treatment the patient developed myelosuppression including grade 4 thrombocytopenia and required blood and platelet transfusions. The second patient was a 29-year-old Caucasian female with stage I (T2 N0 M0 B0) disease. This patient previously had been treated for mycosis fungoides using helical tomotherapy (HT) at a dose of 20 Gy to a localized region and experienced mild thrombocytopenia at that time. The patient then underwent retreatment 17 months later with TSHT to a dose of 12 Gy in 6 fractions with a mean bone marrow dose of 2.3 Gy and V10 = 4.28%. This patient once again experienced myelosuppression that included grade 4 thrombocytopenia. She also required blood and platelet transfusions.

CONCLUSIONS:

Both patients treated with TSHT experienced severe bone marrow suppression including grade 4 thrombocytopenia. This was more severe than expected considering the relatively low overall prescription dose and despite a planning constraint placed on the bone marrow of a mean dose of < 2 Gy. These outcomes suggest that patients treated using TSHT should be closely monitored for myelosuppression and caution used even when treating to a dose of 12 Gy.

KEYWORDS:

Cutaneous lymphoma; Helical tomotherapy; Mycosis fungoides; T-cell lymphoma; Total skin electron beam therapy

PMID:
29653544
PMCID:
PMC5899362
DOI:
10.1186/s13014-018-1013-2
[Indexed for MEDLINE]
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9.
An Bras Dermatol. 2018 Jan-Feb;93(1):33-38. doi: 10.1590/abd1806-4841.20185468.

The efficacy and safety of phototherapy in geriatric patients: a retrospective study.

Author information

1
Department of Dermatology, Faculty of Medicine, Eskisehir Osmangazi University, Eskişehir, Turkey.
2
Deparrment of Dermatology, Istanbul Training and Research Hospital, Istanbul, Turkey.

Abstract

BACKGROUND:

While phototherapy is a well-established treatment for many dermatoses, data from the literature regarding its use in elderly patients are quite limited.

OBJECTIVE:

In this study, we aimed to determine the phototherapy indications in geriatric patients and to evaluate the effectiveness and reliability of phototherapy in this group.

METHODS:

This study included 95 patients of 65 years of age and older who were treated in our phototherapy unit between 2006 and 2015. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit.

RESULTS:

Phototherapy was administered to 28 (29.5%) patients for mycosis fungoides, 25 (26.3%) patients foplaque type psoriasis, 12 (12.6%) patients for palmoplantar psoriasis, 12 (12.6%) patients for generalized pruritus, and 18 (19%) for other dermatoses. Of the patients, 64.2% had received a narrowband UVB (NB-UVB), 21.1% oral psoralen UVA (PUVA), and 14.7% local PUVA treatment. A complete response was achieved in 76.9-85.7% of the mycosis fungoides and in 73.71-100% of the psoriasis vulgaris patients treated with NB-UVB and PUVA, respectively. All the patients with generalized pruritus were treated with NB-UVB, and 80% of these patients achieved significant improvement. The erythema rate was found to be 0.43% per session for NB-UVB treatment and 0.46% per session for PUVA treatment as a side effect.

STUDY LIMITATIONS:

The limitations of our study are that it was retrospective and the remission durations of the patients are not known.

CONCLUSION:

This study showed that phototherapy is effective and reliable in the elderly population with proper dose increases and close follow-up.

PMID:
29641694
PMCID:
PMC5871359
DOI:
10.1590/abd1806-4841.20185468
[Indexed for MEDLINE]
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10.
Int J Dermatol. 2018 Jun;57(6):675-680. doi: 10.1111/ijd.13967. Epub 2018 Mar 30.

Immune privilege disruption in folliculotropic mycosis fungoides: investigation of major histocompatibility complex antigen expression.

Author information

1
Department of Dermatology, University of São Paulo Medical School, São Paulo, Brazil.
2
Department of Dermatopathology, Northwestern University, Chicago, USA.
3
Department of Dermatology, Federal University of Parana, Curitiba, Brazil.

Abstract

BACKGROUND:

Folliculotropic mycosis fungoides (FMF) is a cutaneous T-cell lymphoma mainly affecting the hair follicle, which seems to represent a place of immune privilege phenomenon.

OBJECTIVES:

To explore a possible role of immune privilege (IP) in FMF analyzing the major histocompatibility complex (MHC) expression.

METHODS:

Immunohistochemistry for HLA-G and MHC-II was performed to formalin-fixed paraffin-embedded cutaneous skin biopsies of FMF patients (n = 43), conventional mycosis fungoides (CMF; n = 13), alopecia areata (AA; n = 13), and normal scalp skin (NS; n = 12).

RESULTS:

HLA-G expression was lower in FMF (34%: 14/41) and CMF (18%: 2/11) groups compared to alopecia areata (92%:11/12) and normal scalp skin group (100%: 12/12). MHC-II expression in hair follicle was greater in the FMF group (18/42: 43%) compared to AA (0%) and NS (0%). HLA-G and MHC-II expression in cellular infiltrate had no difference among FMF and CMF groups and was different compared to the AA group.

CONCLUSIONS:

Our data support the hypothesis of disruption of immune privilege based on the lower expression of HLA-G and higher expression of MHC-II in the follicular epithelium in mycosis fungoides compared to alopecia areata and normal scalp skin. The lack of difference between FMF and CMF groups did not support the role of these molecules as a driver of folliculotropism. The expression of MHC molecules seems to be different between neoplastic and inflammatory infiltrates. The definitive significance of expression of the MHC molecules remains unclear, and more studies are necessary to fully understand the role of these molecules in cutaneous lymphomas.

PMID:
29603194
DOI:
10.1111/ijd.13967
[Indexed for MEDLINE]
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11.
Cutis. 2018 Feb;101(2):E4-E6.

Hypopigmented discoloration on the thigh.

Author information

1
Dermatology Associates, Inc, Perrysburg, Ohio; Department of Pathology, College of Medicine and Life Sciences, University of Toledo, Ohio, USA.
2
Department of Pathology, College of Medicine and Life Sciences, University of Toledo, Ohio, USA.
PMID:
29554167
[Indexed for MEDLINE]
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12.
J Cutan Pathol. 2018 Jun;45(6):458-462. doi: 10.1111/cup.13140. Epub 2018 Apr 6.

Tumor-stage mycosis fungoides in palmoplantar localization with large-cell transformation and partial CD30 expression shows complete response to brentuximab vedotin.

Author information

1
Section of Dermatology, Department of Surgery, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine, Lebanon, New Hampshire.
2
Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine, Lebanon, New Hampshire.
3
Department of Pathology and Laboratory Medicine, White River Junction VA Medical Center, White River Junction, Vermont.
4
Section of Hematology and Oncology, Department of Medicine, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine, Lebanon, New Hampshire.
5
Department of Hematology and Oncology, White River Junction VA Medical Center, White River Junction, Vermont.
6
Department of Dermatology, White River Junction VA Medical Center, White River Junction, Vermont.
7
Department of Radiation Oncology, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine, Lebanon, New Hampshire.

Abstract

Mycosis fungoides in palmoplantar localization (MFPP) is a rare variant of MF that is confined to the hands and feet. Patients commonly receive treatment over many years for suspected palmoplantar dermatitis before the diagnosis is made. Most MFPP patients remain at patch or plaque stage, and often respond to treatment with radiotherapy. Herein, we describe a 77-year-old man who suffered 6 years of hand and foot dermatitis that failed multiple treatments, most notably TNF-α inhibitors and mycophenolate mofetil. He eventually developed a tumor on the hand, which was biopsied to reveal a dense dermal infiltrate of large lymphocytes (CD3+/CD4-/CD8-/TCR-BetaF1+/partial CD30+). A subsequent biopsy of an eczematous patch from his hand revealed an epidermotropic and syringotropic infiltrate comprised of smaller lymphocytes with a concordant immunophenotype and matching clonal peak with TCR gene rearrangement. He was diagnosed with MFPP and started on radiotherapy with a modest response; therefore, a decision was made to start brentuximab vedotin, which resulted in a complete response. MFPP is an exceedingly rare variant of MF that can show large-cell transformation and progress in stage. We highlight a possible association between disease progression and immunosuppressants and the potential role for treatment with brentuximab.

KEYWORDS:

TNF-α inhibitors; brentuximab vedotin; large cell transformation; mycosis fungoides in palmoplantar localization; mycosis fungoides palmaris et plantaris

PMID:
29512830
DOI:
10.1111/cup.13140
[Indexed for MEDLINE]
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13.
Am J Dermatopathol. 2018 Mar;40(3):185-190. doi: 10.1097/DAD.0000000000000955.

Index Case of Cutaneous Follicular Mycosis Fungoides With Central Nervous System Involvement and Review of Literature.

Author information

1
Department of Dermatology, University of Colorado Anschutz Medical College, Aurora, CO.
2
Department of Neurology, Alpert Medical School, Brown University, Providence, RI.
3
Department of Neurology, University of Colorado Anschutz Medical College, Aurora, CO.
4
Department of Dermatology, Weill Cornell Medical College, New York, NY.
5
Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
6
Department of Pathology, University of Colorado Anschutz Medical College, Aurora, CO.
7
Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY.

Abstract

Central nervous system involvement by mycosis fungoides (MF) is rare and is usually seen in advanced stages of the disease. We describe a patient with early-stage follicular MF who presented with changes in mental status. Despite an initial diagnosis of vasculitis based on clinical and brain biopsy results, the postmortem examination revealed extensive infiltration of MF cells throughout the brain with leptomeningeal involvement. This case in addition to the accompanied review of literature illustrates the importance of the awareness of central nervous system involvement by MF and highlights the need for an urgent neurologic evaluation in patients with a history of MF now presenting with neurologic signs or symptoms.

PMID:
29470304
DOI:
10.1097/DAD.0000000000000955
[Indexed for MEDLINE]
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14.
Dermatol Online J. 2018 Jan 15;24(1). pii: 13030/qt9kq75581.

Eosinophilic infiltrate resembling eosinophilic cellulitis (Wells syndrome) in a patient with mycosis fungoides.

Author information

1
Department of Internal Medicine, Baylor College of Medicine, Houston, Texas. emge@bcm.edu.

Abstract

Mycosis fungoides (MF) is a T-cell, non-Hodgkin lymphoma that primarily involves the skin. Extracutaneous involvement, such as in the parotidgland, is characteristic of end-stage disease. Eosinophilic cellulitis, or Wells syndrome, is a rare inflammatory dermatitis that involves a dermal infiltrate of eosinophils. We report a case of an 80-year-old man with a long-standing diagnosis of stage IIB MF who acutely developed parotid gland involvement and marked hypereosinophilia that most likely represented eosinophilic cellulitis. Activated T cells from his MF were likely a trigger factor for the development of his eosinophilic cellulitis. To our knowledge, this is the first reported case of an MF patient with atypical parotid gland involvement andeosinophilic cellulitis.

PMID:
29469767
[Indexed for MEDLINE]
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15.
Dermatol Online J. 2017 Aug 15;23(8). pii: 13030/qt1xn9q4n9.

Oral involvement of cutaneous T-cell lymphoma.

Author information

1
Tulane University School of Medicine, New Orleans, Louisiana. jaccetta@tulane.edu.

Abstract

Methylisothiazolinone (MI) is commonly used as a preservative in personal care products and is a frequent cause of allergic contact dermatitis. We present a patient with allergic contact dermatitis caused by MI in hair care products and discuss this allergen to bring attention to this common cause of contact dermatitis, and to highlight its frequent use in hair care products. If allergy to MI is suspected, testing should be performed to this individual preservative, as testing solely for the combination preservative, methylisothiazolinone/methylchloroisothiazolinone (Kathon CG®), may miss many cases of MI allergy.

PMID:
29469745
[Indexed for MEDLINE]
16.
Dermatol Online J. 2017 Sep 15;23(9). pii: 13030/qt9mv8r0j7.

Psoriasiform mycosis fungoides: a rare form of the disease with review of the literature.

Author information

1
Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Abstract

BACKGROUND:

Mycosis fungoides (MF) typically presents as erythematous scaly patches or plaques that may progress to cutaneous tumors. Although MF may be presented like other dermatoses, initial presentation as psoriasiform plaques simulating psoriasis is rare. Differentiating MF from psoriasis is important because systemic therapies used for psoriasis can worsen MF. We describe a case of psoriasiform MF and we also review the clinicopathological features of similar cases in the literature.

CASE:

A 46-year-old woman was referred to our clinic with a history of psoriasiform plaques for 13 years. She had multiple, generalized, indurated plaques with thick psoriasiform scales that were unresponsive to topical treatments. The histopathology showed marked psoriasiform epidermal hyperplasia with epidermotropic atypical lymphocytes compatible with MF. Immunohistochemical (IHC) staining showed that atypical lymphocytes were positive for CD3, CD4, CD8, and CD5. Of note, upper dermal and intraepidermal large atypical lymphocytes were CD30 positive. The review of similar psoriasiform MF cases revealed that they had all been treated as psoriasis for many years and finally diagnosed as MF especially after deterioration induced by immunosuppressive therapies.

CONCLUSIONS:

In presumed cases of psoriasis that are unresponsive to treatment, progressive, or ulcerative, biopsy should be considered to rule out MF, particularly before starting a potent immunosuppressive agent.

PMID:
29469726
[Indexed for MEDLINE]
17.
Drug Des Devel Ther. 2018 Jan 31;12:241-254. doi: 10.2147/DDDT.S137106. eCollection 2018.

Clinical potential of mechlorethamine gel for the topical treatment of mycosis fungoides-type cutaneous T-cell lymphoma: a review on current efficacy and safety data.

Author information

1
Division of Dermatology, Medical College of Georgia at Augusta Health, Augusta, GA, USA.
2
School of Medicine, University of North Carolina at Chapel Hill, Carolinas Medical Center, Charlotte, NC, USA.
3
Department of Hematology/Oncology, Levine Cancer Institute, Carolinas Medical Center, Charlotte, NC, USA.

Abstract

Nitrogen mustard is a chemotherapeutic agent that has a well-documented safety and efficacy profile in the treatment of cutaneous T-cell lymphoma. Development of nitrogen mustard formulations and treatment regimens has been studied extensively over the last 40 years. In the last 5 years, a new gel formulation has been developed that is associated with a decrease in delayed hypersensitivity reactions. The authors in this review found that while the gel formulation may result in a decrease of allergic contact dermatitis, this advantage has been replaced by a higher number of irritant contact reactions and a decrease in complete response rate. The gel formulation has a complete response rate of 13.8%, which is a decrease in efficacy when compared to aqueous-based preparations of similar concentrations.

KEYWORDS:

CTCL; Valchlor®; cutaneous T-cell lymphoma; mechlorethamine gel; mycosis fungoides; nitrogen mustard

PMID:
29440874
PMCID:
PMC5798535
DOI:
10.2147/DDDT.S137106
[Indexed for MEDLINE]
Free PMC Article
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18.
BMJ Case Rep. 2018 Feb 5;2018. pii: bcr-2017-222710. doi: 10.1136/bcr-2017-222710.

Successful treatment of extensive splanchnic vein thrombosis in a patient with mycosis fungoides.

Author information

1
Topiwala National Medical College, Mumbai, Maharashtra, India.
2
Surgical Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India.
3
Department of Medical Gastroenterology, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India.
4
Department of Interventional Radiology, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India.

Abstract

A 33-year-old man of a Middle Eastern origin presented to us with abdominal pain and distension secondary to refractory ascites of 1-month duration. The patient had a history of taking oral retinoic acid 25 mg for 4 months for mycosis fungoides. Investigations revealed thrombosis of hepatic veins with extensive thrombosis of the porto-mesenteric axis. A combination of transjugular intrahepatic portosystemic shunt, balloon angioplasty and thrombolysis with recombinant tissue plasminogen activator was successfully used to treat his condition.

KEYWORDS:

cancer intervention; haematology (incl blood transfusion); interventional radiology; skin cancer

PMID:
29437735
DOI:
10.1136/bcr-2017-222710
[Indexed for MEDLINE]
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19.
Strahlenther Onkol. 2018 May;194(5):444-453. doi: 10.1007/s00066-018-1263-9. Epub 2018 Jan 19.

Dose optimization of total or partial skin electron irradiation by thermoluminescent dosimetry.

Author information

1
Department of Radiation Oncology, University Hospital, LMU Munich, Marchioninistraße 15, 81377, Munich, Germany.
2
Department of Radiation Oncology, University Hospital, LMU Munich, Marchioninistraße 15, 81377, Munich, Germany. stefanie.corradini@med.uni-muenchen.de.

Abstract

BACKGROUND:

Due to the complex surface of the human body, total or partial skin irradiation using large electron fields is challenging. The aim of the present study was to quantify the magnitude of dose optimization required after the application of standard fields.

METHODS:

Total skin electron irradiation (TSEI) was applied using the Stanford technique with six dual-fields. Patients presenting with localized lesions were treated with partial skin electron irradiation (PSEI) using large electron fields, which were individually adapted. In order to verify and validate the dose distribution, in vivo dosimetry with thermoluminescent dosimeters (TLD) was performed during the first treatment fraction to detect potential dose heterogeneity and to allow for an individual dose optimization with adjustment of the monitor units (MU).

RESULTS:

Between 1984 and 2017, a total of 58 patients were treated: 31 patients received TSEI using 12 treatment fields, while 27 patients underwent PSEI and were treated with 4-8 treatment fields. After evaluation of the dosimetric results, an individual dose optimization was necessary in 21 patients. Of these, 7 patients received TSEI (7/31). Monitor units (MU) needed to be corrected by a mean value of 117 MU (±105, range 18-290) uniformly for all 12 treatment fields, corresponding to a mean relative change of 12% of the prescribed MU. In comparison, the other 14 patients received PSEI (14/27) and the mean adjustment of monitor units was 282 MU (±144, range 59-500) to single or multiple fields, corresponding to a mean relative change of 22% of the prescribed MU. A second dose optimization to obtain a satisfying dose at the prescription point was need in 5 patients.

CONCLUSIONS:

Thermoluminescent dosimetry allows an individual dose optimization in TSEI and PSEI to enable a reliable adjustment of the MUs to obtain the prescription dose. Especially in PSEI in vivo dosimetry is of fundamental importance.

KEYWORDS:

Cutaneous T‑cell lymphoma; Mycosis fungoides; Partial skin electron irradiation; Skin metastases; Thermoluminescent dosimetry; Total skin electron irradiation

PMID:
29350236
DOI:
10.1007/s00066-018-1263-9
[Indexed for MEDLINE]
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20.
Intern Med. 2018 May 15;57(10):1445-1453. doi: 10.2169/internalmedicine.9668-17. Epub 2018 Jan 11.

Synchronous Occurrence of Mycosis Fungoides, Diffuse Large B Cell Lymphoma and Acute Myeloid Leukemia.

Author information

1
Department of Hematology, Sakai Hospital Kindai University Faculty of Medicine, Japan.
2
Department of Hematology and Rheumatology, Kindai University Faculty of Medicine, Japan.

Abstract

Patients with mycosis fungoides (MF), the most common subtype of primary cutaneous T-cell lymphoma, have an increased risk of developing secondary malignancies. We herein report two rare cases of MF concurring with diffuse large B cell lymphoma (B lymphoid lineage) and acute myeloid leukemia (myeloid lineage) in two otherwise healthy elderly patients. Potential etiologic factors, including the impact of the therapy-associated inflammatory response on the development of secondary tumors in patients with MF, are discussed. Further clinical, experimental and genetic studies are needed to elucidate possible physiopathogenic associations among the three concurrent malignancies occurring in the cases presented here.

KEYWORDS:

TET2; acute myelomonocytic leukemia; diffuse large B-cell lymphoma; mycosis fungoides

PMID:
29321428
PMCID:
PMC5995719
DOI:
10.2169/internalmedicine.9668-17
[Indexed for MEDLINE]
Free PMC Article
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