Background The World Health Organization and European Organization for Research and Treatment of Cancer （WHOEORTC） classification in 2005 promoted the comparisons of primary cutaneous lymphoma （PCL） subtypes betwe...Background The World Health Organization and European Organization for Research and Treatment of Cancer （WHOEORTC） classification in 2005 promoted the comparisons of primary cutaneous lymphoma （PCL） subtypes between different countries.The relative frequency of PCL varied according to geography.The study aimed to analyze the relative frequency and survival of PCLs in China and to compare the data with the published results from other countries.Methods We analyzed 98 patients with PCLs over a 6-year period and reclassified them according to the most recent WHO-EORTC classification （2005）.Disease-specific survival rate and curves according to specific subtypes such as mycosis fungoides,lymphomatoid papulosis,and primary cutaneous peripheral T-cell lymphoma,unspecified was also calculated.Results The relative rate of PCL in China was distinct from those in Western countries.Our study showed a higher frequency of cutaneous T-and NK-cell lymphomas （CTCLs） （94%）,and a lower frequency of cutaneous B-cell lymphomas （CBCLs） （6%）.The 5-year survival rate of the total PCLs was 82%.There was no significant difference in the 5-year survival rate （P 〉0.05 by Log-rank test） between CTCL （80%） and CBCL （100%）.Conclusions The higher percentage of CTCL in China may provide a clue to further study the etiological factors of PCLs.Racial variations in factors such as HLA determinants may play a role in the development of CTCL.展开更多
BACKGROUND Mycosis fungoides is the most common primary cutaneous T-cell lymphoma,whereas generalized erythroderma is rare.In this report,we describe a case of mycosis fungoides with generalized erythroderma using com...BACKGROUND Mycosis fungoides is the most common primary cutaneous T-cell lymphoma,whereas generalized erythroderma is rare.In this report,we describe a case of mycosis fungoides with generalized erythroderma using complete clinical data and[18F]fluoroDglucose positron emission tomography/computed tomography(18F-FDG PET/CT)images.CASE SUMMARY Systemic skin redness with desquamation for three years confirmed mycosis fungoides within one month.The patient underwent left axillary lymphadenectomy biopsy;pathological biopsy suggested abnormal T-cell lesions consistent with mycosis fungoides involving lymph nodes.The patient received methotrexate,5 mg twice weekly,as part of their chemotherapy regimen.Patients January half after discharge,no obvious cause of high fever,left axillary lymph nodes with red heat pain,and rupture entered our hospital for treatment.CONCLUSION The 18F-FDG PET/CT is essential for early diagnosis and timely treatment.展开更多
The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed ...The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed at the goal of achieving and sustaining remission. Increasingly, low dose total skin electron beam therapy (TSEBT) is being utilized as a skin directed component in combination therapy for advanced stage CTCL. Researchers are seeking to better define the utility of low dose TSEBT as a method of debulking skin disease while simultaneously treating other disease compartments and in combination with sustained maintenance therapies of both the skin directed and systemic varieties. Data exists showing the efficacy of low dose TSEBT in early and advanced disease. There is also data documenting prolonged treatment responses with TSEBT plus adjuvant skin directed therapies such as PUVA and topical nitrogen mustard. Emerging data examining the role of low dose TSEBT in the prestem cell transplant preparation is also promising. This brief review summarizes the utility of low dose TSEBT in multiagent treatment regimens in CTCL.展开更多
Papular mycosis fungoides(MF) is a rare presentation of MF. Six illustrative cases of papular MF were retrospectively reviewed. Five of the cases studied by immunohistochemistry had variable numbers(range: 1%-20%) of ...Papular mycosis fungoides(MF) is a rare presentation of MF. Six illustrative cases of papular MF were retrospectively reviewed. Five of the cases studied by immunohistochemistry had variable numbers(range: 1%-20%) of CD30+ cells in the dermal infiltrate, a finding that is characteristic of lymphomatoid papulosis but may occasionally occur in typical early MF. Although none of our papular MF patients had progressive disease, lesions with relatively high numbers of CD30+ cells in 3 patients did not respond well to skin-directed treatments used for MF. Interestingly, these patients had evidence of coexisting clonal B cell populations in the blood(one with clonal B cell lymphocytosis and two with B-cell chronic lymphocytic leukemia). We conclude that:(1) papular MF may contain CD30+ cells, thereby causing confusion with lymphomatoid papulosis; and(2) papular MF, like more typical MF, may be associated with clonal B-cell proliferations including chronic lymphocytic leukemia.展开更多
Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage ...Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital.The median age of these patients was 53.36±14.45 years.Female-male ratio was 1:1.2.The average course of disease was 10.82±3.37 years.All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55±9.51(40-74) Gy in an average of 24.55±5.57(20-40) fractions,5 fractions per week.Results The median follow-up time was 55.27±29.3(13-103) months.No severe acute or chronic side effects of irradiation were observed.Complete clinical response(CR) rate of the radiated sites was 54.5%(6/11),partial response(PR) rate was 36.4%(4/11),and the overall response rate(CR+PR) was 90.9%.One patient showed no response.Conclusion Local radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.展开更多
Hypopigmented mycosis fungoides (HMF) is an uncommon variant of cutaneous T-cell lymphoma. It is more frequent in dark-skinned people, particularly children. The HMF diagnose is difficult, especially in early stages b...Hypopigmented mycosis fungoides (HMF) is an uncommon variant of cutaneous T-cell lymphoma. It is more frequent in dark-skinned people, particularly children. The HMF diagnose is difficult, especially in early stages because this condition resembles benign skin diseases. Thus is histopathological analysis very important for the diagnosis. We report a case of a 7-year-old child with widespread HMF confirmed by histopathology that showed cells tagging along the dermal/epidermal junction and extending into the epidermis in a pattern of epidermotropism and focal cell aggregates in the epidermis (Pautrier’s microabscess). We demonstrate the importance of clinical suspicion for this cutaneous neoplasia in patients with hypopigmentated lesions.展开更多
Background: Mycosis fungoides (MF) is not an uncommon T-cell lymphoma in Iraqi population which is increasing over years. There are many diagnostic techniques to confirm the diagnosis including histopathological and i...Background: Mycosis fungoides (MF) is not an uncommon T-cell lymphoma in Iraqi population which is increasing over years. There are many diagnostic techniques to confirm the diagnosis including histopathological and immunohistochemical tests. Objectives: To evaluate all cases of MF including clinical, histopathological and immunohistochemical tests with CD markers. Patients and Methods: This clinical, histopathological, immunohistochemical outpatient based study took place in the Department of Dermatology, Baghdad Teaching Hospital, Medical City, Baghdad, Iraq during the period from May 2012-September 2013. Twenty five patients with MF were included in the present work. History was obtained from each patient regarding all socio-demographic aspects related to the disease. Also, clinical evaluation was carried out for all patients. Incisional biopsies for ordinary histopathology and immunohistochemical tests were done. Then staging was carried out depending on TNMB classification for all patients. Results: All cases of MF were confirmed after clinical evaluation, histopathology and immunohistochemical examination. Male to female ratio was 1.5:1. The mean age of onset at presentation was 47.45 ± 16.9 years. Itching was found in 84% of cases. The patch stage was seen in 36% patients. The hypopigmentaed MF was presented in 12% cases. The lower extremities were seen in 80%. Lymph node involvement as a manifestation of the disease was seen in 44% cases. The histopathological features of MF went parallel with the clinical stage of disease. Immunohistochemical study aids in the diagnosis of patch and erythrodermic stages. The most patients presented with stage IB (36%). Conclusion: MF seems to be not uncommon problem in Iraqi population and is increasing over years. The disease was diagnosed early commonly with patch stage (stage IB) while hypopigmented MF was not a common variant when compared with neighboring countries.展开更多
Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress ...Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress in about 20% of such patients. About 30% of patients have been reported to develop advancedstage disease and, at present, there is no cure for the disease. A number of systemic approaches have been used for advanced-stage mycosis fungoides(IIB-IV) and transformed disease. Aggressive approaches seem to be warranted in such patients. The scope of this review is the stem cell transplantation in mycosis fungoides and its leukemic variant, Sèzary syndrome.展开更多
Introduction: The mycosis fungoides (MF) is the most common cutaneous T-cell lymphomas representing about 50% of all cutaneous lymphomas. Its pathogenesis remains poorly understood. Thus, its rising incidence rates ca...Introduction: The mycosis fungoides (MF) is the most common cutaneous T-cell lymphomas representing about 50% of all cutaneous lymphomas. Its pathogenesis remains poorly understood. Thus, its rising incidence rates call for an explanation. Several environmental and occupational factors have been implicated in the pathogenesis of this disease. Objective: To determine the potential occupational factors correlated in the occurrence of MF. Methods: Case-control study was carried out at the teaching Hospital Farhat Hached of Sousse (Tunisia) to investigate associations of MF and occupational risk factors. A questionnaire on lifetime job history was administered to 24 cases and controls. Cases were patients with MF and whose diagnosis was suspected by a dermatologist and confirmed by pathological examination. Controls were patients consulting in the occupational medicine department and matched to cases by age and gender. Bivariate analysis was used to calculate odds ratios (OR) and 95% confidence intervals (CI). Results: The average age of onset of the disease was 55.41 ± 17.4 years with a sex ratio of 2.42. We found an increased risk of MF associated with tobacco consumption higher than 40 Pack-year (OR = 6.6;95% CI = 1.22 to 35.4), exercise in the textile sector (OR = 9.4;CI = 1.06 to 84.3) and exposure to diesel exhaust (OR = 9.47;95% CI = 1.06 to 84.3). Conclusion: Occupational factors could play a role in the pathogenesis of MF. Regarding our study, smoking, working in the textile industry and exposure to diesel exhaust appear to be involved in the pathogenesis of MF. Further investigation is needed to confirm these findings.展开更多
Mycosis fungoides(MF) is a cutaneous T-cell lymphoma that can undergo local progression with possible systemic dissemination. We report a case of a patient affected by MF with a pancreatic mass that was a diagnostic c...Mycosis fungoides(MF) is a cutaneous T-cell lymphoma that can undergo local progression with possible systemic dissemination. We report a case of a patient affected by MF with a pancreatic mass that was a diagnostic challenge between primitive tumor and pancreatic metastasis from MF. Clinical setting findings and imaging studies raised the suspicion of a pancreatic primary neoplasm. A diagnostic clue was provided by the combined histomorphologic/immunohistochemical study of pancreatic and cutaneous biopsies, which revealed a pancreatic localization of MF. Considering the rarity of metastatic localization of MF to the pancreas, we next investigated whether chemokinechemokine receptor interactions could be involved in the phenomenon to provide new insight into the possible mechanisms underlying metastatic localization of MF to the pancreas. Histological analyses of archival pancreatic tissue demonstrated that glucagon-secreting cells of the pancreatic islets expressed the CCL27 chemokine, which may have attracted in our case metastatic MF cells expressing the complementary receptor CCR10.展开更多
Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been show...Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been shown to result in long-term disease-free intervals, with even curative potential. Radiation is also effective as palliative treatment for the localized lesion resistant to the topic or other treatments. In the current study, we report using high dose rate (HDR) radiation treatment for a patient with resistant mycosis fungoides involving the wrist. We report a convenient treatment with an ideal radiation dose distribution, and a excellent clinical outcome.展开更多
Total skin electron therapy (TSET) is used for the treatment of Mycosis Fungoides. Several tech-niques have been developed, in order to achieve homogeneous dose distribution over the complete body surface. To implemen...Total skin electron therapy (TSET) is used for the treatment of Mycosis Fungoides. Several tech-niques have been developed, in order to achieve homogeneous dose distribution over the complete body surface. To implement a TSET technique, one has to optimize a variety of parameters. Monte Carlo simulation of TSET can facilitate this optimization. The aim of this study was to commission and optimize a TSET technique using the 4 and 6 MeV electron and the high dose rate facility on the Elekta Precise accelerator. The EGS4nrc/BEAMnrc Monte Carlo code was used. The beam data were calculated and measured at two different scoring planes for a single beam. The Model was validated by comparing the simulation with measurements. Two different vertical angles were used to obtain a uniform dose. The angle was optimized for best dose uniformity. The Rando phantom is placed on a rotating platform and rotates 60 degrees apart to facilitate the six patient position orientations. The doses delivered in a phantom by complete treatment were measured with Kodak EDR2 films and TLDs. The dose distribution varied among various scanning directions by 2 - 3 mm and 3 - 4 mm for 4 and 6 MeV respectively. The composite percentage depth dose of all six dual fields for the 4 and 6 MeV yielded an R80 of ~4 mm and ~6 mm, respectively. Dose uniformity was ±6% for 4 MeV and ±5% for 6 MeV. The bremsstrahlung contamination was 0.9% - 1.3%. Good agreements were found with published literature and inline with international protocols.展开更多
Mycosis fungoides, the most common primary cutaneous lymphoma, may present with a broad spectrum of clinical features. As both clinical and dermatopathological findings in mycosis fungoides occasionally closely imitat...Mycosis fungoides, the most common primary cutaneous lymphoma, may present with a broad spectrum of clinical features. As both clinical and dermatopathological findings in mycosis fungoides occasionally closely imitateother dermatoses, correct diagnosis may be a challenge both for clinicians as well as dermatopathologists. As a consequence, diagnosis of cutaneous lymphoma may be initially missed and, therefore, prompt and adequate therapeutic measures delayed. Hence, the purpose of our article was to give an overview of hitherto published "mimickers" of mycosis fungoides with a review of its diverse clinical features to alert the clinicians about the wide spectrum of this dissimulating disease. By integrating our own encountered atypical cases of mycosis fungoides we provide a comprehensive illustrated histological and moleculargenetic workup thereof and thereby critically revise the different available diagnostic tools of daily routine. Finally, we derive a practical algorithm to obtain the correct diagnosis even in such ambiguous cases of mycosis fungoides.展开更多
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma(CTCL), and is characterized by a clonal expansion of malignant CD4+ T lymphocytes with skinhoming properties. Clinically and pathologically, mycos...Mycosis fungoides is the most common form of cutaneous T-cell lymphoma(CTCL), and is characterized by a clonal expansion of malignant CD4+ T lymphocytes with skinhoming properties. Clinically and pathologically, mycosis fungoides can be categorized into patch, plaque and tumor stages. The clinical course of mycosis fungoides is usually chronic and indolent, but a proportion of patients may develop progressive disease with peripheral blood, lymph node and visceral organ involvement. Sézarysyndrome is an aggressive leukemic form of CTCL characterized by a clonal population of malignant T cells in the peripheral blood. Various forms of skin-directed and systemic treatments are available for mycosis fungoides and Sézary syndrome. However, current treatments are generally not curative, and can only control the disease. Currently, the etiology and pathogenesis of mycosis fungoides and Sézary syndrome are not well defined. Proposed mechanisms include chronic antigenic stimulation by infectious agents, expression of specific adhesion molecules, altered cytokine production, mutations of oncogenes and tumor suppressor genes, and avoidance of apoptosis. In recent years, a number of chemokine receptors and their corresponding chemokine ligands have been found to contribute to the migration and survival of lymphoma cells in mycosis fungoides and Sézary syndrome, including CC chemokine receptor 4(CCR4), CCR10, C-X-C chemokine receptor type 4(CXCR4), CCR7, CCR3 and CXCR3. Since chemokines and chemokine receptors have been found to play important roles in the pathophysiology of mycosis fungoides and Sézary syndrome, they may be potentially useful targets for the development of new treatments for these diseases in the future.展开更多
Background: Mycosis Fungoides (MF) is the most common and indolent form of Cutaneuse T-cell Lymphomas (CTCL), that usually occurs in old adults. Objectives: To determine epidemiologic features and patients characteris...Background: Mycosis Fungoides (MF) is the most common and indolent form of Cutaneuse T-cell Lymphomas (CTCL), that usually occurs in old adults. Objectives: To determine epidemiologic features and patients characteristics of MF in Isfahan (Iran). Methods: We performed a retrospective study in MF clinic of alzahra hospital that is the main center for treatment of MF patients in Isfahan (Iran) and evaluated clinicopathologic features. Results: In 3 years 25 patients were referred to Alzahra MF clinic. 18 patients diagnosed as MF. Seven (38.9%) patients were male and 11 (61.1%) were female with male to female ratio of 1:1.57. The mean age of patients was 41.06 years. 88.9% of our patients were in stages ΙA and ΙB. Conclusion: Most of our patients presented in early stages that were similar to other studies, while male: female ratio is different from other studies.展开更多
Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and viscera...Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and visceral organs in later stage and ultimately progression to systemic lymphoma.It can result in secondary skin infection which is a frequent cause of death. At present,no curative therapy existed.Therapeutic purpose is to induce remission,reduce tumor burden and protect immune function of patients.A case of patient with advanced severe mycosis fungoides receiving CHOP plus interferon a -2a was reported here,with disease-free survival of 7 months and overall survival of over 17.0 months,and current status as well as developments of mycosis fungoides were briefly introduced.展开更多
Objectives. To detect telomerase activity in patients with mycosis fungoides (MF) and to study therole of telomerase in the tumorigenesis of MF.Methods. The technique of PCR-ELISA was employed to detect telomerase act...Objectives. To detect telomerase activity in patients with mycosis fungoides (MF) and to study therole of telomerase in the tumorigenesis of MF.Methods. The technique of PCR-ELISA was employed to detect telomerase activity in 35 patientswith various stages of MF.Results. 92.3% tumor stage of MF, 78.6% plaque stage of MF and 75.0% patch stage of MF hadpositive telomerase activity. The control samples had no telomerase activity. Telomerase activity in tumorstage of MF was significantly higher than that in plaque stage, while the latter was higher than that inpatch stage. Telomerase activity was correlated with the stage of MF.Conclusion. High level of telomerase activity frequently occurred in patients with MF, suggestingthat telomerase might play an important role in the tumorigenesis of MF and is a useful marker for thediagnosis of MF possibly.展开更多
Objective： The purpose of this study was to present the dosimetric study and evaluation the dose delivered to the skin tumor by using diode detector with total skin electron therapy （TSET）. Methods： The total skin...Objective： The purpose of this study was to present the dosimetric study and evaluation the dose delivered to the skin tumor by using diode detector with total skin electron therapy （TSET）. Methods： The total skin electron irradiation （TSEI） technique was used to treat ten patients with histological confirmed mycosis fungoides according to the Stanford staging system at the Radiotherapy Department, National Cancer Institute, Cairo University, Egypt. High dose rate electron beams with low electron energy 5 MeV from a Siemens linear accelerator were used for treatment. Diodes were calibrated at TSET distance 300 cm and field size （35 × 35） cm^2. Results： The result of diodes measurements showed the dose to flat surface of the body was within ：1：10 % from the prescribed dose. Special areas of the body such as the perineum ＆ eyelid showed large deviation up to 30% variation from the prescription dose. Conclusion： The diode results of this study will be used as a quality assurance check for all new patients treated with TSET and to compare it to the prescribed dose delivered to the patients. It is recommends to evaluate the diodes measurements for all patients throughout the full treatment cycle and to identify individually the boost dose areas.展开更多
BACKGROUND Mycosis fungoides(MF)is a form of lymphoma derived from heterogeneous T cells,and eyelid involvement is extremely rare.The common methods to treat eyelid involvement are radiotherapy and chemotherapy,but th...BACKGROUND Mycosis fungoides(MF)is a form of lymphoma derived from heterogeneous T cells,and eyelid involvement is extremely rare.The common methods to treat eyelid involvement are radiotherapy and chemotherapy,but their efficacies are limited.Herein,we report a case of advanced-stage MF eyelid involvement,propose ultrasound(US)-guided microwave ablation(MWA)therapy and present a literature review.CASE SUMMARY A male patient was admitted to our hospital in June 2018 and diagnosed with MF via radiological and histopathological examinations.The patient’s condition was not well controlled by various conventional chemotherapies.US-guided MWA was performed to relieve the patient’s symptoms and improve his quality of life,showing satisfactory efficacy.CONCLUSION Eyelid involvement is one of the most troublesome clinical problems for advanced-stage MF patients.This is the first report on the use of US-guided MWA as a palliative therapy for MF eyelid involvement;the treatment successfully relieved the patient’s clinical symptoms and reduced his anxiety behaviours.Our study sheds new light on methods for improving the clinical management of eyelid involvement in MF.展开更多
To better understand the pathogenesis of Sézary cells, distinguish them from reactive skin-infltrating T-cells and improve disease treatment, efforts have been made to identify molecular targets deregulated by th...To better understand the pathogenesis of Sézary cells, distinguish them from reactive skin-infltrating T-cells and improve disease treatment, efforts have been made to identify molecular targets deregulated by the malignant process. From immunophenotypic analysis and subtractive differential expression experiments to pan-genomic studies, many approaches have been used to identify markers of the disease. During the last decade several natural killer （NK） cell markers have been found aberrantly expressed at the surface of Sézary cells. In particular, KIR3DL2/CD158k, expressed by less than 2% of healthy individuals CD4＋ T-cells, is an excellent marker to identify and follow the tumor burden in the blood of Sézary syndrome patients. It may also represent a valuable target for specifc im-munotherapy. Other products of the NK cluster on chromosome 19q13 have been detected on Sézary cells, raising the hypothesis of an NK reprogramming process associated with the malignant transformation that may induce survival functions.展开更多
文摘Background The World Health Organization and European Organization for Research and Treatment of Cancer （WHOEORTC） classification in 2005 promoted the comparisons of primary cutaneous lymphoma （PCL） subtypes between different countries.The relative frequency of PCL varied according to geography.The study aimed to analyze the relative frequency and survival of PCLs in China and to compare the data with the published results from other countries.Methods We analyzed 98 patients with PCLs over a 6-year period and reclassified them according to the most recent WHO-EORTC classification （2005）.Disease-specific survival rate and curves according to specific subtypes such as mycosis fungoides,lymphomatoid papulosis,and primary cutaneous peripheral T-cell lymphoma,unspecified was also calculated.Results The relative rate of PCL in China was distinct from those in Western countries.Our study showed a higher frequency of cutaneous T-and NK-cell lymphomas （CTCLs） （94%）,and a lower frequency of cutaneous B-cell lymphomas （CBCLs） （6%）.The 5-year survival rate of the total PCLs was 82%.There was no significant difference in the 5-year survival rate （P 〉0.05 by Log-rank test） between CTCL （80%） and CBCL （100%）.Conclusions The higher percentage of CTCL in China may provide a clue to further study the etiological factors of PCLs.Racial variations in factors such as HLA determinants may play a role in the development of CTCL.
文摘BACKGROUND Mycosis fungoides is the most common primary cutaneous T-cell lymphoma,whereas generalized erythroderma is rare.In this report,we describe a case of mycosis fungoides with generalized erythroderma using complete clinical data and[18F]fluoroDglucose positron emission tomography/computed tomography(18F-FDG PET/CT)images.CASE SUMMARY Systemic skin redness with desquamation for three years confirmed mycosis fungoides within one month.The patient underwent left axillary lymphadenectomy biopsy;pathological biopsy suggested abnormal T-cell lesions consistent with mycosis fungoides involving lymph nodes.The patient received methotrexate,5 mg twice weekly,as part of their chemotherapy regimen.Patients January half after discharge,no obvious cause of high fever,left axillary lymph nodes with red heat pain,and rupture entered our hospital for treatment.CONCLUSION The 18F-FDG PET/CT is essential for early diagnosis and timely treatment.
文摘The treatment of advanced stage MF is especially challenging as single agent overall response rates are in the 35% range and chronic recurrence is the rule. The treatment of CTCL across all stages of disease is aimed at the goal of achieving and sustaining remission. Increasingly, low dose total skin electron beam therapy (TSEBT) is being utilized as a skin directed component in combination therapy for advanced stage CTCL. Researchers are seeking to better define the utility of low dose TSEBT as a method of debulking skin disease while simultaneously treating other disease compartments and in combination with sustained maintenance therapies of both the skin directed and systemic varieties. Data exists showing the efficacy of low dose TSEBT in early and advanced disease. There is also data documenting prolonged treatment responses with TSEBT plus adjuvant skin directed therapies such as PUVA and topical nitrogen mustard. Emerging data examining the role of low dose TSEBT in the prestem cell transplant preparation is also promising. This brief review summarizes the utility of low dose TSEBT in multiagent treatment regimens in CTCL.
文摘Papular mycosis fungoides(MF) is a rare presentation of MF. Six illustrative cases of papular MF were retrospectively reviewed. Five of the cases studied by immunohistochemistry had variable numbers(range: 1%-20%) of CD30+ cells in the dermal infiltrate, a finding that is characteristic of lymphomatoid papulosis but may occasionally occur in typical early MF. Although none of our papular MF patients had progressive disease, lesions with relatively high numbers of CD30+ cells in 3 patients did not respond well to skin-directed treatments used for MF. Interestingly, these patients had evidence of coexisting clonal B cell populations in the blood(one with clonal B cell lymphocytosis and two with B-cell chronic lymphocytic leukemia). We conclude that:(1) papular MF may contain CD30+ cells, thereby causing confusion with lymphomatoid papulosis; and(2) papular MF, like more typical MF, may be associated with clonal B-cell proliferations including chronic lymphocytic leukemia.
文摘Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides(MF).Methods From January 2008 to January 2013,a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital.The median age of these patients was 53.36±14.45 years.Female-male ratio was 1:1.2.The average course of disease was 10.82±3.37 years.All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55±9.51(40-74) Gy in an average of 24.55±5.57(20-40) fractions,5 fractions per week.Results The median follow-up time was 55.27±29.3(13-103) months.No severe acute or chronic side effects of irradiation were observed.Complete clinical response(CR) rate of the radiated sites was 54.5%(6/11),partial response(PR) rate was 36.4%(4/11),and the overall response rate(CR+PR) was 90.9%.One patient showed no response.Conclusion Local radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.
文摘Hypopigmented mycosis fungoides (HMF) is an uncommon variant of cutaneous T-cell lymphoma. It is more frequent in dark-skinned people, particularly children. The HMF diagnose is difficult, especially in early stages because this condition resembles benign skin diseases. Thus is histopathological analysis very important for the diagnosis. We report a case of a 7-year-old child with widespread HMF confirmed by histopathology that showed cells tagging along the dermal/epidermal junction and extending into the epidermis in a pattern of epidermotropism and focal cell aggregates in the epidermis (Pautrier’s microabscess). We demonstrate the importance of clinical suspicion for this cutaneous neoplasia in patients with hypopigmentated lesions.
文摘Background: Mycosis fungoides (MF) is not an uncommon T-cell lymphoma in Iraqi population which is increasing over years. There are many diagnostic techniques to confirm the diagnosis including histopathological and immunohistochemical tests. Objectives: To evaluate all cases of MF including clinical, histopathological and immunohistochemical tests with CD markers. Patients and Methods: This clinical, histopathological, immunohistochemical outpatient based study took place in the Department of Dermatology, Baghdad Teaching Hospital, Medical City, Baghdad, Iraq during the period from May 2012-September 2013. Twenty five patients with MF were included in the present work. History was obtained from each patient regarding all socio-demographic aspects related to the disease. Also, clinical evaluation was carried out for all patients. Incisional biopsies for ordinary histopathology and immunohistochemical tests were done. Then staging was carried out depending on TNMB classification for all patients. Results: All cases of MF were confirmed after clinical evaluation, histopathology and immunohistochemical examination. Male to female ratio was 1.5:1. The mean age of onset at presentation was 47.45 ± 16.9 years. Itching was found in 84% of cases. The patch stage was seen in 36% patients. The hypopigmentaed MF was presented in 12% cases. The lower extremities were seen in 80%. Lymph node involvement as a manifestation of the disease was seen in 44% cases. The histopathological features of MF went parallel with the clinical stage of disease. Immunohistochemical study aids in the diagnosis of patch and erythrodermic stages. The most patients presented with stage IB (36%). Conclusion: MF seems to be not uncommon problem in Iraqi population and is increasing over years. The disease was diagnosed early commonly with patch stage (stage IB) while hypopigmented MF was not a common variant when compared with neighboring countries.
文摘Mycosis fungoides and Sèzary syndrome are the most common subtypes of cutaneous T-cell lymphomas. Even though, in early-stage disease, Mycosis fungoides commonly has a more indolent course, disease will progress in about 20% of such patients. About 30% of patients have been reported to develop advancedstage disease and, at present, there is no cure for the disease. A number of systemic approaches have been used for advanced-stage mycosis fungoides(IIB-IV) and transformed disease. Aggressive approaches seem to be warranted in such patients. The scope of this review is the stem cell transplantation in mycosis fungoides and its leukemic variant, Sèzary syndrome.
文摘Introduction: The mycosis fungoides (MF) is the most common cutaneous T-cell lymphomas representing about 50% of all cutaneous lymphomas. Its pathogenesis remains poorly understood. Thus, its rising incidence rates call for an explanation. Several environmental and occupational factors have been implicated in the pathogenesis of this disease. Objective: To determine the potential occupational factors correlated in the occurrence of MF. Methods: Case-control study was carried out at the teaching Hospital Farhat Hached of Sousse (Tunisia) to investigate associations of MF and occupational risk factors. A questionnaire on lifetime job history was administered to 24 cases and controls. Cases were patients with MF and whose diagnosis was suspected by a dermatologist and confirmed by pathological examination. Controls were patients consulting in the occupational medicine department and matched to cases by age and gender. Bivariate analysis was used to calculate odds ratios (OR) and 95% confidence intervals (CI). Results: The average age of onset of the disease was 55.41 ± 17.4 years with a sex ratio of 2.42. We found an increased risk of MF associated with tobacco consumption higher than 40 Pack-year (OR = 6.6;95% CI = 1.22 to 35.4), exercise in the textile sector (OR = 9.4;CI = 1.06 to 84.3) and exposure to diesel exhaust (OR = 9.47;95% CI = 1.06 to 84.3). Conclusion: Occupational factors could play a role in the pathogenesis of MF. Regarding our study, smoking, working in the textile industry and exposure to diesel exhaust appear to be involved in the pathogenesis of MF. Further investigation is needed to confirm these findings.
基金Supported by Cinque per mille e Ricerca Corrente,Ministero della Salute to Istituto Giannina Gaslini
文摘Mycosis fungoides(MF) is a cutaneous T-cell lymphoma that can undergo local progression with possible systemic dissemination. We report a case of a patient affected by MF with a pancreatic mass that was a diagnostic challenge between primitive tumor and pancreatic metastasis from MF. Clinical setting findings and imaging studies raised the suspicion of a pancreatic primary neoplasm. A diagnostic clue was provided by the combined histomorphologic/immunohistochemical study of pancreatic and cutaneous biopsies, which revealed a pancreatic localization of MF. Considering the rarity of metastatic localization of MF to the pancreas, we next investigated whether chemokinechemokine receptor interactions could be involved in the phenomenon to provide new insight into the possible mechanisms underlying metastatic localization of MF to the pancreas. Histological analyses of archival pancreatic tissue demonstrated that glucagon-secreting cells of the pancreatic islets expressed the CCL27 chemokine, which may have attracted in our case metastatic MF cells expressing the complementary receptor CCR10.
文摘Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma accounting for approximately half of all cutaneous T-cell lymphomas. Radiation therapy is an effective treatment for early stage MF and has been shown to result in long-term disease-free intervals, with even curative potential. Radiation is also effective as palliative treatment for the localized lesion resistant to the topic or other treatments. In the current study, we report using high dose rate (HDR) radiation treatment for a patient with resistant mycosis fungoides involving the wrist. We report a convenient treatment with an ideal radiation dose distribution, and a excellent clinical outcome.
文摘Total skin electron therapy (TSET) is used for the treatment of Mycosis Fungoides. Several tech-niques have been developed, in order to achieve homogeneous dose distribution over the complete body surface. To implement a TSET technique, one has to optimize a variety of parameters. Monte Carlo simulation of TSET can facilitate this optimization. The aim of this study was to commission and optimize a TSET technique using the 4 and 6 MeV electron and the high dose rate facility on the Elekta Precise accelerator. The EGS4nrc/BEAMnrc Monte Carlo code was used. The beam data were calculated and measured at two different scoring planes for a single beam. The Model was validated by comparing the simulation with measurements. Two different vertical angles were used to obtain a uniform dose. The angle was optimized for best dose uniformity. The Rando phantom is placed on a rotating platform and rotates 60 degrees apart to facilitate the six patient position orientations. The doses delivered in a phantom by complete treatment were measured with Kodak EDR2 films and TLDs. The dose distribution varied among various scanning directions by 2 - 3 mm and 3 - 4 mm for 4 and 6 MeV respectively. The composite percentage depth dose of all six dual fields for the 4 and 6 MeV yielded an R80 of ~4 mm and ~6 mm, respectively. Dose uniformity was ±6% for 4 MeV and ±5% for 6 MeV. The bremsstrahlung contamination was 0.9% - 1.3%. Good agreements were found with published literature and inline with international protocols.
文摘Mycosis fungoides, the most common primary cutaneous lymphoma, may present with a broad spectrum of clinical features. As both clinical and dermatopathological findings in mycosis fungoides occasionally closely imitateother dermatoses, correct diagnosis may be a challenge both for clinicians as well as dermatopathologists. As a consequence, diagnosis of cutaneous lymphoma may be initially missed and, therefore, prompt and adequate therapeutic measures delayed. Hence, the purpose of our article was to give an overview of hitherto published "mimickers" of mycosis fungoides with a review of its diverse clinical features to alert the clinicians about the wide spectrum of this dissimulating disease. By integrating our own encountered atypical cases of mycosis fungoides we provide a comprehensive illustrated histological and moleculargenetic workup thereof and thereby critically revise the different available diagnostic tools of daily routine. Finally, we derive a practical algorithm to obtain the correct diagnosis even in such ambiguous cases of mycosis fungoides.
文摘Mycosis fungoides is the most common form of cutaneous T-cell lymphoma(CTCL), and is characterized by a clonal expansion of malignant CD4+ T lymphocytes with skinhoming properties. Clinically and pathologically, mycosis fungoides can be categorized into patch, plaque and tumor stages. The clinical course of mycosis fungoides is usually chronic and indolent, but a proportion of patients may develop progressive disease with peripheral blood, lymph node and visceral organ involvement. Sézarysyndrome is an aggressive leukemic form of CTCL characterized by a clonal population of malignant T cells in the peripheral blood. Various forms of skin-directed and systemic treatments are available for mycosis fungoides and Sézary syndrome. However, current treatments are generally not curative, and can only control the disease. Currently, the etiology and pathogenesis of mycosis fungoides and Sézary syndrome are not well defined. Proposed mechanisms include chronic antigenic stimulation by infectious agents, expression of specific adhesion molecules, altered cytokine production, mutations of oncogenes and tumor suppressor genes, and avoidance of apoptosis. In recent years, a number of chemokine receptors and their corresponding chemokine ligands have been found to contribute to the migration and survival of lymphoma cells in mycosis fungoides and Sézary syndrome, including CC chemokine receptor 4(CCR4), CCR10, C-X-C chemokine receptor type 4(CXCR4), CCR7, CCR3 and CXCR3. Since chemokines and chemokine receptors have been found to play important roles in the pathophysiology of mycosis fungoides and Sézary syndrome, they may be potentially useful targets for the development of new treatments for these diseases in the future.
文摘Background: Mycosis Fungoides (MF) is the most common and indolent form of Cutaneuse T-cell Lymphomas (CTCL), that usually occurs in old adults. Objectives: To determine epidemiologic features and patients characteristics of MF in Isfahan (Iran). Methods: We performed a retrospective study in MF clinic of alzahra hospital that is the main center for treatment of MF patients in Isfahan (Iran) and evaluated clinicopathologic features. Results: In 3 years 25 patients were referred to Alzahra MF clinic. 18 patients diagnosed as MF. Seven (38.9%) patients were male and 11 (61.1%) were female with male to female ratio of 1:1.57. The mean age of patients was 41.06 years. 88.9% of our patients were in stages ΙA and ΙB. Conclusion: Most of our patients presented in early stages that were similar to other studies, while male: female ratio is different from other studies.
基金supported by the Jiangsu province Natural Science Foundation of China(No.BK2010579)
文摘Mycosis fungoides is a common cutaneous T-cell lymphoma,which is usually characterized by chronic,indolence progression,with absence of typical symptoms in early stage,metastasis to lymph nodes,bone marrow and visceral organs in later stage and ultimately progression to systemic lymphoma.It can result in secondary skin infection which is a frequent cause of death. At present,no curative therapy existed.Therapeutic purpose is to induce remission,reduce tumor burden and protect immune function of patients.A case of patient with advanced severe mycosis fungoides receiving CHOP plus interferon a -2a was reported here,with disease-free survival of 7 months and overall survival of over 17.0 months,and current status as well as developments of mycosis fungoides were briefly introduced.
文摘Objectives. To detect telomerase activity in patients with mycosis fungoides (MF) and to study therole of telomerase in the tumorigenesis of MF.Methods. The technique of PCR-ELISA was employed to detect telomerase activity in 35 patientswith various stages of MF.Results. 92.3% tumor stage of MF, 78.6% plaque stage of MF and 75.0% patch stage of MF hadpositive telomerase activity. The control samples had no telomerase activity. Telomerase activity in tumorstage of MF was significantly higher than that in plaque stage, while the latter was higher than that inpatch stage. Telomerase activity was correlated with the stage of MF.Conclusion. High level of telomerase activity frequently occurred in patients with MF, suggestingthat telomerase might play an important role in the tumorigenesis of MF and is a useful marker for thediagnosis of MF possibly.
文摘Objective： The purpose of this study was to present the dosimetric study and evaluation the dose delivered to the skin tumor by using diode detector with total skin electron therapy （TSET）. Methods： The total skin electron irradiation （TSEI） technique was used to treat ten patients with histological confirmed mycosis fungoides according to the Stanford staging system at the Radiotherapy Department, National Cancer Institute, Cairo University, Egypt. High dose rate electron beams with low electron energy 5 MeV from a Siemens linear accelerator were used for treatment. Diodes were calibrated at TSET distance 300 cm and field size （35 × 35） cm^2. Results： The result of diodes measurements showed the dose to flat surface of the body was within ：1：10 % from the prescribed dose. Special areas of the body such as the perineum ＆ eyelid showed large deviation up to 30% variation from the prescription dose. Conclusion： The diode results of this study will be used as a quality assurance check for all new patients treated with TSET and to compare it to the prescribed dose delivered to the patients. It is recommends to evaluate the diodes measurements for all patients throughout the full treatment cycle and to identify individually the boost dose areas.
基金Supported by Zhenjiang Social Development Fund,No.SH2021028Zhenjiang Social Development Fund,No.SH2018035the“169”Project of Zhenjiang City,No.YLJ201931.
文摘BACKGROUND Mycosis fungoides(MF)is a form of lymphoma derived from heterogeneous T cells,and eyelid involvement is extremely rare.The common methods to treat eyelid involvement are radiotherapy and chemotherapy,but their efficacies are limited.Herein,we report a case of advanced-stage MF eyelid involvement,propose ultrasound(US)-guided microwave ablation(MWA)therapy and present a literature review.CASE SUMMARY A male patient was admitted to our hospital in June 2018 and diagnosed with MF via radiological and histopathological examinations.The patient’s condition was not well controlled by various conventional chemotherapies.US-guided MWA was performed to relieve the patient’s symptoms and improve his quality of life,showing satisfactory efficacy.CONCLUSION Eyelid involvement is one of the most troublesome clinical problems for advanced-stage MF patients.This is the first report on the use of US-guided MWA as a palliative therapy for MF eyelid involvement;the treatment successfully relieved the patient’s clinical symptoms and reduced his anxiety behaviours.Our study sheds new light on methods for improving the clinical management of eyelid involvement in MF.
基金the Inserm, Société de Recherches Dermatologiques (SRD C.S), and Société Franaise de Dermatologie (SFD A.M-C) for their support as well as the European Union through the Euro-Trans-Bio grant (M.B and A.B)
文摘To better understand the pathogenesis of Sézary cells, distinguish them from reactive skin-infltrating T-cells and improve disease treatment, efforts have been made to identify molecular targets deregulated by the malignant process. From immunophenotypic analysis and subtractive differential expression experiments to pan-genomic studies, many approaches have been used to identify markers of the disease. During the last decade several natural killer （NK） cell markers have been found aberrantly expressed at the surface of Sézary cells. In particular, KIR3DL2/CD158k, expressed by less than 2% of healthy individuals CD4＋ T-cells, is an excellent marker to identify and follow the tumor burden in the blood of Sézary syndrome patients. It may also represent a valuable target for specifc im-munotherapy. Other products of the NK cluster on chromosome 19q13 have been detected on Sézary cells, raising the hypothesis of an NK reprogramming process associated with the malignant transformation that may induce survival functions.
