OBJECTIVETo evaluate the efficacy and safety of topical PUVA treatment of refractory lesions of mycosis fungoides.

METHODSFrom January 2008 to 2014, a total of 10 patients (4 males and 6 females) with mycosis fungoides were treated with topical PUVA in Peking Union Medical College Hospital, including 7 cases in plaque stage and 3 cases in tumor stage. The average number of lesions were 1.9±0.9. The median age of these patients was (46.0±9.4) years. The average course of disease was (12.4±7.7) years. Psoralen was applied topically on treatment area 30 min before total body UVA irradiation treatment, 3 times a week. And the efficiency and safety of the therapy were evaluated.

RESULTSAll the patients were treated with topical PUVA with a median total dose of (161.60±135.96) J/cm2 in an average of (18.10±14.61) fractions. Total dose of UVA was (1 953.25±829.73) J/cm2, and total number of treatment was (261.90±116.79) fractions. The total treatment time was (45.80±26.64) months. Complete clinical response (CR) rate was 60.0%, partial response (PR) rate was 30.0%, and the overall response rate (CR+PR) was 90.0%. One patient showed no response. No severe acute or chronic side effects were observed.

CONCLUSIONTopical PUVA therapy is effective in the treatment of refractory lesions of mycosis fungoides with little severe side effects.

Adult ; Female ; Ficusin ; therapeutic use ; Humans ; Male ; Middle Aged ; Mycosis Fungoides ; drug therapy ; pathology ; PUVA Therapy ; Photosensitizing Agents ; therapeutic use ; Treatment Outcome

OBJECTIVETo determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides (MF).

METHODSFrom 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.

RESULTSThe 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.

CONCLUSIONLocal radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.

Adult ; Aged ; Antineoplastic Agents ; administration & dosage ; therapeutic use ; Chemoradiotherapy, Adjuvant ; methods ; Disease-Free Survival ; Female ; Humans ; Interferons ; administration & dosage ; therapeutic use ; Lymphoma, T-Cell, Cutaneous ; drug therapy ; pathology ; radiotherapy ; Male ; Middle Aged ; Mycosis Fungoides ; drug therapy ; pathology ; radiotherapy ; Neoplasm Staging ; PUVA Therapy ; methods ; Palliative Care ; methods ; Radiotherapy Dosage ; Skin Neoplasms ; drug therapy ; pathology ; radiotherapy ; Treatment Outcome

OBJECTIVETo investigate the significance of detecting TCR gene clonal rearrangement in the diagnosis of mycosis fungoides (MF) and to optimize the primers used for detecting the TCR gene clonal rearrangement with PCR in paraffin embedded tissues of MF.

METHODSNineteen cases of MF were enrolled into the study. A panel of 10 antibodies were used for immunophenotypic analysis and polymerase chain reaction for TCR-γ and TCR-β gene rearrangement detection in this study.

RESULTSTCR gene clonal rearrangements were detected in all 19 cases, in which 84.2% cases (16/19) had TCR-γ gene clonal rearrangements. The positive rates of the primers T(VG)/T(JX), V(2-5)/V(8-12)/JGT(1) and BIOMED-2-TCR-γ were 47.4%, 78.9% and 31.6%, respectively. The positive rate of V(2-5)/V(8-12)/JGT(1) was statistically significantly higher than that of T(VG)/T(JX) and BIOMED-2-TCR-γ (P < 0.05). No TCR gene clonal rearrangement was detected using the primers V(γ11)/V(γ101)/Jγ12 and V(γ11)/V(γ101)/J(p12). TCR-β gene clonal rearrangement was detected in 31.6% (6/19) cases.

CONCLUSIONSTCR gene clonal rearrangement analysis is a useful tool in the diagnosis of MF and TCR-γ gene is a good target gene for the detection. The primers T(VG)/T(JX), V(2-5)/V(8-12)/JGT(1) and BIOMED-2-TCR-γ can be used in clinicopathologic detection for TCR gene clonal rearrangement and V(2-5)/V(8-12)/JGT(1) may be the first choice.

Adolescent ; Adult ; Aged ; Antigens, CD7 ; metabolism ; Base Sequence ; CD2 Antigens ; metabolism ; CD3 Complex ; metabolism ; CD4 Antigens ; metabolism ; Child ; Child, Preschool ; Female ; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Humans ; Leukocyte Common Antigens ; metabolism ; Male ; Middle Aged ; Molecular Sequence Data ; Mycosis Fungoides ; diagnosis ; genetics ; metabolism ; pathology ; Paraffin Embedding ; Receptors, Antigen, T-Cell, alpha-beta ; genetics ; Receptors, Antigen, T-Cell, gamma-delta ; genetics ; Skin Neoplasms ; diagnosis ; genetics ; metabolism ; pathology ; Young Adult

OBJECTIVETo update the clinical characteristics of mycosis fungoides in Chinese patients.

METHODWe retrospectively analyzed the clinical data of 51 patients (29 men and 22 women) with mycosis fungoides in PUMC hospital from 1984 to 2006, to determine the ages at diagnosis, clinicopathologic characteristics of skin lesions, systemic manifestation, misdiagnosis and treatment of these patients.

RESULTSThe mean age was (44.24 +/- 2.05) years at the diagnosis. Most patients were characterized by the typical evolution of patches, plaques and tumors, with some variations and subtypes. Clinical manifestations included generalized lesions (68.6%) and itchy (70.6%). Epidermotropism (68.6%) and Pautrier's microabscesses (52.9%) were common histopathologic features. The positive rate of T-cell receptor gene rearrangement was 81.3%, and was independent of the histological features. Previous misdiagnosis rate was 64.7%. Skin-targeted therapies and biologic therapies were effective approaches to relieve the skin rash at early stage, and combined chemotherapy was typically applied in more advanced cases.

CONCLUSIONMycosis fungoides has various clinical characteristics and careful differential diagnosis should be made in clinical practice.

Diagnostic Errors ; Female ; Gene Rearrangement ; Humans ; Male ; Mycosis Fungoides ; diagnosis ; pathology ; Receptors, Antigen, T-Cell ; genetics ; Retrospective Studies ; Skin Neoplasms ; diagnosis ; pathology

Papuloerythroderma of Ofuji (PEO) is an uncommon entity of unknown etiology, characterized by coalescing erythematous papules sparing skin folds. A number of cases have described the association of this disorder with malignant with pathology, mainly with cutaneous T cell lymphoma. Such reports give rise to the suggestion that PEO may be a precursor of lymphoma or a form of cutaneous T cell lymphoma. We report a case of PEO, which was diagnosed as cutaneous T-cell lymphoma only 2 months after the development of skin lesions, and rapidly progressed. This case suggests the presence of a variant of cutaneous T cell lymphoma with clinical feature of PEO, which is different from mycosis fungoides or S zary syndrome.
Lymphoma ; Lymphoma, T-Cell, Cutaneous* ; Lymphoma, T-Cell, Peripheral ; Mycosis Fungoides ; Pathology ; Skin

A seven-year-old castrated male Yorkshire terrier dog was presented for a recurrent skin disease. Erythematous skin during the first visit progressed from multiple plaques to patch lesions and exudative erosion in the oral mucosa membrane. Biopsy samples were taken from erythematous skin and were diagnosed with epitheliotropic T cell cutaneous lymphoma by histopathology and immunochemical stain. In serum chemistry, the dog had a hypercalcemia (15.7 mg/dl) and mild increased alkaline phosphatase (417 U/l). Immunohistochemistry was performed to detect parathyroid hormone-related peptide (PTH-rP) in epitheliotropic cutaneous lymphoma tissues but the neoplastic cells were not labeled with anti-PTH-rP antibodies. The patient was treated with prednisolone and isotretinoin. However, the dog died unexpectedly.
Animals ; Dog Diseases/drug therapy/*pathology ; Dogs ; Fatal Outcome ; Isotretinoin/therapeutic use ; Male ; Mycosis Fungoides/drug therapy/pathology/*veterinary ; Prednisolone/therapeutic use ; Skin Neoplasms/drug therapy/pathology/*veterinary

OBJECTIVESTo detect telomerase activity in patients with mycosis fungoides (MF) and to study the role of telomerase in the tumorigenesis of MF.

METHODSThe technique of PCR-ELISA was employed to detect telomerase activity in 35 patients with various stages of MF.

RESULTS92.3% tumor stage of MF, 78.6% plaque stage of MF and 75.0% patch stage of MF had positive telomerase activity. The control samples had no telomerase activity. Telomerase activity in tumor stage of MF was significantly higher than that in plaque stage, while the latter was higher than that in patch stage. Telomerase activity was correlated with the stage of MF.

CONCLUSIONHigh level of telomerase activity frequently occurred in patients with MF, suggesting that telomerase might play an important role in the tumorigenesis of MF and is a useful marker for the diagnosis of MF possibly.

Humans ; Mycosis Fungoides ; enzymology ; pathology ; Neoplasm Staging ; Skin Neoplasms ; enzymology ; pathology ; Telomerase ; metabolism

Within the past few years, an increasing number of reports of Hodgkin's disease following the diagnosis of, and frequently coexisting with, mycosis fungoides have appeared. Previously, Hodgkin's disease found in the lymph nodes of the patient diagnosed as mycosis fungoides was considered as a transformed form of the mycosis fungoides. But, now it has been proven that Hodgkin's disease and mycosis fungoides are histologically and immunohistochemically distinct disease entities. We report a well-documented case of a man who developed Hodgkin's disease and mycosis fungoides simultaneously as a composite lymphoma. Our case emphasizes the importance of considering the diagnosis of another lymphoma in patients with mycosis fungoides who have lymphadenopathy. The cutaneous mycosis fungoides and the Hodgkin's disease should be treated as an independent disease.
Adult ; Case Report ; Hodgkin Disease/*pathology ; Human ; Male ; Mycosis Fungoides/*pathology ; Neoplasms, Multiple Primary/*pathology ; Skin Neoplasms/*pathology