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PubMed1987

Electron microscopic and immunolabeling studies of the lesional and normal skin of patients with mycosis fungoides treated by total body electron beam irradiation.

Biopsy specimens were taken from lesional and normal skin of nine patients with mycosis fungoides before and after total body electron beam therapy. By electron microscopy, lesional skin had one and one-half to ten times as many epidermal Langerhans cells and indeterminate cells as did the normal skin. In successfully treated lesional skin 1 month after the end of electron beam therapy, the density of epidermal Langerhans cells and indeterminate cells had decreased markedly. In incompletely resolved lesions, Langerhans cells and indeterminate cells were still at pretreatment levels. Epidermal T6 and Ia antigens showed the same pattern of response. Epidermal cell suspensions from lesional and normal skin before and after electron beam therapy were assayed for epidermal thymocyte activating factor. The values of production of this factor did not correlate with the source of the epidermal cells, response to therapy, or the patient's disease course. Skin lesions resembling xerosis and parapsoriasis and histologically lacking the criteria for mycosis fungoides appeared during clinical remissions. These nonspecific skin lesions had densities of epidermal Langerhans cells, indeterminate cells, and T6-positive and Ia-positive cells comparable to levels found in pretreatment lesional skin.

Antibodies, Monoclonal↗
PubMed2000

Radiotherapy of advanced mycosis fungoides: indications and results of total skin electron beam and photon beam irradiation.

BACKGROUND: The goals of this retrospective study of advanced mycosis fungoides are (1) to describe the indications of a combination of total skin electron beam and photon beam irradiation and (2) to analyze the results of total body or segmental photon irradiation for patients with extension beyond the skin. METHODS: From January 1975 to December 1995, 45 patients with pathologically-confirmed mycosis fungoides or Sézary syndrome received a combination of TSEB and photon beam irradiation for advanced disease: 34 males and 11 females, mean age 61 years (range 27-87 years). The mean follow-up was 111 months (range 18-244 months, median 85 months). Whole-skin irradiation treatment to a depth of 3-5 mm with a 6-MeV electron beam was produced by a linear accelerator to a total dose of 24-30 Gy in 8-15 fractions, 3-4 times a week. In cases of thick plaques or tumors that were beyond the scope of low energy electron beams or for treating nodal areas (especially in the head and neck area or axilla involvement), regional irradiation (RRT) with Co-60 photon beams was followed by whole-skin electron beam irradiation (15 patients). In cases of diffuse erythrodermia, Sézary syndrome, nodal or visceral involvement, total body irradiation was delivered with a 25-MV photon beam using a split-course regimen to prevent hematological toxicity (22 patients). The first course consisted of 1.25 Gy delivered in ten fractions and 10 days. Subsequently, patients received TSEB. Four to 6 weeks after TSEB, they received a second course of 1.25 Gy. The cumulative TBI dose ranged from 2.5 to 3 Gy in about 3 months. Hemi-body irradiation (HB) with Co-60 (and a bolus) was given in cases of multiple regional tumors with large and thick infiltration of the skin to a dose of 9-12 Gy (using fractions of 1-1.5 Gy/day) which, once flattened, were boosted with whole-skin electron beam therapy (8 patients). RESULTS: At 3 months, the overall response rate was 75% with 23/45 (51%) patients in complete response and 24% in partial response; one patient had stable lesions and 1 patient presented progressive disease. The overall response rate was 81% for T3 patients, 61% for T4, 79% for N1 and 70% for N3. The complete response rate was 67% for T3 and 28% for T4. Sixty-four percent of N1 patients and 41% of N3 had a complete response. The 5-year actuarial overall survival was 37% for T3 and 44% for T4 (P = 0.84). Patients with clinically abnormal lymph nodes that were pathologically negative (N1) presented a 5-year survival of 63%. Patients with pathologically positive lymph nodes (N3) experienced a 5-year survival rate of 32% (P = 0.040). CONCLUSIONS: TSEB provides an excellent quality of life by reducing itching and discharge from the skin. Patients with more advanced disease may be treated and cured by the addition of photon beams in combination with TSEB. A selection of patients with advanced skin disease and regional extension may be cured by a combination of TSEB and photon beam irradiation. The regional treatment allows the use of electrons after the reduction of the plaques or thick tumors and a prophylactic irradiation of the adjacent nodal area.

Adult↗
PubMed1975

Frequent low doses of intravenous mechlorethamine for late-stage mycosis fungoides lymphoma.

Ninety-two courses of daily low-dose intravenous nitrogen mustard were administered to 46 patients with advanced cutaneous lymphomas (mycosis fungoides, Sezary syndrome and lymphoma cutis). Seventy-eight % of patients showed objective clinical remission, and 35% reached a clinically disease-free state following 1 or more courses. The response rate was greater in patients with the plaque-tumor type of mycosis fungoides than in those with erythrodermic variants. Therapy was free of significant side effects.

Adult↗
PubMed1980

Ultrastructural identification of T lymphocytes in tissue sections of mycosis fungoides.

An immunoelectronomicroscopic method has been employed to demonstrate in situ the T lymphocyte nature of the dermal and epidermal infiltrates to mycosis fungoides. A specific antiserum to the human T lymphocyte surface antigen (HTLA) was used in an indirect reaction with a peroxidase labeled anti-immunoglobulin. After histochemically revealing the peroxidase activity, T cells were easily identified by the deposition of electron dense material on the cytoplasmic membrane. Counterstaining with uranyl acetate and lead citrate enabled us morphologically to observe the maturity of infiltrating T lymphocytes and to identify Sézary cells and other cells such as eosinophils in the infiltrate. Our results confirm the T cell nature of the dermal infiltrate in mycosis fungoides and show that the epidermal infiltrate of cells forming Pautrier micro-abscesses are predominantly T lymphocytes.

Adolescent↗
PubMed1986

Treatment of advanced stage mycosis fungoides with bleomycin, doxorubicin, and methotrexate with topical nitrogen mustard (BAM-M).

Ten patients with advanced stage (TNM IIB-IVB) mycosis fungoides were treated with a combination chemotherapy program consisting of bleomycin and methotrexate weekly, doxorubicin every 3 weeks, and topical nitrogen mustard daily (BAM-M). Seven patients obtained histologically documented complete remissions ranging from 4 to 105+ months in duration. Median survival is 16.5+ months. Three patients in whom splenomegaly was detected during their staging evaluation underwent splenectomy. These three patients have had unmaintained disease-free survivals of 36+, 100+, and 105+ months. This study indicates that BAM-M is effective therapy for advanced stage mycosis fungoides and suggests that the therapeutic role of splenectomy should be evaluated further.

Adult↗
PubMed1986

[Mycosis fungoides and Sézary syndrome. A retrospective study of 60 patients with correlation between histologic classification and survival time].

47 patients suffering from mycosis fungoides and 13 with Sézary's syndrome were retrospectively studied with regard to prognosis in relation to the histologic substrate of cutaneous lesions. On account of the wide spectrum of T-cell cytomorphology as well as the pattern and composition of the infiltrate, we distinguished 4 histopathological types: small cell lymphoid type (45%), mixed cell polymorphic type (40%), large cell type (immunoblastic and pleomorphic, 10%), and medium-sized cell type (5%). Statistical analysis revealed survival rates of 10 years in 68.3% for the small cell lymphoid type and in 30.3% for the mixed cell polymorphic type. All patients showing the large cell type died within 6 years after diagnosis; those bearing the medium-sized cell type died within 2 years after diagnosis. The forms of mycosis fungoides showing the last two types of infiltrate may be classified as lymphomas of extreme malignancy. The different histological features are not only significant with regard to the diagnosis but also permit prognostic estimation before systemic signs have become evident.

Adult↗
PubMed1994

Presence of Epstein-Barr virus in cutaneous lesions of mycosis fungoides and Sézary syndrome.

It has been suggested that prolonged antigenic stimulation contributes to the development of epidermotropic cutaneous T cell lymphoma (CTCL), mycosis fungoides and Sézary syndrome, characterized by a cutaneous infiltration of proliferating helper T cells. Since Epstein-Barr virus (EBV) antibodies were increased in CTCL sera, we investigated a possible etiologic role for EBV in epidermotropic CTCL by looking for the EBV genome in 25 cutaneous biopsies of mycosis fungoides or Sézary syndrome and 12 reactional inflammatory skin lesions. The use of a non-isotopic in situ hybridization procedure based on the detection of Epstein-Barr encoded RNAs with biotinylated oligonucleotide probes (EBER) revealed 32% of the lesions with CTCL to be positive for EBV (3 in dermis, 3 in epidermis, 2 both in dermis and epidermis), as compared to no detection of the EBV genome in the reactional inflammatory skin lesions. Moreover, a combined hybridization (EBER probe) and immunochemistry technique (anti-CD3 or anti-Kil monoclonal antibody) permitted the identification of EBV in T cells of dermis and in keratinocytes, respectively. The identification of EBV in epidermotropic CTCL suggests that this virus could play a role in the development of these CTCLs, either as an etiological agent or more probably as a chronic activating agent. Indeed, the infection of keratinocytes by EBV could activate them and so induce the production of in situ cytokines (IL1a, IL6, TNFa) playing a role in the development of tumoral infiltrate.

Adult↗
PubMed1983

Palliative radiation treatment of cutaneous mycosis fungoides--a dose response.

Between 1966 and 1981, 20 patients (191 lesions) underwent palliative radiation therapy for control of biopsy-proven cutaneous mycosis fungoides. Six patients (47 lesions) and an additional 34 lesions from the remaining 14 patients with complete response to treatment were excluded from the study because of follow-up of less than one year. Included in the remaining 110 lesions were all recurrences and all partial responses. The modalities for treatment included superficial X rays, Cobalt-60 or electron beam irradiation. The total tumor doses employed ranged from 600-4000 cGy. The 110 lesions (14 patients) were retrospectively analyzed to determine the dose required for local control of the lesions. Fifty-three percent of the lesions were classified as plaques, 20% as tumors less than or equal to 3 cm in diameter, and 27% as tumors greater than 3 cm in diameter. Complete response to treatment was observed in 95% of the plaque lesions, 95% of the tumors less than or equal to 3 cm in diameter and 93% of tumor greater than 3 cm in diameter. A complete response to treatment was noted in all lesions receiving greater than 2000 cGy. In the total population of lesions having a complete response, a local infield recurrence rate of 42% was noted in the group receiving less than or equal to 1000 cGy, 32% in those receiving 1001-2000 cGy, 21% in those receiving 2001-3000 cGy, and 0% in the group receiving greater than 3000 cGy. No infield recurrence was seen when the treated lesion received a total tumor dose greater than or equal to TDF of 49. Of those lesions which recurred, the mean time to recurrence for the first three dose ranges above were 5 months, 10 months and 16 months respectively. Eighty-three percent of the 30 recurrences were seen within one year of treatment; 100% of the recurrences occurred within two years of treatment. The data from this study indicate that tumor doses equivalent to at least 3000 cGy at 200 cGy per fraction, five fractions per week (TDF greater than or equal to 49) are needed for adequate local control of cutaneous mycosis fungoides lesions.

Cobalt Radioisotopes↗
PubMedDate not supplied

Primary peripheral T-cell lymphoma of the face other than mycosis fungoides. Computed tomography and magnetic resonance findings.

OBJECTIVE: To describe the radiologic findings of primary peripheral T-cell lymphoma (PTCL) of the face other than mycosis fungoides. METHODS: Computed tomography (CT) and magnetic resonance imaging (MRI) findings of 5 consecutive patients with pathologically proven primary facial PTCL other than mycosis fungoides were retrospectively evaluated. Patients with PTCL involving the sinonasal cavity or lymph nodes were excluded. RESULTS: Diagnoses of patients included in this study consisted of natural killer/T-cell lymphoma (n = 2), subcutaneous panniculitis-like T-cell lymphoma (n = 1), anaplastic large cell lymphoma (n = 1), and PTCL not otherwise specified (n = 1). Infiltration or swelling of the superficial space of the face was noted on both CT and MRI, mimicking inflammation or infection. Also seen were well-enhancing small nodular (n = 2) or infiltrative mass-like lesions (n = 2) within the areas of infiltration, which showed intermediate signal intensity on T2-weighted images. One patient demonstrated infiltration and swelling alone. CONCLUSIONS: Primary facial PTCL is a rarely encountered tumor and demonstrates infiltration or swelling mimicking inflammation or infection. Nodular or infiltrative mass-like lesions may be helpful for its diagnosis.

Adult↗
PubMed1981

Effect of high-dose thymidine infusions in patients with mycosis fungoides.

The antitumor effect of thymidine has been demonstrated in patients with leukemia and lymphoma. This report summarizes the treatment of three patients with mycosis fungoides, a chronic T-cell lymphoma. Four courses of thymidine (75 g/m2/day) were administered by continuous infusion for 4-7 days. Steady-state serum thymidine levels were in the range of 1-3 mM. Associated toxicities were minimal and consisted of milk headache and anorexia. Myelosuppression was manifested by transient declines in the peripheral leukocyte count. One patient had extensive clearing of diffuse erythematous plaques on the trunk and extremities that persisted for over one month. A second patient had partial clearing of plaques that persisted for two weeks following therapy and a third patient had a minimal response with 25% reduction in lymphadenopathy and noduloulcerative lesions. These responses indicate the effectiveness of thymidine as a single agent in the treatment of mycosis fungoides.

Aged↗
PubMed2002

Diffuse pulmonary involvement by mycosis fungoides: high-resolution computed tomography and pathologic findings.

The authors report the high-resolution computed tomography (HRCT) appearance of diffuse pulmonary involvement by mycosis fungoides in a 74-year-old woman whose clinical and radiographic manifestations simulated pneumonia. The HRCT showed multiple, dense, peribronchovascular nodules with surrounding ground-glass opacity and several wedge-shape peripheral opacities. The autopsy specimen revealed angiocentric and peribronchovascular involvement of mycosis fungoides and pulmonary infarctions distal to angiocentric infiltration of the tumor cells.

Aged↗
PubMed1994

Estimates of nuclear volume in plaque and tumor-stage mycosis fungoides. A new prognostic indicator.

It is well documented that mycosis fungoides (MF), a cutaneous T-cell lymphoma, has a variable clinical course. Unbiased stereological estimates of three-dimensional volume-weighted mean nuclear size (nucl vV) of mycosis cells were obtained in a retrospective study of 18 patients with a total of 34 biopsies of cutaneous plaque and tumor-stage MF. The value of nucl vV in the first sampled biopsy, as well as the average and highest values, were determined in biopsies from each patient. The patients were divided into two groups, either above or below the group median. There was a strong positive correlation between shorter survival and an average value of nucl vV > 104 microns 3 or a highest value of nucl vV > 126 microns 3 (2p = 0.002 and 0.003, respectively). A nucl vV > 91.6 microns 3 in the first biopsy was also suggestive of a shorter survival (2p = 0.07). There was no clear pattern of nucl vV evolution in the patients with multiple biopsies, but the impact of various therapeutic regimens cannot be assessed. Certain estimates of nucl vV appear to be good prognostic indicators in plaque and tumor-stage MF, but further study of a larger series of patients is needed to corroborate these results and assess the impact of differing therapeutic regimes.

Adult↗
PubMed2007

Juvenile mycosis fungoides treated with bexarotene and PUVA.

A 14-year-old Caucasian boy presented with a 4-month history of a slightly pruritic eruption that began on the hips and later extended to the trunk and upper and lower limbs. The patient did not present fever, weight loss, or asthenia. Physical examination revealed multiple, red, desquamative, oval patches with areas of healthy skin between them, which covered nearly 50% of the body surface area. The palms, soles, face, and mucosa were not affected. In addition, he presented two violet-colored infiltrated plaques on the left thigh and right buttock (Fig. 1). There were multiple, > 1 cm, freely mobile, axillary and inguinal nodes. In follow-up, the patient developed two red-colored, mobile, well-delimited cutaneous nodules of 2.5 cm in diameter in the right hemithorax and lumbar area. The lumbar nodule regressed spontaneously before treatment. The clinical diagnosis was mycosis fungoides. We obtained three skin biopsies, one from a patch lesion and the others from a nodule; the third was sent to a reference hospital to determine the rearrangement. Histologic examination was similar in the three biopsies and revealed an atypical lymphoid infiltrate in the superficial dermis with epidermotropism and a tumoral nodule of atypical, small-sized lymphocytes in the deep dermis and subcutaneous level (Fig. 2). The atypical infiltrate was CD3+, CD4+, CD8-, T-cell intracellular antigen (TIA)+/-, Epstein-Barr-encoded RNA (EBER)-, and CD56-. The biopsy of one left axillary adenopathy was compatible with mycosis fungoides (Fig. 3). Amongst the additional tests carried out was a blood analysis showing 5300 leukocytes (neutrophils, 35%; lymphocytes, 40.7%; monocytes, 16.8%; eosinophils, 6.40%) without Sézary cells, normal lactate dehydrogenase (LDH), immunoglobulin E (IgE) of 497 U/mL (normal, 3-100 U/mL), and beta2-microglobulin of 3.09 mg/L (normal, 1.64 +/- 0.58 mg/L). A bone marrow study and a thoraco-abdomino-pelvic scan were normal. The rearrangement in the skin was monoclonal, whereas in peripheral blood and lymph nodes it was polyclonal. With the diagnosis of mycosis fungoides stage IVA (according to the TNM classification), treatment was initiated with psoralen plus ultraviolet light A (PUVA), three times a week, plus oral bexarotene at a dose of 300 mg/m2/day. The parents were informed that this treatment was not approved for this age group and informed consent was obtained. The clinical tolerance to bexarotene was very good, although low doses of atorvastatin (10 mg/day) and 75-100 mg of thyroxine were needed to control the expected adverse reactions to oral retinoid. After 32 sessions of PUVA and 6 months of treatment with oral bexarotene, the skin patches regressed, except for the plaque on the left buttock and the nodule on the right hemithorax (Fig. 4). There was no evidence of lymphadenopathy clinically or via sonographic evaluation. Bexarotene was discontinued after patient clearance and resolution of adenopathies. Nevertheless, 5 months after discontinuation of oral treatment, the patient developed multiple, scaling, nonconfluent macules on the trunk and arms affecting almost 30% of the body surface area, which disappeared with the application of methylprednisolone aceponate. He did not present significant lymphadenopathies.

Adolescent↗
PubMed1987

Isotretinoin and cutaneous helper T-cell lymphoma (mycosis fungoides).

Retinoids, including isotretinoin, have demonstrated antiproliferative and antineoplastic activity in laboratory and clinical trials. In a phase II trial, 25 patients with extensive mycosis fungoides were evaluated for response to isotretinoin. There was a 44% (11 patients) objective clinical response rate with three clinical complete responses without concomitant evidence of pathologic clearing of the disease. An additional 24% (six patients) showed a minor degree of clinical improvement. The median time to response was two months (range, 0.5 to eight months) and the median response duration was eight months or longer (range, one to 25 months). Chronic toxic reactions consisted primarily of drying of the skin and mucous membranes and resulted in dose reduction in the majority of patients. It is concluded that isotretinoin produces significant clinical benefit to some patients with mycosis fungoides.

Adult↗