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PubMed2000

T-cell lymphoproliferative disorder of vitreous associated with mycosis fungoides.

59-year-old man with a history of mycosis fungoides developed loss of visual acuity and visual field in the left eye. Epiretinal lesions were present in the right eye and multifocal choroidal lesions, optic disc edema, and vitritis were present in the left eye. A diagnostic vitrectomy was performed and cytologic examination of the vitreous confirmed the diagnosis of T-cell lymphoproliferative disorder. Systemic and intrathecal chemotherapy resulted in marked improvement in ocular signs and symptoms. At last follow-up, the patient was found to have improved visual acuity in the left eye; however, significant worsening of his systemic condition developed and he died shortly thereafter.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1977

Mycosis fungoides. Intraocular and central nervous system involvement.

A 58-year-old man with mycosis fungoides was treated with chemotherapeutic agents and radiation for the recurrent cutaneous form of this disease. Five years after the onset, he developed blurring of vision, neurologic signs, and then lapsed into coma. Chemotherapy and radiation treatment resulted in improvement of the ocular and systemic picture. Within three months, however, the patient's vision deteriorated and swelling of both optic discs, along with retinal and vitreous infiltrates, was noted. Local radiation of the eyes was followed by improvement of the ocular changes. Five months later the patient died. Results of a histologic examination of the eyes showed extensive involvement of the retina and vitreous by tumor cells. The CNS was remarkably free of tumor cells and it was assumed that the radiation and chemotherapy were responsible for this.

Central Nervous System Diseases↗
PubMed1998

Value of clonality studies of cutaneous T lymphocytes in the diagnosis and follow-up of patients with mycosis fungoides.

Histological features of early mycosis fungoides (MF) can simulate numerous inflammatory lesions and histological confirmation of MF is often delayed, compared with clinical diagnosis. Recently, using molecular techniques, the detection of a dominant T-lymphocyte clone has been reported in cutaneous lesions of MF. The aim of the present study was to determine the diagnostic value of a dominant T-lymphocyte clone as assessed by PCR-DGGE in early MF. Histopathological and molecular analyses were performed on cutaneous lesions from 104 patients clinically suspected as having MF. In this population, the positive predictive value of a PCR gamma(+) was 0.86. In addition, four of six patients whose lesions were PCR gamma(+) (detectable dominant T-cell clone) but not histologically MF progressed to MF within 2-48 months. In order to evaluate the relevance of PCR gamma-DGGE in MF follow-up, serial biopsies were performed in 24 patients. In 89 per cent of cases, the presence or absence of a PCR gamma(+) was constant during the course of the disease. When present, the DGGE imprint of PCR products was case-specific. These data demonstrate the diagnostic value in MF of T-lymphocyte clonality assessed by PCR gamma-DGGE on cutaneous lesions and show that the technique can be used in MF follow-up to evaluate residual disease with high specificity.

Clone Cells↗
PubMed1998

Intramammary lymph node involvement by mycosis fungoides diagnosed by fine-needle aspiration biopsy.

We report on a case of a patient with diagnosis of mycosis fungoides (MF) who subsequently developed enlargement of the intramammary lymph nodes, which were demonstrated to be involved by tumor on fine-needle aspiration (FNA) cytology. The patient was a 66-yr-old female who had recently noticed the appearance of circumscribed bilateral breast nodules. A mammographic study showed clearly outlined nodules of low density. FNA showed atypical small and large lymphocytes with cerebriform appearance, consistent with intramammary lymph node infiltration by MF. To our knowledge this is the first report of intramammary palpable lymph nodes involved by MF and confirmed by FNA. This case provides support for the use of fine-needle aspiration biopsy as an accurate method for staging MF successfully.

Aged↗
PubMed1978

Total skin electron beam therapy in mycosis fungoides.

Thirty-nine patients with mycosis fungoides were treated with total skin electron beam irradiation from 1971 to 1976, and all received minimum follow-up of 12 months. Details of treatment technique and dosimetry are given. Sixteen patients cleared completely. Seven patients are in long-term remission (1 1/2 to 4 1/2 years). Eleven patients died. Those who failed are now being treated with topical therapy with varying degrees of control. Some have been retreated with local irradiation. Most of the patients had extensive disease; for this group, total skin electron beam therapy was especially useful, as many of those who failed developed only minimal disease that was well controlled by topical measures.

Administration, Topical↗
PubMed1979

Mycosis fungoides and Hodgkin's disease occurring in the same patient: report of three cases.

The patients with the typical clinical course and pathology of Mycosis Fungoides (MF) were found also to have Hodgkin's Disease (HD), nodular sclerosing type. In two cases, HD was diagnosed 2 years after the diagnosis of MF; in the third case, both diseases were diagnosed simultaneously. Previous claims of MF transforming into second lymphomas are reviewed. The possible significance of our observed association of MF and HD is discussed.

Hodgkin Disease↗
PubMed1980

Dermatopathic lymphadenopathy and lymph node involvement in mycosis fungoides.

Lymph node involvement in mycosis fungoides (MF) is associated with a poor prognosis, Histologically, in most cases of clinical lymphadenopathy the excised lymph node shows dermatopathic lymphadenopathy (DL). The diagnosis of MF involvement can readily be made when the lymph node tissue has partly or wholly been replaced by atypical lymphoreticular tissue. Early involvement of a dermatopathic lymph node by MF may be difficult to diagnose. A histologic study was performed on 30 lymph nodes from 24 patients with MF. Most of these lymph nodes had been excised as part of the staging procedure. The maximal follow-up period was five years. A classification of lymph node involvement into four categories is suggested and correlations with clinical courses and results of DNA cytophotometry of lymph node imprints are shown. Lymph nodes showing the histologic picture of DL can be divided into two groups: a group with MF involvement (category I) and a group with MF involvement (category II). The latter group is considered to represent early involvement of lymph nodes by MF. Partial or complete replacement of lymph node tissue by atypical lymphoreticular tissue corresponds with cagegories III and IV, respectively.

Adult↗
PubMed1980

Early diagnosis of mycosis fungoides and Sézary's syndrome by morphometric analysis of lymphoid cells in the skin.

Mycosis fungoides (MF) and Sézary's syndrome (SS) are cutaneous T-cell lymphomas characterized ultrastructurally by the presence of lymphoid cells with deep and narrow nuclear indentations (cerebriform mononuclear cells or CMC). Early diagnosis of MF and SS is difficult because in their early stages they often resemble various forms of chronic, benign skin lesions. By measuring the frequency distribution of a nuclear shape parameter (nuclear contour index or NCI) of lymphoid cells in skin infiltrates using computer assisted planimetry, we tried to classify suspect cases into the malignant and benign groups. From 12 patients with MF or SS (malignant group) and 11 patients with chronic, benign skin lesions (benign group) the frequency distribution of the NCI of the lymphoid cell population was measured. Nonlinear discriminant analysis selected the 70th and 25th percentile of the NCI distribution of the lymphoid cells in the skin infiltrates as parameters by which these patients could be classified correctly into the malignant or benign groups with a probability of over 95%. The predictive value of these parameters was tested on ten patients suspected of having cutaneous T-cell lymphomas. Three cases were classified as benign and 7 as malignant. In a three-year follow up cutaneous T-cell lymphomas did not develop in any of the 3 cases classified as benign, MF developed in 5 of 7 cases classified as malignant, 1 patient has lymphomatoid papulosis and 1 patient is still suspect for MF. These results are compared with those of DNA cytophotometry performed on skin imprint preparations. It is concluded that morphometry of lymphoid cells in skin lesions of patients suspect for MF and SS can make an important contribution to an early diagnosis of MF or SS.

Adult↗
PubMed1980

Cell-mediated immunity in patients with mycosis fungoides in clinical remission: effect of thymic humoral factor on the immunocompetence of the lymphocytes.

In 10 patients with mycosis fungoides (MF) who were in clinical remission following local treatment with nitrogen mustard, cell-mediated immunity was investigated by means of tests for lymphocytes, forming rosettes in the peripheral blood, and the graft-vs-host reaction as well as intradermal skin tests. The effect of thymic humoral factor (THF) on T lymphocytes with impaired immunecompetence was also investigated. The results provided evidence of various kinds of damage to the immunologic system in most of the patients. Of the 10 patients, 4 had low percentages of T cells. Eight of the 10 had a graft-vs.-host reaction which was weak or negative, and in 4 of these, the administration of THF to the lymphocytes induced an improvement in immunoreactivity. These results together with other recently reported data indicate that the determination of cell-mediated immunity in patients with MF may be of prognostic importance. Those patients whose T lymphocytes respond to treatment with THF are probably the best candidates for immunotherapy.

Adult↗
PubMed1981

Lymph node biopsy in mycosis fungoides.

Ninety lymph node biopsy specimens from 76 patients with mycosis fungoides (MF) were reviewed. Dermatopathic lymphadenopathy was the most common change found in biopsies of palpable lymph nodes obtained from patients with MF. The paracortical expansion and histiocytes with elongated, folded, and delicate nuclei were characteristic. Lymph node involvement by MF lymphoma had a broad spectrum of histologic appearance. Most commonly, MF lymphoma was composed of atypical, often hyperconvoluted, lymphoid cells showing a wide variation in size. The pretreatment biopsies from 38 patients were graded according to the number of atypical small lymphocytes (AL) in the paracortical region and the results were correlated with survival. No significant differences in survival were found among the various grades, which ranged from dermatopathic lymphadenopathy without AL to frank involvement by MF lymphoma. Patients with more extensive skin disease tended to have a higher grade and to do poorly.

Biopsy↗
PubMed1981

Staging lymphography with respect to lymph node histology, treatment, and follow-up in patients with mycosis fungoides.

Extracutaneous involvement in mycosis fungoides (MF) is associated with a poor prognosis. Therefore, a pretreatment staging procedure is used to determine the extent of the disease. As part of the staging procedure lymphography was performed in 36 patients with MF. The radiographic findings and the correlation of these findings with the histopathologic changes in lymph nodes and the clinical course are described. Lymphography in MF shows a variety of abnormalities. However, these abnormalities correlate in only 61% of the patients with the extent and the clinical course of the disease. It is concluded that lymphography is not a useful clinical tool in the staging of MF.

Adult↗
PubMed1981

Quantitative electron microscopic comparison of lymphocyte nuclear contours in mycosis fungoides and in benign infiltrates in skin.

Quantitative electron microscopy was used to measure lymphocyte nuclear contours in skin biopsies from 109 patients, 77 with benign disorders, 16 with early mycosis fungoides (MF), and 16 with controversial lesions. A nuclear contour index (NCI) was calculated on electron micrographs by dividing the nuclear profile circumference by the square root of the nuclear area. Significant differences were found between the group mean NCI for MF (NCI = 6.1 +/- 0.1) and for the benign group (NCI = 4.6 to 5.4 +/- 0.1; P less than 0.005). The group mean NCI for the controversial cases was significantly lower than that of the MF group (P less than 0.05). It is suggested that a definite electron microscopic diagnosis of MF requires first a patient mean NCI of 6.1 or more, and second, at least 6% of lymphocytes with an NCI of 9 or more. These criteria give a false positive rate of 3% in benign and controversial disorders and a 50% false negative rate for early MF. Only lymphocytes with an NCI of 16 or more appear specific for MF (or Sezary's syndrome), but are not found in all cases.

Aged↗
PubMed1981

Prognostic variables in mycosis fungoides.

The prognostic importance of various factors was analyzed for 347 patients registered by the Mycosis Fungoides Cooperative Group (MFCG). Extent of skin involvement at the time of registration and number of sites of clinically enlarged lymph nodes were clearly the most important prognostic variables, and were combined into a single variable, TN stage, for adjusted analyses of other factors. Sex, history of tonsillectomy, immunoglobulin E, and telangiectasia did not significantly affect survival. The effects of race and allergic history were questionable and need further study. Age, pruritus, burning, alopecia, ulcers, and erosion were important when studied alone, but their effects were no longer statistically significant after adjustment for TN stage. Chills and malaise, the latter a symptom of debility, retained prognostic significance after such adjustment. This analysis demonstrates the importance of considering the joint effects of multiple prognostic factors rather than analyzing them one at a time.

Adult↗
PubMed1982

Noncutaneous peripheral T-cell lymphoma histologically resembling mycosis fungoides.

We have studied the clinical and pathologic features of three patients with a distinctive non-Hodgkin's lymphoma of the diffuse mixed cell type. The tumors were characterized by a mixture of lymphoid cells of widely varying size and degree of nuclear transformation. A distinctive feature of the neoplastic cells was the presence of cerebriform nuclear contours identical to those seen in mycosis fungoides. Immunologic studies performed on lymph nodes from two patients revealed that the neoplastic cells were T-cells. Characteristic clinical features of the patients were an advanced stage of disease at diagnosis, absence of skin lesions, presence of lung involvement, poor response to aggressive chemotherapy, and a short survival time (9-13 months). The features described closely resemble those of the pleomorphic variant of Japanese adult T-cell leukemia-lymphoma.

Adult↗
PubMed1983

Ointment-based mechlorethamine treatment for mycosis fungoides.

The treatment of skin disease with topical mechlorethamine has been restricted because of the frequent development of contact dermatitis. A series of 43 patients with mycosis fungoides in Stages 1A (17), IB (22), II (2), and III (2) were treated with an ointment-based mechlorethamine, prepared by an anhydrous method. Complete clearing occurred in 26 patients over a 42-month evaluation period. The incidence of contact dermatitis was very low. Only 1 of 31 patients exposed to mechlorethamine for the first time, and only 3 of 12 patients with a history of previous hypersensitivity to mechlorethamine, developed contact dermatitis to the ointment-based mechlorethamine.

Administration, Topical↗
PubMed1984

Mycosis fungoides and lymphoplasmacytoid immunocytoma in the same patient. A case report.

The simultaneous occurrence of two different lymphomas in a 57-year-old white woman is reported: mycosis fungoides and a leukemic lymphoplasmacytoid immunocytoma. The first was confirmed by histologic study and electron microscopy, and the latter by histologic study and immunoperoxidase staining. The lymphoid cells in the involved bone marrow and peripheral blood expressed the same surface immunoglobulin as was found in the cytoplasm of the immunocytoma cells, i.e., IgM-lambda. The clonal B-cell expansion was brought into a lasting remission by chlorambucil, but the cutaneous lymphoma proved to be refractory to therapy. The patient died 38 months after diagnosis.

Chlorambucil↗
PubMed1986

The value of nuclear contour index in the diagnosis of mycosis fungoides. An assessment of current ultrastructural morphometric diagnostic criteria.

Skin biopsy specimens from 43 patients with established mycosis fungoides (MF) and 28 with non-MF lymphoid skin infiltrations were studied by transmission electron microscopy. The nuclear contour index (NCI) of infiltrating lymphoid cells was obtained by means of a Zeiss Videoplan computer and measuring tablet. Classification of the material into MF and non-MF groups was done according to the diagnostic criteria used by McNutt et al. (1980, 1981) and Meijer et al. (1980); this study evaluates the usefulness of these previously published criteria based on quantitative electron microscopy. Statistical analysis confirmed that the mean NCI (MNCI) of the lymphoid cells of patients with MF is significantly greater than that in patients with other lymphoid infiltrates (P less than 0.001). However, all published diagnostic criteria (McNutt et al., 1980, 1981, and Meijer et al., 1980) tested in the authors' larger patient population yielded lower sensitivity than has been reported. The diagnostic criteria based on values of MNCI, percentage of lymphoid cells with NCI greater than 7, and percentage of lymphoid cells with nuclear profile area of greater than 30 microns2 reported by McNutt et al. (1981) appear to be better than others, and provide the highest sensitivity rate, which is in the range of 44% to 47%. There were no false-positive findings. It was concluded that the use of ultrastructural morphometry according to the techniques and diagnostic criteria of McNutt et al. (1981) is useful in the diagnosis of MF.

Adolescent↗
PubMed1987

Extracutaneous mycosis fungoides.

The clinical course of 40 patients with histologically documented extracutaneous mycosis fungoides (ECMF) was reviewed. Thirty one patients had documentation of nodal disease only (Stage IVA). Nine patients had histologic evidence of visceral involvement (Stage IVB). A wide variety of topical, regional and/or systemic therapies were used in the management of these patients. The median survival for the entire group was 14.5 months. Eleven patients (28%) obtained a complete response (CR) and had a median survival of 21 months as compared with 8 months among 29 patients not obtaining a CR. One patient is without evidence of disease (NED) at 10 years. Multiple prognostic factors were examined with respect to survival. Prognostic factors found to be significant at the P = 0.05 level included ECMF at presentation of skin disease (versus at the time of relapse), the ability to achieve a CR, and management incorporating the use of topical therapy, especially electron beam treatment. Tabulation of all trials of chemotherapy either at diagnosis of ECMF or subsequently revealed that cyclophosphamide, vincristine and prednisone (CVP) and cyclophosphamide, vincristine, prednisone, and bleomycin (COP-Bleo) were the most effective chemotherapeutic combinations. In a multivariate analysis with survival as the endpoint, the best model consisted of only two covariates: ECMF at presentation of skin disease, and the use of topical therapy. Other covariates found not to be significant at the P = 0.05 level included age, gender, clinical extent of cutaneous and extracutaneous disease, Stage IVA versus Stage IVB disease, the presence of Sezary cells in the peripheral blood smear, and management incorporating the use of systemic therapy.

Adult↗