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[Disseminated type of pagetoid reticulosis? Mycosis fungoides?].

We present a case of a 69 year old man who died with a progressive systemic disease. This case demonstrated a multiple clinical evidence with aspects of psoriasis, parapsoriasis en plaques, mycosis fungoides, lymphogranulomatosis and finally of the disseminated type of pagetoid reticulosis. The last biopsies always demonstrated the typical histology of pagetoid reticulosis. The earlier biopsies revealed findings similar to mycosis fungoides and even in part similar to lymphogranulomatosis.

Aged↗

Mycosis fungoides. Small-bowel involvement complicated by perforation and peritonitis.

Mycosis fungoides (MF) is a malignant lymphoma that arises in the skin and subsequently may involve lymph nodes and viscera as part of the natural course of the disease. The disease has distinct histologic characteristics that may be recognized in extracutaneous tissues. A patient who had MF with ulcerating cutaneous nodules is presented herein. Death resulted from perforation of the small bowel involved by the disease and consequent peritonitis.

Female↗

A canine cutaneous lymphoproliferative disease resembling mycosis fungoides in man.

A dog with a lymphoproliferative disease resembling mycosis fungoides in man, the second reported canine case, had slowly progressive, multicentric cutaneous nodules and plaques. Nearly half the body surface was involved. The lesions consisted of multiple, flat, infiltrative, cutaneous tumors composed of heterogeneous, pleomorphic cells, some of which resembled histiocytes or activated lymphocytes. Intracutaneous foci of tumor cells and numerous tumor cells with bizarre convoluted nuclei were distinctive features that permitted the lesions to be distinguished from other non-epithelial cutaneous neoplasms.

Animals↗

Interferon-alpha-associated development of bullous lesions in mycosis fungoides.

We report a 67-year-old woman with mycosis fungoides (MF) who was receiving subcutaneous injections of recombinant interferon alpha-2b (IFN-alpha). After 2 months of IFN-alpha treatment, bullous lesions appeared on her trunk and extremities. A skin biopsy specimen from the trunk revealed histopathologic features of bullous MF. Bullous lesions with specific infiltrates of MF are very rare; to the best of our knowledge, this is the first case showing specific bullous lesions of MF developed under IFN-alpha treatment.

Aged↗

[Histological, electron-optical and histochemical findings in mycosis fungoides].

Skin excisions were investigated in 5 patients with verified mycosis fungoides. Findings yielded by light and electron microscopy helped to confirm the findings anticipated, and were correlated with histochemical observations. One of the cases involved a substantially higher activity of lysosomal enzymes, particularly KF and beta-glucuronidase in the skin infiltrate mycotic cells. In the other cases, this sort of activity was low. The significance of high ATPase activity in the peripheral cell membrane remains unclear. In one case, involvement in the T lymphocyte series was confirmed by the formation of rosettes.

Aged↗

[Breakdown of the energy of a fixed 7 MeV electron bundle in the treatment of mycosis fungoides (author's transl)].

In order to treat patients with mycosis fungoides, the authors were led to breakdown the energy of a 7 MeV electron bundle emitted by a linear accelerator. With this aim, they sought to determine the nature, thickness and position of the decelerating which should be used to obtain adequate flow and energy of the electron bundle. They then explain the technique of total body irradiation of the patients, and in particular the way in which the functions of the bundle are effected. Finally, in order to diminish under-dosage in regions of the human body irradiated tangentially, they suggest the use of a perspex screen of variable thickness.

Electrons↗

Evaluation of staining methods of resin embedded cutaneous tissue sections of mycosis fungoides.

Biopsies from lesional skin of patients with histologically confirmed mycosis fungoides were embedded in resin and semi-thin ( I micrometer) sections were submitted to a variety of staining procedures, including Toluidine blue, Richardson's Huber, Giemsa, Paragon and Tribasic stains. The stained sections were examined with the light microscope and the quality of cellular morphological detail afforded by the different stains was assessed. The Toluidine blue, Richardson's and Paragon stains were found to be technically easy to perform and gave the best representation of nuclear and cytoplasmic detail Of these, the authors favoured Richardson's stain. The technique of processing the tissue, and staining a mounting procedures are detailed.

Adolescent↗

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↗

Mycosis fungoides. Electron beam therapy.

The most effective treatment of late mycosis fungoides is total skin electron beam therapy. The beam at the Hammersmith Hospital in London has been adapted to treat these patients. Patients with advanced disease who have failed more conservative methods of treatment are irradiated. The electron beam is modified by the use of carbon and copper scatterers to produce an 80 percent depth dose at 5.5, 8 and 11.5 millimeters below the skin surface. The dose achieved in most patients is between 1500 rads and 2600 rads given in 10 to 13 treatments over 5-7 weeks. Recently the higher dose range has been employed and lithium flouride studies have shown that giving these doses from each of 4 fields, the dose achieved on the skin is approximately twice the given dose. The management of patients and the effects of treatment are discussed.

Electrons↗

[Systemic chemotherapy in mycosis fungoides (author's transl)].

The clinical experience with chemotherapy in mycosis fungoides is practically limited to the action of different types of chemotherapeutic agents on the lesions in far-advanced cases. A large number of agents from almost all classes--alkylating agents, antimetabolites, antibiotics, alkaloids, hormones--have some efficacy in clearing skin lesions. Despite this fact, combination chemotherapy has been the subject of a very small number of publications with conflicting results. The influence on survival, the comparative effects of combination versus single-agent chemotherapy, the value of chemotherapy combined to other treatment modalities have not been submitted to scientific evaluation. Considering the low frequency of the disease and the great variability of the clinical picture, it seems mandatory to implement large cooperative trials, possibly on an international scale.

Adrenal Cortex Hormones↗

Total skin electron beam irradiation for cutaneous T-cell lymphoma (mycosis fungoides).

Forty patients with the cutaneous T-cell lymphoma (mycosis fungoides) were treated with total skin electron beam irradiation. The total follow-up period was up to 116 months (median 57.5 months). The patients were irradiated with a total dose of 35 Gy over 10 weeks, using the six-field technique. Ten of these patients had lymph node involvement and were subsequently treated with chemotherapy. After the initial electron irradiation complete remission of the skin lesions was obtained in 87.5% of the patients. Relapse of skin lesions occurred in 52% of the patients after 2-72 months (median 4 months). Second line therapy consisted primarily of topical nitrogen mustard. The overall survival rate at 5 years was 70%. Despite the side-effects this treatment was tolerated well by all patients.

Adult↗

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↗

CD38- and Leu6-positive cells in dermal lesions of mycosis fungoides.

The phenotypes of mononuclear cells in 16 patients with mycosis fungoides (MF) (8 were in tumor stage and 8 in pretumor stage) were tested by ABC immunochemical technique. The results showed that the percentage of CD38 and Leu6(+) cells in tumor stage was higher than that in pretumor stage. CD38 was identified to be a marker of the immature lymphocytes. The change of CD38 might be helpful in staging MF and predicting the prognosis of the disease. In addition, Leu6(+) cells, oval or round in shape and without dendritic processes were found in tumor stage, suggesting that they might be immature lymphoid cells. This feature needs further investigation.

ADP-ribosyl Cyclase↗

Transition of Sézary syndrome into mycosis fungoides after complete clinical and molecular remission under extracorporeal photophoresis.

Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common clinical variants of cutaneous T cell lymphoma. Although thought to be closely related to mature T helper cells, the relation between the neoplastic cells in MF and SS is still not fully clarified. This report describes a patient with complete remission of SS under treatment with extracorporeal photophoresis (ECP), who subsequently developed typical plaques of MF and large cell lymphoma (LCL). Serial polymerase chain reaction analyses confirmed identical T cell receptor beta and gamma gene rearrangements in SS, MF, and LCL, and complete disappearance of the circulating malignant T cell clone from the peripheral blood after ECP. These findings indicate that the neoplastic cells in SS, MF, and LCL are derived from a common precursor T cell, despite the change in clinical phenotype.

Aged↗

Follicular mycosis fungoides mimicking a cutaneous B-cell lymphoproliferative disorder.

Follicular mycosis fungoides (MF) is an uncommon histological variant of MF characterized by infiltrates of atypical lymphocytes around and within the epithelium of the hair follicles (folliculotropism). Here we report a patient with rapidly progressive follicular MF on the face, associated with concurrent typical MF lesions elsewhere. The histology was unusual, as apart from dense lymphoid infiltrates showing folliculotropism and epidermotropism, there was a prominent B-cell component with germinal centres, leading to an initial diagnosis of cutaneous B-cell lymphoma. The final diagnosis of follicular MF was established on demonstration of clonal T-cell receptor gene arrangements and lack of clonality for heavy chain gene rearrangements. This case illustrates a variant of MF that has a more rapid progression than the otherwise indolent course of classical MF over many years, and the diagnostic pitfalls, whereby the histology can mimic a B-cell proliferative disorder.

B-Lymphocytes↗

Mycosis fungoides with a CD56+ immunophenotype.

We report 3 cases of mycosis fungoides (MF) with a CD56+ cytotoxic immunophenotype. Each patient presented with a different clinical phenotype: one exhibited limited poikilodermatous patches (skin stage T1); one, widespread hypopigmented lesions (skin stage T2); and one, poikiloderma with a single cutaneous tumor (skin stage T3). MF was confirmed both histologically and by the presence of a T-cell receptor clone in lesional skin in all cases. CD56 and T-cell intracellular antigen-1 were expressed by the malignant lymphocytes in all patients and two expressed CD8. No sample demonstrated loss of the pan T-cell markers CD2 or CD3. None of the 3 developed systemic disease and T-cell receptor gene analysis of peripheral blood was polyclonal in all cases. Only 3 cases of CD56+ MF have been reported previously, none of which exhibited tumor-stage disease. Currently, the disease in our patients appears to be behaving in a manner similar to that predicted for MF with a normal immunophenotype but the prognosis has to be guarded in view of the rarity of this subtype.

Adult↗

Reactivity of monoclonal antibody BE 2 in different stages of mycosis fungoides and in benign dermal infiltrates.

The cutaneous infiltrate in mycosis fungoides (MF) is predominantly composed of T4-positive T-cells. Attempts to distinguish the early stages of this condition from benign inflammatory infiltrates using anti-T3, T4 and other T-cell-associated antibodies have hitherto been unsuccessful. Recently a monoclonal antibody BE 2 has been described as selectively reacting against leukemic cells in patients with cutaneous T-cell lymphoma. To investigate whether the BE 2 antigen is differentially expressed in different stages of MF and benign dermatoses, thus facilitating diagnosis, especially of early MF, the reactivity of monoclonal antibody BE 2 against cutaneous infiltrates from such conditions was assessed. In the early stages of MF only a small number of reactive cells was present. In benign inflammatory infiltrates, especially in those that clinically and histologically were hardly distinguishable from early MF, BE 2 reactivity was essentially the same as in eczematous-stage MF. Lesions from plaque and tumor stage MF contained large numbers of BE 2-reactive cells. Our results indicate that expression of BE 2 is associated with the stage of a given MF lesion and is essentially identical in early MF and eczematous lesions with a similar histopathological appearance.

Antibodies, Monoclonal↗