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[Further contribution to the etiology of mycosis fungoides].
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[The treatment of mycosis fungoides].
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[THE CIRCUMSCRIBED EOSINOPHILIC HYPERKERATOTIC-VEGETATING FORM OF MYCOSIS FUNGOIDES].
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[Remarks on the nosology of mycosis fungoides].
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Plasma cells in the dermal infiltrate of mycosis fungoides are of polyclonal origin.
There are several reports on the occurrence of immunoglobulin-producing B-cell lymphomas in Sézary's syndrome and mycosis fungoides, which may be the consequence of the helper activity of the neoplastic T-cells. Therefore we investigated skin biopsies of 50 patients with mycosis fungoides regarding the presence of plasma cells and their immunoglobulin profile. Nine of these patients had plasma cell nests, most frequently located at the lower edge of the infiltrate. IgE was detectable consistently, and IgG, IgM and IgA could also be demonstrated in the majority of these cases; kappa- and lambda-chains were present in equal amounts. Our results demonstrate polyclonal activation of plasma cells in a subgroup of mycosis fungoides patients.
Transformation in mycosis fungoides: the role of methotrexate.
BACKGROUND: Large cell transformation in patients with mycosis fungoides (MF) has been well reported in the literature. Although the risk factors have not been clearly elucidated, advanced stage seems to be associated with a higher incidence of transformation. Because MF is a rare disorder, little is known about the influence of other factors such as immunosuppressive therapy in the occurrence of transformation. OBJECTIVE: We questioned the role of methotrexate (MTX) in the transformation of MF to large cell lymphoma (LCL). METHODS: We identified all patients with MF who were registered in our cutaneous lymphoma database. Transformation was defined by the presence of large cells exceeding 25% of the infiltrate in at least one skin biopsy. In one patient, we followed the histologic, immunophenotypic, and genotypic changes taking place as transformation occurred. RESULTS: A total of 134 patients with MF were identified. Of 21 patients who received MTX, 3 transformed, and of the 113 patients in the non-MTX group, only 2 transformed. The incidence of transformation in the patients who received MTX was significantly higher than in those who did not receive the drug (14.3% vs. 1.8%; p = 0.03). This significance was maintained, even after controlling for stage and sex. For one patient who transformed, we demonstrated an identical dominant T-cell clone in all skin specimens, including the large cell lymphoma. CONCLUSION: Our results demonstrate a significant association between MTX exposure and transformation to LCL in patients with MF. In light of the small sample size, short followup of patients, and the inherent tendency of mycosis fungoides to transform, the role for MTX in transformation is unproven and needs to be confirmed in a multicenter study.
Defining early mycosis fungoides.
This editorial review summarizes the results of 5 meetings sponsored by the International Society for Cutaneous Lymphoma at which the clinicopathologic and ancillary features of early mycosis fungoides were critically examined. Based on this analysis, an algorithm was developed for the diagnosis of early mycosis fungoides involving a holistic integration of clinical, histopathologic, immunopathologic, and molecular biological characteristics. A novel aspect of this algorithm is that it relies on multiple types of criteria rather than just one, for example, histopathology. Before its finalization, the proposed diagnostic algorithm will require validation and possibly further refinement at multiple centers during the next several years. It is anticipated that a more standardized approach to the diagnosis of early mycosis fungoides will have a beneficial impact on the epidemiology, prognostication, treatment, and analysis of clinical trials pertaining to this most common type of cutaneous lymphoma.
Mycosis fungoides: a case study and nursing implications.
Mycosis fungoides is a rare and rapidly progressive form of cutaneous T-cell lymphoma. This article provides an overview of the disease process, as well as a case study to illustrate the complex nursing care of these clients.
Palmoplantar hyperkeratosis in mycosis fungoides.
The association of palmoplantar hyperkeratosis and mycosis fungoides (MF) has been described less frequently in the dermatologic literature. We present a patient with MF who developed hyperkeratosis of the palms and soles. Histologic examination of a specimen from the left palm showed microscopic changes of MF.
Second lymphomas and other malignant neoplasms in patients with mycosis fungoides and Sezary syndrome: evidence from population-based and clinical cohorts.
OBJECTIVE: To assess risks for developing second malignancies in patients with mycosis fungoides or Sézary syndrome. DESIGN: Retrospective study of 2 cohorts. SETTING: Nine population-based US cancer registries that constitute the Surveillance, Epidemiology, and End Results Program (SEER-9), and Stanford University referral center cohort of patients with cutaneous lymphoma. Patients with mycosis fungoides or Sézary syndrome from the SEER-9 registry diagnosed and followed up from 1984 through 2001 and from the Stanford University cohort diagnosed and followed up from 1973 through 2001. MAIN OUTCOME MEASURES: Relative risk was estimated using the standardized incidence ratio (SIR). The expected cancer incidence for both cohorts was calculated using age-, sex-, race-, and calendar year-specific SEER-9 incidence rates for the general population. Nonmelanoma skin cancers were excluded because these cancers are not routinely reported by the SEER database. RESULTS: In the SEER-9 cohort (n = 1798), there were 197 second instances of cancer (SIR = 1.32; 95% confidence interval [CI], 1.15-1.52) at all sites. Significantly elevated risk (P<.01) was observed for Hodgkin disease (6 cases; SIR = 17.14; 95% CI, 6.25-37.26) and non-Hodgkin lymphoma (27 cases; SIR = 5.08; 95% CI, 3.34-7.38). Elevated risk (P<.05) was also observed for melanoma (10 cases; SIR = 2.60; 95% CI, 1.25-4.79), and urinary cancer (21 cases; SIR = 1.74; 95% CI, 1.08-2.66). In the Stanford University cohort (n = 429), there were 37 second instances of cancer (SIR = 1.04; 95% CI, 0.76-1.44). Elevated risk (P<.01) was observed for Hodgkin disease (3 cases; SIR = 27.27; 95% CI, 5.35-77.54). Elevated risk (P<.05) was also observed for biliary cancer (2 cases; SIR = 11.76; 95% CI, 1.51-42.02). CONCLUSION: Updated SEER (population based) and Stanford (clinic based) data confirm the generalizability of earlier findings of increased risk of lymphoma in patients with mycosis fungoides or Sézary syndrome.
Defective monocyte chemotaxis in mycosis fungoides.
Monocyte-macrophage function was studied in 14 patients with histologically confirmed mycosis fungoides. Three component parts of the monocyte-macrophage system were examined. Monocyte bacterial phagocytosis and bacterial killing were normal. However, a defect in directional mobility (chemotaxis) was found in patients with mycosis fungiodes (P less than .0025) when compared to 35 healthy controls. Defective chemotaxis was present regardless of disease stage or therapy. This defect in monocyte chemotaxis represents a previously unrecognized immune deficiency and may help explain the frequent infections found in patients with mycosis fungoides.
Tumour progression in a patient with granulomatous mycosis fungoides.
We report a patient with granulomatous mycosis fungoides whose disease transformed into a high grade blast lymphoma with angiocentric features within 12 months of the initial diagnosis. This repudiates previous claims that granulomatous inflammation is protective in cutaneous T-cell lymphoma.
Second cutaneous malignancies in patients with mycosis fungoides treated with topical nitrogen mustard.
Previous reports of skin cancer occurring in mycosis fungoides patients who have been treated with topical nitrogen mustard have been of carcinomas occurring predominantly on sun-exposed skin. Four of twenty-nine mycosis fungoides patients in our clinic treated with topical nitrogen mustard developed six skin cancers on sun-protected skin. Four lesions were squamous cell carcinomas and two were basal cell carcinomas. Few other risk factors for the development of skin cancer were present in our patients. These cases support the hypothesis that topical nitrogen mustard is a carcinogen or tumor promote and emphasize the need for careful examination of the skin of patients treated with topical nitrogen mustard.
Epidermal Langerhans' cell densities influence survival in mycosis fungoides and Sézary syndrome.
Because Langerhans' cells (LC) (CD1a-positive epidermal cells) have been discussed to be involved in the pathogenesis of mycosis fungoides and Sézary syndrome, the authors examined the influence of densities of Langerhans' cells and, concurrently, of other phenotypes retrospectively on survival of 35 patients. Cell densities were assessed on cryostat sections (alkaline phosphatase antialkaline phosphatase-technique) of the respective diagnostic biopsy specimens. Additionally, two clinical parameters (age, stage of disease) were evaluated. CD1a-positive epidermal cells were demonstrated to be the only cell population being significantly associated (P = 0.011) with survival. Death resulting from mycosis fungoides and Sézary syndrome was significantly (P = 0.003) less frequent in patients with epidermal CD1a-positive cell densities higher than 90 cells/mm2 (optimal break point) as compared with patients with lower numbers. These results suggest that Langerhans' cells have a significant impact on prognosis of patients with mycosis fungoides and Sézary syndrome. They play an important role in the host defense mechanisms against these lymphomas rather than to favor their progression as proposed recently.
Topical mechlorethamine therapy for mycosis fungoides.
Patients with limited skin involvement by mycosis fungoides were treated with daily topical mechlorethamine. Seven of thirteen patients had a complete remission of diseases. Six of these seven experienced a contact dermatitis and four underwent a successful topical desensitization programme. The possible beneficial therapeutic effects of developing a contact allergy are discussed.
[Mycosis fungoides bullosa--a case report (author's transl)].
An extremely rare variant of mycosis fungoides, presenting with bullous lesions, is reported. Histopathological examination of the skin revealed subepidermal blister formation, a diffuse mononuclear infiltrate in the uppermost parts of the dermis and the characteristic intraepithelial Pautrier microabscesses. Lutzner cells were found at the ultrastructural level. The mechanism of blister formation in mycosis fungoides is outlined.