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2 cases of malignant melanoma in 2 male patients, 68 and 63 years old, associated with mycosis fungoides and parapsoriasis en plaques, respectively, are reported. The parapsoriasis later on developed into mycosis fungoides. A pathogenetic linkage based on decreased cellular immunity in mycosis fungoides is suggested.
Nine patients with mycosis fungoides in different stages were treated with Bleomycin. Much better results were obtained with this new drug in the six patients with the infiltrative or beginning tumour stage than in the patients in the advanced tumour stage. Complete remission was not seen. In one case the results were objectivized by DNA cytophotometry. The role of Bleomycin in the treatment of mycosis fungoides is discussed. It is concluded that Bleomycin is not the medicament of choice for the treatment of mycosis fungoides.
BACKGROUND: Hypopigmented mycosis fungoides is a rare variant of mycosis fungoides (MF) that usually has a predilection for young individuals with dark complexion. OBJECTIVE: The aim is to describe new cases of hypopigmented MF with confirmed T-cell receptor gene rearrangement analysis. METHODS: This article includes case reports and a literature review. RESULTS: Three out of four hypopigmented MF patients had a positive TCR gene rearrangement. A fifth patient is reported who had hypopigmented mycosis fungoides and classical Pautrier microabscesses, for whom no TCR gene rearrangement analysis was performed. CONCLUSION: Although hypopigmented MF has a predilection for dark-complexioned populations, it can also affect Caucasian patients. In challenging cases, polymerase chain reaction can be a useful method for detecting early cases of hypopigmented MF.
Protein gene product 9.5 (PGP 9.5), a ubiquitin COOH-terminal hydrolase initially considered specific for neural and neuroendocrine tissues, is expressed in a variety of epithelial and mesenchymal tumors. During immunohistochemical evaluation of a cutaneous epitheliotropic T-cell lymphoma (mycosis fungoides [MF]) in a dog, strong reactivity for PGP 9.5 was observed. This unexpected result prompted us to examine PGP 9.5 immunoreactivity in 13 additional cases of canine mycosis fungoides. All tumors were confirmed as T-cell epitheliotropic lymphoma by histopathology and immunohistochemistry for CD3. Eight of 14 cases were positive for PGP 9.5, with reactivity mainly in the cytoplasm and less commonly in the nucleus. One case had strong reactivity in the cell membrane, sometimes with concurrent paranuclear staining. Immunoreactivity did not correlate with location (epidermal, dermal, and adnexal) of tumor cells. Disease outcome did not vary between PGP 9.5-positive and negative tumors. Although PGP 9.5 immunoreactivity in MF did not predict tumor behavior in these dogs, it has had prognostic value in certain human carcinomas. This unexpected staining of lymphocytes in mycosis fungoides with an antibody to PGP 9.5 demonstrates its presence in nonneuroendocrine tumors and precludes its use as the sole diagnostic marker in discrete cell tumors in the skin.
A comparative light and electron microscopic study was carried out on skin biopsies from 5 mycosis fungoides patients (stages II and III). In addition, certain zinc-iodide-osmium (ZIO) procedures that selectively reveal the Langerhans cells (LC) were also applied. Several morphometric analyses, such as relative frequency, cell size, nuclear:cell ratios and nuclear contour indexes, were performed. Two main cell lineages were found in the dermal infiltrate: the lymphoid cells, represented by lymphoblastlike cells (LB), lymphocytes (L), and mycosis fungoides cells (MFC), and the Langerhans cells. A pattern of cellular organization of the dermal infiltrate was recognized. The LC appeared as the "organizing" cell. The morphometric study indicated that all LB, L, and MFC have the chance, at the same time, to be in contact with a LC. The application of the ZIO procedures clearly indicated a "translocation" of the LC, since they were scarce or absent from the epidermis and numerous in the dermis. Evidence is presented indicating that differentiation of LB into L and malignant transformation of L into MFC are processes occurring predominantly in the dermis. It is postulated that in the chronic stages of mycosis fungoides, L-LC contacts occur mainly in the dermis and not in the epidermis and that the development of malignant clones may occur in the dermis.
The purpose of this study was to investigate the biologic activity of DAB486IL-2 when administered three times daily, in terms of toxicity, pharmacokinetics, and anti-tumor effects in patients with IL-2R expressing hematologic malignancies, especially mycosis fungoides. 20 patients were enrolled in this dose escalation phase I trial. Patient cohorts were treated at levels of 0.03 mg/kg, 0.05 mg/kg, 0.07 mg/kg and 0.09 mg/kg every 8 hours for a total of 12 doses, every 21 days as toxicity and response warranted. Eight patients experienced transient fevers associated with DAB486IL-2 administration. One patient experienced grade 3 hypotension, and a second developed fluid retention manifested as weight gain/pedal edema. Dose limiting toxicity consisted of one episode of transient grade 4 hepatic transaminase elevation, and 8 episodes of transient asymptomatic grade 3 hepatic transaminase elevation. At the maximum tolerated dose DAB486IL-2 exhibited biphasic clearance kinetics; transient receptor saturation may be one mechanism for this observation. Initial serum concentration and apparent steady state level (plateau) directly correlated with the dose administered, but no difference in area under the concentration curve with greater dose was observed. Biologic activity was noted in patients with mycosis fungoides with skin lesion clearing and relief of pruritus. One patient with mycosis fungoides, and one patient with a relapsed intermediate grade non-Hodgkin's lymphoma achieved partial responses. The novel mechanism of action, toxicity profile, and evidence of biologic activity in refractory cancer patients, support development of more active constructs of this agent; such trials are underway.
STUDY OBJECTIVE: To determine the optimal staging evaluation at the time of initial diagnosis of mycosis fungoides or the Sézary syndrome. DESIGN: Retrospective review of a uniformly staged inception cohort. SETTING: Single-institution tertiary care center. PATIENTS: 152 consecutive patients who had mycosis fungoides with or without the Sézary syndrome within 6 months of the initial definitive diagnosis. INTERVENTION: A detailed staging evaluation including physical examination, routine laboratory studies, chest roentgenogram, lymphangiogram, peripheral blood smear, lymph node biopsy, bone marrow aspirate or biopsy, and liver biopsy in selected patients. MEASUREMENTS AND MAIN RESULTS: Univariate adverse prognostic features at initial diagnosis in patients with mycosis fungoides included (P less than 0.01) one or more cutaneous tumors or generalized erythroderma, adenopathy, blood smear involvement with Sézary cells, lymph node effacement, eosinophilia, and visceral involvement. Important, independent prognostic factors in a multivariate analysis are the presence of visceral disease and type of skin involvement. CONCLUSIONS: A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, greater than 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).
A patient with mycosis fungoides developed meningeal disease while his skin disease was in remission with systemic chemotherapy. His central nervous system involvement with mycosis fungoides was controlled with intrathecal methotrexate for 7 months. The proliferating cells recovered from the spinal fluid showed similarities to the Sézary cell by light and electron microscopy. Surface receptor sutudies suggested that these cells were lymphoid cells of thymic derivation. Although mycosis fungoides has been shown to spread to the central nervous system in autopsied cases, reports of clinical neurologic disease are rare, and in only one earlier report have malignant cells have been found in the spinal fluid. Thus, as in other lymphoproliferative disorders, prompt consideration of meningeal involvement in a patient exhibiting neurologic symptoms while in peripheral remission may allow earlier treatment of this complication.
A patient with tumor-stage mycosis fungoides and lymph node involvement had sea-blue (ceroid-containing) histiocytes in her bone marrow. Leukopenia, eosinophilia, and a mild type II beta-hyperlipoproteinemia were also features. Sea-blue histiocytes were demonstrated in the skin after the mycosis fungoides infiltrate had cleared with treatment. There is a possible interrelationship between the sea-blue histiocytes, the abnormal mycosis cells in the skin, and lipid metabolism.
Mycosis fungoides (MF) in deep-seated lymph nodes, spleen or liver appears to be associated with a lack of response of the disease to topical external therapy. Fourteen patients with mycosis fungoides were clinically staged and had a staging laparotomy. Mycosis fungoides was found in the lymph nodes of 3 of these patients, in the spleen in 4 and in the liver in 2 cases. Patients with adenopathy, or with splenomegaly or abnormal spleen scans, should be considered for surgical staging to determine the extent of extracutaneous disease. The documentation of extracutaneous MF becomes necessary in the development of new therapeutic approaches.
PURPOSE: The specific goal of this retrospective study is to evaluate the role of total skin electron beam therapy (TSEBT) in the treatment of Mycosis Fungoides (MF) and to assess the most significant prognostic factors in univariate and multivariate analyses. MATERIAL AND METHODS: From January 1985 to December 1999, 92 TSEBT (Stanford Standing technique) were performed on a total of 86 patients (63 with Mycosis Fungoides, 6 with Sezary Syndrome and 17 with Cutaneous Lymphomas). This study considers only the Mycosis Fungoides group, which consisted of 60 cases evaluable for response, survival and toxicity. The distribution of patients by stage (MFCG Staging Classification, 1991) was 21, 5, 12, 22 for stages I, II, III and IV, respectively. RESULTS: The overall response rate was 96.6% (58/60) with complete response (CR) in 50/60 patients (83.3%) and partial response (PR) in 8 cases (13.3%). The five-year and ten-year actuarial overall survival (OS) was 50% and 45%, respectively. Local control, intended as control of the disease in the skin, was 35% at five years and 20% at ten years, and was correlated with skin involvement. The prognostic factors confirmed by the multivariate analysis for both overall survival and local control were: T (p<0.001) and response after TSEBT (p<0.001). The treatment was very well tolerated. CONCLUSIONS: Our study showed good results in terms of response and survival with a long follow-up time (mean value 40 months). We confirm that TSEBT yields very good results in early-stage MF; additional trials of combined modality and investigational therapies are needed to improve the outcome for advanced-stage disease.
We report a 61-year-old woman with a 1-year-history of widespread erythematous scaly patches and plaques as well as red/purplish to brownish confluent plaques. Ulcerated lesions with a purulent, hemorrhagic exudate and sharp elevated borders were located on the lower extremities. Diagnosis of granulomatous mycosis fungoides was supported by histopathologic findings showing an inflammatory reaction with epithelioid and large giant cells associated with features characteristic of mycosis fungoides. Immunohistochemical studies showed a T-helper phenotype of neoplastic cells (CD3+, CD4+, CD45RO+) with expression of the cytotoxic protein TIA-1. Molecular analysis of TCRgamma gene demonstrated a monoclonal rearrangement in the lesional skin. After failure of conventional therapies, 6 cycles of gemcitabine treatment produced partial remission of cutaneous lesions and stable disease throughout a 12-month follow-up period, suggesting that gemcitabine is a promising chemotherapeutic agent for refractory mycosis fungoides.
Mycosis fungoides is a cutaneous T-cell lymphoma. Its presence, which denotes an altered immune system, may make treatment of otherwise simple cutaneous infections difficult. In the case presented here, a patient with widespread tinea corporis poorly responsive to several oral antifungals was noted as having a background poikilodermatous slightly scaly eruption. Results of a skin biopsy during therapy with oral antifungal medications showed evidence of tinea corporis; atrophy of the epidermis; a superficial, perivascular, and interstitial lymphocytic infiltrate with numerous atypical lymphocytes; and exocytosis of atypical lymphocytes into the epidermis with formation of microabscesses-findings consistent with the diagnosis of mycosis fungoides. Treatment with PUVA (oral psoralen and UVA light) and oral itraconazole led to long-term remission of the mycosis fungoides and the associated tinea corporis. Immune suppression may have contributed to the recalcitrant nature of our patient's dermatophyte infection. Underlying cutaneous, systemic, or iatrogenic disorders associated with immune dysfunction should be considered in patients with recalcitrant dermatophyte infections.
Two cases of an inflammatory vitiligo-like condition that simulated mycosis fungoides are reported. Both patients presented acquired hypopigmented macules sharply limited by an erythematous and papular border. The clinical aspect was suggestive of inflammatory vitiligo. Mycosis fungoides was suspected on skin specimens showing a dense band-like lymphocytic infiltrate with discrete nuclear atypias and marked exocytosis. This infiltrate was made of CD3 positive lymphocytes. CD8 positive lymphocytes were numerous in one case, few in the other. There was a loss of melanocytes in the lesional skin and absence of dominant T-cell clones in both cases. No repigmentation was observed after PUVA or local chemotherapy. The authors emphasized that erythematous and papular borders surrounding hypopigmented macules, CD8 positive lymphocytic infiltrate, absence of T-cell clonal rearrangement are helpful to rule out mycosis fungoides.
BACKGROUND: Lipoprotein-T-cell interactions are being reported with increasing frequency, and there is evidence that lipoproteins play a role in immunoregulation. We describe a patient with mycosis fungoides and hyperlipidemia who developed xanthomatization in one preexisting plaque. The case is unique in that some of the lipidized cells were atypical T cells. In previously reported cases of mycosis fungoides with dystrophic xanthomatosis, the lipid-containing cells have been identified only as histiocytes. OBSERVATIONS: Immunopathologic features, electron microscopy, and lipid stains of the xanthomatized plaque demonstrated that some of the lipid-laden cells were atypical T cells. CONCLUSIONS: In mycosis fungoides, malignant T cells may be intimately involved in processing of tissue lipids. We suggest that low-density lipoprotein receptors on activated T cells facilitated the cytoplasmic lipidization in this case.
Mycosis fungoides is a low-grade lymphoproliferative disorder caused by CD4+ lymphocytes. It is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumors, or erythroderma. Diagnosis of early mycosis fungoides can be difficult due to the nonspecific nature of cutaneous and histologic findings. However, recent advances in the application of histologic criteria, coupled with molecular biology tools such as immunophenotyping and polymerase chain reaction, have improved diagnostic accuracy. Independent prognostic factors include the extent and nature of skin involvement, the presence of extracutaneous disease, blood involvement, age > or = 60 years, and lactate dehydrogenase elevation. Accordingly, patients with limited patches and/or plaques (stage IA or IIA) experience long-term survival comparable to that of matched controls. The median survival is 11 years for patients with extensive patch/plaque (stage IB or IIA), 3.2 years for those with cutaneous tumors (stage IIB), 4.6 years for those with erythroderma (stage III), 1.2 years for those with pathologic nodal involvement (stage IVA), and 0.9 years for those with visceral disease (stage IVB). Over time, mycosis fungoides may progress to Sézary syndrome or transform to large-cell histology.
Follicular mycosis fungoides, a subtype of cutaneous T-cell lymphoma, is often difficult to treat. We present a case of a female with follicular mycosis fungoides who showed an excellent response to low-dose (150 mg/m2) oral bexarotene (Targretin). To our knowledge, this is the first reported case of follicular mycosis fungoides demonstrating a response to bexarotene.
A 62-year-old man with a 13-year history of mycosis fungoides presented with a 2-month history of alopecia of the scalp. The mycosis fungoides had remained untreated for the previous 3.5 years. A biopsy specimen from the scalp showed follicular mucinosis in association with mycosis fungoides.