1/21. Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease.A 58-year-old man had long-standing lesions of presumed large plaque parapsoriasis. Following treatment for nodal Hodgkin's disease (HD), these became more infiltrated, with a diagnosis of mycosis fungoides (MF). A few months later, nodules appeared on the right leg, which was lymphoedematous after inguinal irradiation for HD. Histopathological examination showed CD3 , CD30-, CD15- large pleomorphic lymphocytes, leading to the diagnosis of transformed MF. The cutaneous lesions were successfully treated with topical nitrogen mustard and interferon alfa-2b then methotrexate, but his general health worsened with depression and malaise, without specific neurological symptoms or extracutaneous spreading of the lymphoma. Cerebral computed tomographic scan revealed a cerebellar subdural collection, arachnoid cyst and quadriventricular hydrocephaly, initially considered to be non-specific. After a few weeks, clinical symptoms of intracranial hypertension appeared, and a cerebrospinal fluid (CSF) examination revealed meningeal involvement by the lymphoma. These cells were CD3-negative and the diagnosis was confirmed by polymerase chain reaction (PCR) study, which revealed an identical clonal rearrangement of the T-cell receptor gamma gene between cutaneous biopsies and the CSF. Repeated intrathecal injections of methotrexate and cranial irradiation were performed and the patient was still alive after 13 months. This case illustrates the possible meningeal involvement of MF that may be preceded by atypical and mild neurological or psychiatric symptoms, which may be dissociated from the evolution of the cutaneous lesions. Moreover, PCR study may be useful for both diagnosis and monitoring.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
2/21. Hypopigmented mycosis fungoides: a report of 7 cases and review of the literature.Over the last 2 decades, hypopigmented macules have been reported with increasing frequency as an initial presentation of mycosis fungoides (MF). We retrospectively reviewed 7 patients with hypopigmented MF. The mean age was 35 years at disease onset, with a mean of 5.5 years' duration of illness before presentation. All of our patients were Fitzpatrick skin type IV or V, and most reported pruritus. Histologic findings in all cases were consistent with MF. Treatment with topical nitrogen mustard produced repigmentation in 4 of 6 patients.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
3/21. Optimizing bexarotene therapy for cutaneous T-cell lymphoma.BACKGROUND: Bexarotene (Targretin oral capsules), the first RXR-selective retinoid "rexinoid" approved for all stages of cutaneous T-cell lymphoma (CTCL), had a response rate (RR) of 45% at the optimal dose of 300 mg/m(2) per day in 2 multicenter trials. With hypertriglyceridemia reported at 79%, bexarotene is often administered with lipid-lowering agents (LLAs). Statins (inhibitors of 3-hydroxy-3-methylglutaryl coenzyme a reductase) may modulate class II major histocompatibility class expression and T-cell responses. OBJECTIVE: We attempted to optimize the clinical response to bexarotene by controlling dose-limiting hypertriglyceridemia and combining bexarotene with other active agents. methods: We prospectively evaluated 70 patients with CTCL at M. D. Anderson Cancer Center who were treated with oral bexarotene as monotherapy or in combination with other active agents. RESULTS: Fifty-four patients receiving bexarotene monotherapy achieved an overall RR of 48%. Thirteen had stage IA-IIA disease (RR = 53%, 1 complete response [CR]); 41 had stage IIB-IVB disease (RR = 46%, 2 CRs). Forty-two (77%) of these also required one or more LLAs: atorvastatin (n = 29, RR 43%), atorvastatin plus fenofibrate (n = 10, RR 90%), or gemfibrozil (n = 3, RR 33%). gemfibrozil was discontinued because it increased bexarotene and triglyceride levels. patients taking 2 LLAs had a significantly higher RR of 90% during monotherapy than those taking one or no LLAs (P <.0001). Forty of 54 patients (74%) received thyroid hormone replacement to normalize thyroxine levels. Four patients receiving monotherapy have complete CRs of >3 years' duration and received maintenance dosing. Sixteen patients with advanced disease treated with bexarotene (225-750 mg/d) in combination with other CTCL therapies achieved an overall RR of 69% (11/16) with concomitant statin therapy. Bexarotene was safely combined with psoralen ultraviolet A (PUVA) plus interferon alfa (IFN-alpha) (n = 2, RR = 50%), with extracorporeal photopheresis (ECP) (n = 8, RR = 75%, 1 CR), with ECP/IFN-alpha (n = 4, RR =50%), with ECP/IFN-alpha/PUVA (n = 1, RR = 100%), and with IFN-alpha/PUVA/topical nitrogen mustard (n = 1, RR = 100%). Two patients receiving IFN-alpha had slight leukopenia, but rhabdomyolysis associated with multiple LLAs did not occur. CONCLUSION: This single-center study supports the safety and efficacy of bexarotene as both a monotherapy and a combination therapy for CTCL. Long durable CRs may be achieved with oral monotherapy. Use of statins with bexarotene may also increase RRs by permitting higher doses to be administered without interruption, by modulating the immune response, or both. When bexarotene is combined with other active CTCL therapies, higher RRs were achieved in patients with advanced disease, without unacceptable side effects.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
4/21. Contact urticaria and anaphylactoid reaction induced by topical application of nitrogen mustard.A 36-year-old woman and a 16-year-old boy, both suffering from mycosis fungoides, developed urticaria and an anaphylactoid reaction after topical whole body application of nitrogen mustard. Prick tests with nitrogen mustard solution produced a weal and flare response. Both patients had previously been treated intermittently with total body application of nitrogen mustard for 2 1/2 years and 1 year respectively without complications.- - - - - - - - - - ranking = 7keywords = mustard (Clic here for more details about this article) |
5/21. Long-term control of mycosis fungoides of the hands with topical bexarotene.BACKGROUND: Limited Stage IA mycosis fungoides (MF) is often treated with topical steroids, which can cause atrophy, or with nitrogen mustard, which imposes several limitations on the patient's lifestyle. Topical bexarotene is a novel synthetic rexinoid with few side-effects that has shown efficacy for treatment of mycosis fungoides skin lesions in recent Phase II-III clinical trials. The Phase I-II trial involving 67 stage IA-IIA MF patients demonstrated complete response (CR) in 21% and partial response (PR) in 42% of the patients. The median time to response was approximately 20 weeks. In the phase III trial of refractory stage IA, IB and IIA MF, the patients demonstrated a 44% response rate (8% CR). patients with no prior therapy for mycosis fungoides responded at a higher rate (75%) than those with prior topical therapies. methods: Case report of a patient with MF limited to the hands treated with topical bexarotene 0.1% gel in a open label phase II clinical trial. RESULTS: Partial response occurred after 2 weeks of topical bexarotene therapy and the lesions were well controlled for 5 years using bexarotene monotherapy, with only occasional mild local irritation. CONCLUSIONS: Topical bexarotene is effective as long-term treatment monotherapy for limited MF lesions. To our knowledge this is the longest use of the drug by any individual.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
6/21. mycosis fungoides associated with malignant melanoma and dysplastic nevus syndrome.BACKGROUND: The increased risk of second malignancies, including nonmelanoma skin cancers, in cutaneous T-cell lymphoma (CTCL) patients has been well documented. However, relatively few studies of malignant melanoma in CTCL patients have been reported. methods: A database of 250 CTCL patients registered over a 3-year period was searched to identify patients with diagnoses of both mycosis fungoides (MF) and malignant melanoma. RESULTS: We identified six cases of MF associated with malignant melanoma and one associated with dysplastic nevus syndrome, which is a marker of increased risk of melanoma. In four patients, melanoma was diagnosed along with or before MF. In the remaining two patients, MF was diagnosed prior to melanoma, although dysplastic nevi were noted at the time MF was diagnosed. These two patients received treatment for their MF (one with topical nitrogen mustard and another with radiation therapy and nitrogen mustard) prior to the histologic confirmation of melanoma. Six patients had early stages of MF (IA or IB), while one patient presented with simultaneous erythrodermic mycosis fungoides involving the lymph nodes as well as melanoma metastatic to the lymph nodes from an unknown primary. CONCLUSION: There is an elevated prevalence of malignant melanoma in MF patients compared to the general US population (P < 0.00001) with a relative risk of 15.3 for observing malignant melanoma in MF patients (95% confidence interval 7.0-33.8). Possible pathologic links between the two diagnoses include effects of mycosis fungoides therapies, immunosuppression secondary to mycosis fungoides, and genetic alterations in the p16 tumor suppressor protein.- - - - - - - - - - ranking = 2keywords = mustard (Clic here for more details about this article) |
7/21. Milia in regressing plaques of mycosis fungoides: provoked by topical nitrogen mustard or not?OBJECTIVE: To report three cases of mycosis fungoides with milia formation in the regressing lesions. patients AND SETTING: dermatology clinic of a university hospital (referral center). Three patients with mycosis fungoides with body surface involvement of 10% in one case (stage IIb) and exceeding 30% in two cases (stages IIb and III). All patients were treated with photochemotherapy and topical nitrogen mustard ointment in a concentration of 0.01%. After approximately 3 months multiple milia erupted on regressing plaques. RESULTS: The presence of milia was evident and was confirmed by histopathology. Regression of mycosis fungoides was noted in these plaques both clinically and in comparison with the pretreatment histologic appearance. Two of the patients showed a histological picture of follicular mucinosis. CONCLUSIONS: We do not know the significance of milia in mycosis fungoides (MF). However, we suggest that follicular rupture or a degenerative process might result in milia formation.- - - - - - - - - - ranking = 5keywords = mustard (Clic here for more details about this article) |
8/21. Efficacy of imiquimod in solitary plaques of mycosis fungoides.mycosis fungoides is the most common type of cutaneous T-cell lymphoma. The extent and type of skin involvement (T classification) and presence of lymph node or visceral involvement are important predictors in selecting the type of treatment. skin directed treatment represents the most appropriate therapy for early stage disease. Current topical treatment including potent topical steroids and nitrogen mustard are associated with adverse effects such as cutaneous atrophy and contact dermatitis. In the present study, we tested the efficacy of imiquimod in mycosis fungoides plaques.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
9/21. Clinical experience: practical management of five patients with cutaneous T-cell lymphoma (CTCL)-related symptoms.There are now a wide variety of therapeutic options for managing patients with cutaneous T-cell lymphoma-related symptoms. These include skin-directed therapies such as psoralen with UVA irradiation (PUVA), topical chemotherapies such as mechlorethamine (nitrogen mustard) and carmustine (BCNU), electron beam radiation, and systemic therapies such as chemotherapy, photopheresis, and interferons. Although treatment algorithms exist for patients with early stage disease, often treatments are individualized, based on patient specific factors, cost, and accessibility of referral centers for specialized therapies. This article provides details of five real-life case studies to illustrate how cutaneous T-cell lymphoma management can be tailored to the needs of each individual patient.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
10/21. Acquired epidermal cysts and mycosis fungoides. A possible pitfall in clinical staging.A 60-year-old man was followed for 5 years for plaque stage mycosis fungoides (MF), follicular mucinosis (FM), and multiple recurrent acquired epidermal cysts on the face, scalp, right axilla, and inguinoscrotal areas. Topical nitrogen mustard and steroids, as well as systemic retinoids controlled the infiltrated lesions of MF, but had no effects on the cysts, which had to be removed surgically. Such cases have to be distinguished from MF in a tumor stage and may suggest that the recurrent cysts represent a paraneoplastic syndrome.- - - - - - - - - - ranking = 1keywords = mustard (Clic here for more details about this article) |
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