Mycosis fungoides with coccidioidomycosis

Mycosis fungoides is an indolent, epidermotropic, cutaneous T-cell lymphoma of helper/memory T cells that presents as heterogeneous, papulosquamous patches, plaques, and tumors. We present a patient with mycosis fungoides and infection with Coccidioides immitis of the skin, which has not been previously reported.     

Total skin electron beam therapy with or without adjuvant topical nitrogen mustard or nitrogen mustard alone as initial treatment of T2 and T3 mycosis fungoides

Purpose: To compare the efficacy of total skin electron beam therapy (TSEBT) with or without adjuvant topical nitrogen mustard (± HN2) with topical nitrogen mustard (HN2) alone as initial management of T2 and T3 mycosis fungoides (MF).

Methods and Materials: A retrospective analysis of 148 patients presenting to Stanford from January, 1970 through January, 1995 within 4 months of pathologic diagnosis of MF. Fifty-five patients with T2 and 27 with T3 disease received TSEBT ± HN2. Fifty-four patients with T2 and 12 with T3 disease received HN2 alone. Boosts with radiotherapy were usually administered to cutaneous tumors of patients with T3 disease.

Results: TSEBT ± HN2 yielded significantly higher complete response (CR) rates than did HN2 alone in patients with T2 and T3 disease (76% vs 39%, p = 0.03 for T2, and 44% vs 8%, p < 0.05 for T3, respectively). In T2 disease, treatment with adjuvant HN2 was associated with a longer freedom from relapse following TSEBT when compared to observation following a CR to TSEBT (p = 0.068). However, no significant differences in survival were observed for different management programs for T2 or T3 disease. In T2 disease, both TSEBT and HN2 were as effective as salvage therapy as when utilized as initial therapy. However, salvage therapy in T3 disease was rarely effective. Limited tumor involvement in T3 disease did not correlate with improved survival compared to more generalized tumorous disease. MF contributed to 27% and 68% of deaths in patients with T2 and T3 disease, respectively.

Conclusion: Because of high response rates, management of significantly symptomatic or extensive T2 MF should include TSEBT, and adjuvant HN2 should be administered after a CR to TSEBT. Patients with T2 disease who fail TSEBT or HN2 can be salvaged with the other modality. TSEBT is also an effective treatment for T3 disease. The small subset of patients with T3 disease may also be treated with HN2 and local radiotherapy to the tumors. Further investigations are necessary to improve the overall outcome for T3 mycosis fungoides.     

Treatment of advanced stage mycosis fungoides with bleomycin, doxorubicin, and methotrexate with topical nitrogen mustard (BAM-M)

Ten patients with advanced stage (TNM IIB-IVB) mycosis fungoides were treated with a combination chemotherapy program consisting of bleomycin and methotrexate weekly, doxorubicin every 3 weeks, and topical nitrogen mustard daily (BAM-M). Seven patients obtained histologically documented complete remissions ranging from 4 to 105+ months in duration. Median survival is 16.5+ months. Three patients in whom splenomegaly was detected during their staging evaluation underwent splenectomy. These three patients have had unmaintained disease-free survivals of 36+, 100+, and 105+ months. This study indicates that BAM-M is effective therapy for advanced stage mycosis fungoides and suggests that the therapeutic role of splenectomy should be evaluated further.     

Mycosis fungoides and pregnancy

Mycosis fungoides is a cutaneous T-cell lymphoma, a subgroup of non-Hodgkin's lymphomas, characterized by skin infiltration and occasionally systemic involvement. The association of pregnancy and mycosis fungoides has not been described previously. A case of mycosis fungoides, stage IVb, in a pregnant woman is reported. Prior to pregnancy, the patient received adriamycin, cyclophosphamide, vincristine prednisolone (CHOP) and bleomycin and total body irradiation. Around the concepcional period she presented a cutaneous relapse palliated with photon radiotherapy. No obstetrics complications were observed during gestation. At 39 week's gestation a cesarean section was performed and a healthy 2900 g boy was delivered. Mycosis fungoides did not worsen during pregnancy and postpartum period. In conclusion mycosis fungoides did not adversely affect pregnancy outcome and gestation did not worsen the malignancy course. This case report may be valuable in managing patients with mycosis fungoides who are currently pregnant or are contemplating pregnancy.     

Mycosis fungoides and sezary syndrome: An update

Mycosis fungoides (MF), and the associated leukemic variant Sezary Syndrome (SS), are the most common group of cutaneous T-cell lymphomas. MF/SS is a non-Hodgkin’s lymphoma of mature, skin-homing, clonal, malignant T lymphocytes that initially presents in the skin as patches, plaques, tumors, or generalized erythema (erythroderma) and can involve the lymph nodes and peripheral blood. Much progress has been made in recent years in understanding the origin of the malignant T cell in MF/SS and the pathophysiology and immunology of the disease. This recent work has made a great impact on diagnosis, prognostication, and treatment. In this review, we survey the MF/SS published literature over the past year and highlight some of the important advances.     

Mycosis fungoides involving the oral cavity

Mycosis fungoides is a malignant T‐cell lymphoproliferative disease with a predilection for cutaneous involvement. Extracutaneous disease is uncommon and oral mucosal involvement is rare. We describe a case of mycosis fungoides involving the hard palate treated with radiotherapy. The relevant literature on this topic is reviewed.     

Mycosis fungoides involving the central nervous system

The clinical records of nine patients, followed at the University of Chicago, with mycosis fungoides (MF) and neurologic deficits due to direct CNS involvement were reviewed. Lymph node and visceral involvement was present in all patients. Clinical findings included cranial nerve deficits in four patients, altered mental status in eight, gait disturbances in three, and leg weakness in one. Helpful diagnostic studies included lumbar puncture with CSF cytology, computerized tomography (CT) brain scans, brain biopsy, and myelogram. Two patients, who were treated with radiation therapy (RT), survived 14 and 40 weeks, respectively. Survival of less than 4 weeks limited treatment in the other patients. Autopsies were performed on six patients and revealed meningeal infiltration and perivascular infiltration of the brain. In addition, the cerebral parenchyma was infiltrated in one patient and a lymphomatous tumor was found in the brainstem of another. We conclude that the varied clinical and pathologic features of CNS involvement with mycosis fungoides resemble those seen in leukemias and other lymphoproliferative disorders. Better awareness of the clinical features may permit earlier diagnosis and initiation of therapy.     

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.     

Skin lesions in chronic granulocytic leukemia. Treatment of a patient with topical nitrogen mustard

A patient is described with an unusual presentation of ulcerating skin lesions in Philadelphia chromosome-positive chronic granulocytic leukaemia. Treatment with topical mustine ointment (nitrogen mustard, mechlorethamine) achieved a good palliative result.