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The possibility of the transformation of mycosis fungoides into a lymphoma of another histologic type is problematic. Convincingly illustrated examples are not to be found in the literature. We report on a well-documented case of a man who developed nodular sclerosing Hodgkin's disease 15 years after first presenting with mycosis fungoides.
Three patients with mycosis fungoides who developed pulmonary abnormalities are described. These abnormalities are correlated with the findings at open lung biopsy and at autopsy. The roentgenographic changes consisted of bilateral, nodular pulmonary infiltrates in two patients, and interstitial infiltrate and plate-like atelectasis with hilar adenopathy in a third patient. Examination of lung biopsies and autopsy tissue revealed interstitial and/or intra-alveolar infiltrates composed of atypical lymphoid cells similar to those seem in the skin biopsies. The short survival after detection of an abnormal chest film suggests that dissemination of mycosis fungoides to the lung heralds a poor prognosis.
During the past eight years, 137 patients with mycosis fungoides were given total skin electron beam therapy (TSEB) using 2.5 to 4 MeV electrons. A single treatment delivers 400 rads to the entire skin surface, and patients are treated once a week for six to eight consecutive weeks. All have been followed for a median of 18 months (range 6-103 months). Although approximately 80 to 90% of the total population were in the advanced stages of the disease and had been previously treated elsewhere, cutaneous lesions and associated symptoms were effectively controlled. The remission lasted 6 to 103 months (median 19 months) after beginning the therapy for 109 mycosis fungoides patients, who were at risk for 12-103 months (median 24 months). Erythroderma and circulating Sezary cells adversely affected the duration of remission and indicate extracutaneous dissemination. Lymphocyte responsiveness to various mitogens and antigens correlated well with duration of the remission that was obtained from a course of TSEB.
For 26 patients with mycosis fungoides (MF), the type and extent of the skin lesions, the percentage of cerebriform mononuclear cells (CMC), and T and B lymphocytes in the peripheral blood and lymph nodes were correlated with MF involvement of regional lymph nodes, the clinical course, and response to therapy. Skin tumors and an involvement of more than 25% of the skin correlated well with lymph node involvement. Normal percentages (2--18%) of CMC in the peripheral blood were found for MF patients without lymph node involvement when compared with those found for patients with benign erythroderma and healthy donors. Elevated circulating CMC percentages (greater than 20%) were observed in 9 of 11 MF patients with lymph node involvement. In the lymph node cell suspensions from nine of ten MF patients with lymph node involvement, increased CMC values were found (greater than 15%), whereas two of three MF patients without lymph node involvement showed percentages comparable (4% and 7%, respectively) with those of the control lymph nodes. In the peripheral blood of patients with MF, decreased percentages of T cells (less than or equal to 55%) were found predominantly for patients with lymph node involvement, whereas normal percentages were noted for most of the patients without lymph node involvement. No consistent differences in the percentage of T and B cells in the lymph node cell suspensions were found between MF patients with and without lymph node involvement. Patients with lymph node involvement showed in general a partial response to therapy with an unfavorable clinical course in contrast to patients without lymph node involvement. Increased percentages of CMC (greater than 20%) and decreased percentages of T cells (less than or equal to 55%) in the peripheral blood, the presence of skin tumors, and involvement of more than 25% of the skin are prognostically unfavorable signs for patients with mycosis fungoides.
The antitumor effect of thymidine has been demonstrated in patients with leukemia and lymphoma. This report summarizes the treatment of three patients with mycosis fungoides, a chronic T-cell lymphoma. Four courses of thymidine (75 g/m2/day) were administered by continuous infusion for 4-7 days. Steady-state serum thymidine levels were in the range of 1-3 mM. Associated toxicities were minimal and consisted of milk headache and anorexia. Myelosuppression was manifested by transient declines in the peripheral leukocyte count. One patient had extensive clearing of diffuse erythematous plaques on the trunk and extremities that persisted for over one month. A second patient had partial clearing of plaques that persisted for two weeks following therapy and a third patient had a minimal response with 25% reduction in lymphadenopathy and noduloulcerative lesions. These responses indicate the effectiveness of thymidine as a single agent in the treatment of mycosis fungoides.
Three patients receiving diphenylhydantoin (DPH) were seen with a reversible process suggesting mycosis fungoides. Clinical and laboratory manifestations included generalized pruritic exfoliative erythroderma, eosinophilia, lymphadenopathy, hepatosplenomegaly, circulating Sézary cells, epidermal Pautrier's microabscesses on skin biopsy, and moderate liver dysfunction. Studies of the distribution and function of the various lymphocyte subpopulations from these patients showed: (1) an increase in the relative and absolute number of T lymphocytes (85--92%); (2) significant stimulation of lymphocyte-blastic transformation by DPH and low response to pokeweed mitogen stimulation; (3) the impaired ability of T gamma lymphocytes to suppress B-cell differentiation and immunoglobulin production. With only one exception, 15 symptom-free patients on DPH showed none of these abnormalities. The clinical manifestations and immunologic abnormalities of patients with this pseudo mycosis fungoides syndrome remitted three to four weeks after DPH administration was discontinued. The proliferation of T lymphocytes and the inhibition of the function of T gamma-suppressor lymphocytes noted in these patients may be significant to the development of other types of pseudolymphoma and to that of true lymphoma.
A 58-year-old man with mycosis fungoides developed headache, back pain, and dorsal column signs. A diagnosis of central nervous system (CNS) involvement by mycosis fungoides was made on examination of his spinal fluid. In this patient, cranial and local radiotherapy and aggressive treatment with intrathecal methotrexate cleared the leukemic cells from his CNS, and maintenance therapy with intrathecal methotrexate has resulted in an 18-month remission.
Hodgkin's disease and mycosis fungoides are only rarely reported to occur in the same individual. The authors report a patient who initially presented with Hodgkin's disease but later died with massive visceral mycosis fungoides. This sequence of events is unique in the world's reported lymphoma experience.
Mycosis fungoides is a neoplastic disease with extranodal dissemination most frequently to the lung. The roentgenographic and clinical manifestations in these patients may simulate pneumonia and delay appropriate treatment. Disseminated disease is thought to occur in the setting of involvement of the peripheral blood with the neoplastic cells. A case of rapid pulmonary involvement with mycosis fungoides which simulated pneumonia is presented in which there was no peripheral blood manifestation of the disease.
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.
Peripheral blood from 16 patients with mycosis fungoides and two patients with Sézary syndrome was examined for T, B, and third-population (K) cells, using a battery of surface markers. T lymphocytes as determined by spontaneous rosette formation with sheep erythrocytes and third-population cells as determined by the Ripley rosette test were present in normal proportions. Surprisingly, B lymphocytes, as determined by surface immunoglobulin and receptors for mouse erythrocytes, were either lacking or were present in low proportions in some patients. Normal proportions were present in others. Repeat studies of two of three patients lacking B lymphocytes, following treatment, revealed normal or low proportions of B cells. Two patients with mycosis fungoides had increased proportions of "null" cells. Study of lymphoid cell subpopulations before and after leukopheresis in a single patient demonstrated a decrease in T cell proportions associated with a concomitant increase in the proportions of "null" cells following this therapy.
Mycosis fungoides is a malignant, cutaneous lymphoma of T-helper (TH or CD4+) cells. At presentation, the disease is usually limited to the skin, with lesions that resemble eczema or psoriasis. Neurologic involvement is uncommon. This case demonstrates the conventional MRI and dynamic contrast enhanced perfusion MRI findings in intracerebral mycosis fungoides. T1-weighted spin echo imaging demonstrated a lesion with slightly decreased signal within the body of the corpus callosum. The lesion was isointense with grey matter on axial T2-weighted imaging. Following administration of contrast, there was patchy heterogeneous enhancement. Multiple relative cerebral blood volume (rCBV) measurements were made and the minimum rCBV was 0.30 with the maximum rCBV being 1.61. The mean rCBV was 0.81 +/- 0.49 (average of 10 measurements and standard deviation).
Mycosis fungoides (MF) is a lymphoma that appears to have a unique relationship with the skin. In recent years our knowledge about this disease has increased considerably, in particular regarding the nature of the malignant cells and their functional characteristics. Furthermore, there is mounting evidence suggesting a viral etiology. Since its first description by Alibert in the early part of the past century, MF has been the subject of much controversy in regard to fundamental issues such as clinical and histological criteria for the establishment of the diagnosis [1] as well as its relationship to other lymphomas and skin diseases. Some of this controversy remains today. Mycosis fungoides is a relatively uncommon disease, the incidence being in the order of 1-2 million per year [1,2]. As is the case with most neoplastic diseases, the incidence increases with age; however, relatively speaking MF is not exceptional among individuals in their twenties or thirties. As for sex distribution there is a slight preponderance of men [3-5].
Visceral involvement is a relatively common but poorly appreciated occurrence in patients with mycosis fungoides. The lungs are the most common site of visceral involvement. Here we present two cases of mycosis fungoides with pulmonary parenchymal involvement, one of them proven by biopsy. Both patients were treated with whole-lung radiation therapy, with complete disappearance of the lesions and improvement of respiratory symptoms. There was no significant radiation-induced pulmonary toxicity. One patient continues to be alive with no recurrence in the lungs 17 months after radiation therapy.
Mycosis fungoides was observed in a 71-year-old male with Mediterranean lymphoma, a B-cell malignancy. It is proposed that this association is not incidental since hypergammaglobulinaemia and even monoclonal gammopathies have repeatedly been described in cutaneous T-cell lymphomas. Mediterranean lymphoma might have resulted from (a) helper cell activities of tumor T-lymphocytes, (b) common antigenic stimuli, or (c) deranged T-B cooperation due to concomitant mycosis fungoides.
The clinical course of a patient with mycosis fungoides, which could be confirmed histologically in the premycotic stage, 2 years after the onset of the disease is reported. The disease led to death after a further 3 years, during which the infiltrative and tumorous stage had appeared. At autopsy, apart from the skin changes typical for mycosis fungoides, involvement of the pia mater, plexus chorioideus, cerebrum and cerebellum was established. This constituted the only manifestation of the disease in the internal organ systems. There were no detectable neurological defects.
Sera from 16 patients with mycosis fungoides at various stages of the disease were investigated for the presence of a leukocyte-migration inhibitory factor. The agarose gel techinque with human peripheral blood leukocytes as migrating indicator cells was used for the purpose. The migration inhibitory effect of patients sera, pooled AB-sera and sera from control persons was tested on peripheral blood leukocytes from patients and controls. Also upconcentrated sera were used. The presence of substances with migration inhibitory effect in serum from patients with mycosis fungoides could not be demonstrated. The significance of these findings is discussed.
Lymphocyte transformation tests with PHA were carried out in 26 patients with mycosis fungoides (MF) in different stages of the disease. In premycotic and infiltrative stages of MF there were no significant disturbances in the lymphocyte response to PHA. Inhibition of DNA synthesis and blastic transformation was observed only in the tumorous stage. The results are in favour of the nosological distinctiveness of mycosis fungoides within the lymphoma group.