[Hemogram and its clinical significance in patients with fungoid mycosis].
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Humans↗
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Mycosis fungoides: subcutaneous and visceral tumors, orbital involvement, and ophthalmoplegia.
A patient with advanced severe mycosis fungoides presented several unusual features, including prominent lesions of the palate and tongue and an orbital tumor with exophthalmos and ophthalmoplegia. A hitherto undescribed feature was the development of multiple, massive subcutaneous tumors unrelated to the dermis or to lymph nodes, and large tumors in the connective tissues of the buttock, flank, and retroperitoneum. The usual sites of extracutaneous dissemination of mycosis fungoides--lymph nodes, spleen, liver, lungs, and blood--were not demonstrably involved. This may be a new pattern of dissemination for this disease. Of practical importance is the immediate and complete relief of exophthalmos and ophthalmoplegia that was obtained with emergency radiotherapy.
Aged↗
Alopecia mucinosa is mycosis fungoides.
Confusion abounds regarding the terms "follicular mucinosis" and "alopecia mucinosa," not only concerning definition and essential character, but of relationships between themselves on one hand and between themselves and mycosis fungoides on the other. We address here those issues in methodical fashion, first in historical perspective by review, scrupulously and critically, of what has been said in the many articles devoted to the subject; we next tell how the terms "alopecia mucinosa" and "follicular mucinosis" came to be and how they are employed currently; we then set forth our own observations pertinent to clinical, histopathologic, and biologic aspects of the condition called, conventionally, "alopecia mucinosa," those observations based on our own findings in sections of tissue cut from 54 biopsy specimens taken from 45 patients, all of them having been signed out previously as "follicular mucinosis;" we proceed to forge clinico-pathologic correlation of lesions in 14 of those 45 patients, utilizing assessments, by examination grossly and microscopically, of attributes in the very same lesion. Last, we propose a concept, and a terminology that derives from it, that synthesizes all that is known now about "alopecia mucinosa" and "follicular mucinosis," in particular the relationship of "alopecia mucinosa" to mycosis fungoides, including "follicular," "syringotropic," and erythrodermic manifestations of it. In short, we affirm that so-called alopecia mucinosa is but one of many morphologic manifestations of mycosis fungoides.
Adolescent↗
T and B cells and cell-mediated immunity in mycosis fungoides.
T anb B cells in peripheral blood were evaluated in 13 patients with mycosis fungoides (MF) and in two patients with Sézary's syndrome. T cells were identified by their capacity to bind sheep red blood cells to their surfaces and the B cells were identified by the presence of surface immunoglobulins in immunofluorescence assay. Seven patients with MF and two with Sézary's syndrome were shown to have T-cell values below normal and elevated levels of null cells, i.e., cells with markers for neither T nor B cells. Low T-cell values and elevated Null-cell values were found predominantly in patients with advanced mycosis fungoides (stages III-IV). Furthermore, these patients had impaired cell-mediated immunity, as shown by in vivo skin testing with recall antigens and attempts at sensitization to dinitrochlorobenzene.
Adult↗
Secondary pancreatic involvement of mycosis fungoides detected by a clinically palpable mass.
A patient with a 4-year history of mycosis fungoides, who presented a pancreatic spreading of the disease, detected by a clinically palpable mass, is reported and discussed with a review of the literature. Mycosis fungoides (MF) is a T cell lymphoma primarily localized to the skin. Laparoscopic staging and autopsy studies, however, have shown that lymph node involvement or visceral spreading is common in the course of the disease [1-3]. Among visceral manifestations of MF, pancreas infiltration is found in 20-40% of autopsied cases [1, 2, 4]. Occurrence of a palpable pancreatic mass has never been described. We report a patient with a long-standing MF, and a secondary pancreatic extension detected by a palpable mass.
Aged↗
Unilesional mycosis fungoides: a distinct entity.
Four cases of solitary lesions showing the histologic features of mycosis fungoides are described. Three patients had an erythematous scaling plaque, whereas the fourth had a solitary nodule. The lesion was located on the arm in two patients, on the foot in one patient, and on the back in one patient. No preceding factors were noted, and no lymphadenopathy or other significant illness was found on examination. Histologic examination demonstrated features of a cutaneous T cell lymphoma with epidermotropism. Monoclonal markers on frozen and paraffin sections revealed a predominant T cell infiltrate. Three patients were treated with local excision. The remaining patient was treated with radiotherapy. The lesions have not recurred, and no new lesions have developed after individual follow-up of 7 months, 7 1/2 months, 13 years, and 18 years. These cases appear to be unique reports of solitary mycosis fungoides behaving in a biologically benign manner. The relationship to Woringer-Kolopp disease and lymphomatoid papulosis is discussed.
Aged↗
Expression of HECA-452 in parapsoriasis and mycosis fungoides.
We have investigated the HECA-452 expression in large plaque parapsoriasis (PP) and mycosis fungoides (MF) patients, evaluating the potential role of this biomarker in both cutaneous disorders. Skin specimens from 72 PP and 61 MF patients were selected in this study. We compared their actual histological diagnosis with their previous diagnosis and we found that all 72 PP patients had the same diagnosis as before (stable PP), while 26 out of 61 MF had a previous PP histological diagnosis (evolving PP). Our results show an increased expression of HECA-452 in MF compared to PP (p<0.01). Furthermore, evolving PP showed a significantly higher level of HECA-452 than stable PP (p<0.05). We conclude that HECA-452 expression increases during the natural history of Mycosis Fungoides. HECA-452 could be used as a biomarker for MF and predict which PP evolves to MF.
Antibodies, Monoclonal↗
Mycosis fungoides simulating acanthosis nigricans.
In this paper, an unusual papillomatous variant of the plaque stage of mycosis fungoides with clinical similarities to acanthosis nigricans is described. In contrast to the classic plaque stage of mycosis fungoides, the dermal infiltrates in this patient showed a rather monomorphous proliferation of blast-like cells that had little or no tendency to infiltrate the epidermis.
Acanthosis Nigricans↗
Leu-M1 antigen expression in advanced (tumor) stage mycosis fungoides.
The authors describe two patients with clinically and histopathologically documented advanced (tumor) stage mycosis fungoides. In each case the large, pleomorphic neoplastic cells lacked the monoclonal antibody-defined cell surface antigens commonly associated with immature and mature T-cells, i.e., T11, Leu-1, T3, T4, T6, T8, and T10, but expressed various T-cell-associated activation antigens, such as HLA-DR, Tac, and T21. Leu-M1, a monocyte-associated antigen, was not expressed by the small, cerebriform neoplastic cells in the plaque stage lesions of either patient. However, Leu-M1 was expressed by most of the large, pleomorphic neoplastic cells present in the nodular lesions of both patients. The pattern of Leu-M1 antigen expression was identical to that previously reported in the Reed-Sternberg cells of Hodgkin's disease. Identification of these two patients suggests using caution in the interpretation of the results of immunophenotypic analysis of cutaneous lymphoid neoplasms and that Leu-M1 should not be used as a diagnostic indicator of Hodgkin's disease or a histiocytic-derived neoplasm. These studies also suggest that Leu-M1 may be preferentially expressed on a subpopulation of activated, rapidly proliferating, and/or dedifferentiated neoplastic T-cells that proliferate in the advanced (tumor) stages of mycosis fungoides.
Aged↗
Observations on the effect of chimeric anti-CD4 monoclonal antibody in patients with mycosis fungoides.
Chimeric (murine/human) anti-CD4 monoclonal antibody was infused into seven patients with mycosis fungoides. Successive patients received doses of 10, 20, 40, and 80 mg of antibody twice a week for 3 consecutive weeks. All patients had some clinical improvement, but responses were of relatively short duration. Serum levels of chimeric antibody varied as a function of dose. At the 80-mg dose level, antibody was readily observed in biopsied skin lesions. Although there was coating by antibody of most CD4 positive cells in the blood, there was no significant depletion of CD4 positive cells. Low-level antibody responses against the mouse Ig variable region and human Ig allotypic constant region determinants were observed in several patients, but none were of clinical significance. All but two patients made primary antibody and T-cell proliferative responses to a simultaneously administered foreign protein test antigen. However, there was marked suppression of the mixed lymphocyte reaction. We conclude that at the dose levels studied, a chimeric anti-CD4 monoclonal antibody (1) had some clinical efficacy against mycosis fungoides; (2) was well tolerated; (3) had a low level of immunogenicity; (4) had immediate immunosuppressive effects; and (5) did not induce tolerance to a co-injected antigen.
Adult↗
[Age, duration of disease and survival period of patients with involvement of the nervous system by malignant lymphomas. A statistical comparison of mycosis fungoides with lymphogranulomatosis (author's transl)].
A statistical comparison dealing with age, duration of disease and survival period of patients with involvement of the nervous system by malignant lymphomas revealed the following data: 1. With mycosis fungoides, involvement of the nervous system occurs later in life than with lymphogranulomatosis; this difference is statistically highly significant (P less than 0.001). 2. There is no statistical difference (P greater than 0.05) in the duration of either type of malignant lymphoma before the nervous system becomes involved. 3. It is of high statistical significance (P less than 0.001) that, once the nervous system has become involved, the survival period is lower with mycosis fungoides than with lymphogranulomatosis. It can be expected that involvement of the nervous system by mycosis fungoides leads with 79% probability (confidential limits 99% = 47.29--96.22%) to death within 6 months.
Age Factors↗
Treatment of parapsoriasis and mycosis fungoides: the role of psoralen and long-wave ultraviolet light A (PUVA).
Psoralen and long-wave ultraviolet light A (PUVA) was used to treat 12 patients with parapsoriasis and 19 patients with mycosis fungoides. Excellent results were obtained in all 12 patients with parapsoriasis: 8 patients had complete clearing, 3 had more than 80% improvement, and 1 had considerable improvement. The response to PUVA in patients with mycosis fungoides varied according to the stage of the disease. Of 10 patients with early disease (stage I or II), 7 had complete clearing and the 3 others had more than 80% improvement. The nine patients with more advanced disease generally did not respond well to PUVA, with the exception of one patient with stage IV disease and one with stage V disease. PUVA may be the treatment of choice for parapsoriasis and is highly effective in the early stages of mycosis fungoides.
Female↗
Mycosis fungoides presenting as areas of hypopigmentation: a report of three cases.
In three patients with histologically documented mycosis fungoides, all or most of the lesions were hypopigmented. Unsuspected microscopic foci of mycosis fungoides were found in a purely macular hypopigmented area in one of these patients who had been treated with vitiligo 15 years earlier. Two of the patients were black, and one was Latin American. Repigmentation in all patients followed treatment with carmustine (BCNU) and mechlorethamine.
Adult↗
Common activated helper-T-cell origin for lymphomatoid papulosis, mycosis fungoides, and some types of Hodgkin's disease.
A hypothesis is proposed to explain the nature of the Reed-Sternberg (RS) cell and the unexpectedly common clinical associations between lymphomatoid papulosis, mycosis fungoides, and some types of Hodgkin's disease. The RS cell appears to be a lymphoblast arising from activated helper T cells. In lymphomatoid papulosis, a cutaneous eruption with histological features of Hodgkin's disease, there is a spectrum of activated helper T cells, including cerebriform cells and large transformed RS-like cells. Clonal expansion of cerebriform cells in lymphomatoid papulosis leads to mycosis fungoides. Similar expansion of large transformed cells results in Hodgkin's disease. Progressive transformation of T4-positive cerebriform cells to Ki-1-positive RS cells accounts for rare cases of coexistent mycosis fungoides and Hodgkin's disease. Confirmation of this hypothesis should focus attention on the family of human T-cell leukaemia/lymphoma viruses as possible aetiological agents in these helper-T-cell disorders.
Antigens, Neoplasm↗
The changing survival of patients with mycosis fungoides: a population-based assessment of trends in the United States.
BACKGROUND: The authors sought to evaluate trends in survival among a population-based group of patients with mycosis fungoides, in response to informal evidence of improved prognosis and concerns about the influence of detection bias on incidence data. METHODS: Data used in this study were drawn from the Surveillance, Epidemiology, and End Results Program of the National Cancer Institute, which includes 9 population-based cancer registries. Together, these registries cover approximately 10% of the U.S. population and have tracked the survival of all mycosis fungoides patients who were registered during the years 1973-1992. Data included follow-up through 1994. RESULTS: There was a total of over 10,000 person-years of follow-up for the 1633 patients studied. Relative survival changed little after 11 years, at which point it was 66%. Advanced age and black race were associated with poorer survival. The prognoses of Asian and Hispanic patients were slightly but not significantly worse than those of whites, and there were no significant geographic differences related to prognosis. The survival of married women was superior to that of other gender and marital-status groups. Prognosis did improve substantially over the 20-year period of study. CONCLUSIONS: Mycosis fungoides is usually not a fatal disease, although the mortality risk is substantial. The authors quantified various prognostic factors and documented an improved prognosis over the 20-year period of study. The data from this study raise concern about possible detection bias in incidence data. These data are also consistent with the concept that beyond 11 years after diagnosis, this disorder has relatively little impact on the risk of death.
Aged↗
Hypopigmented mycosis fungoides.
We report the case of a 25-year-old Jamaican woman with hypopigmented mycosis fungoides. She first developed a hypopigmented patch on her arm at the age of 11 years. Further lesions developed on the trunk and limbs over a period of 10 years. The lesions were completely impalpable. Skin biopsy showed an infiltrate of atypical lymphocytes, some with cerebriform nuclei, suggesting a diagnosis of mycosis fungoides. The lesions cleared with PUVA therapy.
Adult↗
Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients.
OBJECTIVES: To determine the disease course of Dutch patients with mycosis fungoides and to define factors related to disease progression and survival. DESIGN: A multicenter, 13-year, retrospective cohort analysis. SETTING: Eight dermatology departments collaborating in the Dutch Cutaneous Lymphoma Group. PATIENTS: Three hundred nine patients with mycosis fungoides registered between October 1985 and May 1997, including 89 patients with limited patches or plaques (stage Ia), 135 with generalized patches or plaques (stage Ib), 46 with skin tumors (stage Ic), 18 with enlarged but uninvolved lymph nodes (stage II), 18 with lymph node involvement (stage III), and 3 with visceral involvement (stage IV). MAIN OUTCOME MEASURES: Response to initial treatment, sustained complete remission, actuarial disease progression, and overall and disease-specific survival per clinical stage. RESULTS: The median follow-up was 62 months (range, 1-113 months). For the entire group, the actuarial overall and disease-specific survival was 80% and 89% at 5 years, and 57% and 75% at 10 years, respectively. The actuarial 5-year disease-specific survival of patients with stage Ia, Ib, and Ic disease was 100%, 96%, and 80%, respectively, and only 40% for patients with stage III disease. Using multivariate analysis, the presence of extracutaneous disease, the type and extent of skin involvement, the response to initial treatment, and the presence of follicular mucinosis were independently associated with higher disease progression and mortality rates. The calculated risks of disease progression at 5 and 10 years gradually increased from 4% to 10% for those with stage Ia disease, from 21% to 39% for those with stage Ib disease, and from 32% to 60% for those with stage Ic disease; for those with stage III disease, the risk remained at 70% at 5 and 10 years. The overall risk of disease progression at 5 and 10 years was 24% and 38%, respectively, for the total study group. CONCLUSION: At least within the first 10 years after diagnosis, disease progression and mycosis fungoides-related mortality occur in only a subset of patients generally presenting with advanced disease.
Adult↗
[Treatment of symptomatic mucinosis follicularis in mycosis fungoides using fast electrons].
Mucinosis follicularis is a chronic type of dermatosis involving the sebaceous glands and outer root sheaths, which can be differentiated into primary and secondary types. The latter - also called symptomatic mucinosis follicularis - causes therapeutic problems because of the underlying malignant lymphoma. Successful treatment using electron beams supported by etretinate is described in detail in a 40-year-old man with symptomatic mucinosis follicularis based on plaque-type mycosis fungoides. After 6 months free of skin symptoms, the mycosis fungoides lesions recurred in the overlapping borders of the irradiation fields. These lesions were treated again with electron beams. A relapse after 32 months was controlled by PUVA. Electron beam therapy is recomended in the plaque type of mycosis fungoides, especially if PUVA has no effect for some reason.
Adult↗