Mycosis fungoides

Mycosis fungoides (MF) constitutes the most frequent cutaneous T-cell lymphoma. Sezary syndrome is considered by some authors to be an erythrodermic leukemic variant of MF, but is classified separately in the new WHO-EORT classification of cutaneous lymphomas. MF usually occurs in old adults with a 2:1 male to female ratio. Its prognosis is variable and strongly conditioned by the extent and type of skin involvement and presence of extracutaneous disease. Patients with stage IA-disease have an excellent prognosis with an overall long-term life expectancy that is similar to an age-, sex-, and race-matched control population. Almost all patients with stage IA MF will die from causes other than MF, with a median survival >33 years. Only 9% of these patients will progress to more extended disease. Patients with stage IB or IIA have a median survival greater than 11 years. These patients with T2 disease have a likelihood of disease progression of 24% and nearly 20% die of MF. Subgroups with stage IB or IIA have similar prognosis. Patients with cutaneous tumors or generalized erythroderma have a median survival of 3 and 4.5 years, respectively. The majority of these patients will die of MF. Extracutaneous dissemination is observed in less than 10% of patients with patch or plaque disease and in 30-40% of patients with tumors or generalized erythrodermatous involvement. Extracutaneous involvement is directly correlated to the extent of cutaneous disease. The most commonly involved organs are lung, spleen, liver, and gastrointestinal tract. Patients with extracutaneous disease at presentation involving either lymph nodes or viscera have a median survival of <1.5 years. Patients with plaque-type or erythrodermic MF may develop cutaneous tumors with large cell histology, often expressing CD30, which share a common clonal origin as observed in their preexisting MF and are associated with a less favourable outcome.     

Mycosis fungoides]

The classical characteristics of Myocsis Fungoides are discussed. 1) The clinical characteristics of the three forms: tumour from the start, erythrodermic and polymorphic forms of Alibert-Buzin. 2) Histological characteristics : dense dermal infiltrates or epidermotropic infiltration, or around a more or less malignant histiocytic reaction, there develops a host reaction, resembling a granuloma. During the course of the disease, one may observe lymphocyte depletion, whilst delayed hypersensitivity tests become negative. The various types of lymphadenopathy are analysed, the possibility of visceral lesions confirmed. However, knowledge of Sezary's syndrome makes his concept of the disease doubtful. A pathological picture similar to Mycosis Fungoides may be observed during Sezary's syndrome, with circulating giant cells. The presence of small Sezary cells, Lutzner-Flandrin variety, is common in the dermal infiltrate, and even more common in the lymph node visceral infiltrates of Mycosis Fungoides. Thus, one is led to the concept according to which the primary phenomenon is probably pathological proliferation of abnormal T lymphocytes (large and small Sezary cells), the histiocytic proliferation would then simply be a secondary reaction. This theory is then discussed.     

Atypical mycosis fungoides with cerebral involvement

Summary This report concerns a 17-year-old male patient with atypical mycosis fungoides (m.f.). Initial examination revealed generalized lymphoma and uncharacteristic livid skin efflorescence. The patient developed bone marrow involvement and meningeal leukaemia 6 months later. Diagnosis was confirmed by immunohistochemistry and electron microscopy. Aggressive chemotherapy yielded no response.     

Mycosis fungoides: A case report

Mycosis fungoides is the commonest cutaneous T cell lymphoma. Clinically it is characterized by patch, plaque, tumor nodules; erythrodermic and poikoilodermous stages which may overlap. Extracutaneous spread takes place in late stages where any organ may be involved. The majority of reported cases are in adult males. A rare case of mycosis fungoides in a 38-year-old female is described. She presented in advance stage of disease with nodules all over the body.     

Mycosis fungoides: epidemiologic observations.

An analysis of cases from a multi-hospital, pathologically verified clinical series and of deaths from US mortality statistics available at the county level for 1950--1975 (excluding 1972) was made in order to obtain information on the etiology of mycosis fungoides (MF). Despite the absence of an appropriate comparison group, the cases seemed to have a high frequency of antecedent allergies, fungal and viral skin infections, sun sensitivity, familial aggregation of lymphoma and leukemia, and employment in a manufacturing occupation (especially petrochemical, textile, metal, and machinery industries). The mortality survey revealed a predominance among males, nonwhites, and residents of the northeastern US, the latter due partly to an association between MF mortality and population size. The influence of occupational exposures was suggested by the excessive MF mortality rates in counties where petroleum, rubber, primary and fabricated metal, machinery, and printing industries were located.     

Mycosis fungoides: a therapeutical review.

In this review of current therapy for mycosis fungoides, the analytical data have been extrapolated from the literature. We have considered the various therapeutic modalities such as topical therapy, photochemotherapy, electron beam radiation therapy, systemic chemotherapy, the combined modalities, as well as the use of interferon and other alternative biological approaches. We conclude with a final section on comments and recommendations that may prove useful in the design of appropriate therapeutic strategies.     

Mycosis fungoides: electron beam therapy in England.

Since 1962, total-skin electron-beam therapy has been available in London, England, for the treatment of patients with mycosis fungoides and skin lymphomas. The 6-mev linear accelerator produces a pencil beam of electrons at 7 mev, which are scattered through a brass scatterer and decelerated by either of two carbon decelerators or a copper decelerator to produce a beam of effective energy of 2.5, 3, or 3.5 mev; these beams have an 80% isodose distribution at 5.5, 8, and 11.5 mm, respectively. The patient receives 200 rads to the anterior, posterior, and right and left lateral fields, for a total dose of between 1600 and 2600 ras in ten treatments over 5--7 weeks. Complete clearing of the disease can be predicted in all patients except those with the most advanced tumors. However, the duration of remission after electron-beam therapy is approximately 18 months and we are presently investigating the combination of psoralens and ultraviolet light therapy as maintenance treatment following lower-dose electron-beam therapy.     

Mycosis fungoides terminating in acute myelocytic leukemia

A 57-year-old woman was admitted to our hospital with suspected leukemia in September 1999. In 1990, systemic erythema had occurred, and mycosis fungoides (MF) had been diagnosed by skin biopsy. Interferon-gamma therapy had not been effective, and the erythema had disappeared after treatment with psoralen and ultraviolet A (PUVA) therapy (1.46 J/cm2). The patient had subsequently done well with a course of topical steroids. On admission this time, the WBC count was 1,600/microliter with 6% blasts. The total nucleated cell count in a bone marrow aspirate was 43.1 x 10(4)/microliter, of which 86.2% were peroxidase-positive blasts. Acute myelocytic leukemia (AML) was diagnosed. Chromosomal analysis demonstrated abnormalities of 48, XX, +4, +8, +add(10)(p11), add(11)(q23) in 10 of 20 cells, and 51, idem, +6, +8, +21, +mar in 8 cells with mixed-lineage leukemia gene rearrangement. Therapies (radiation, chemotherapy and PUVA) for MF, and the altered immune response seen in patients with this disease, especially in the more advanced stages, collectively termed cutaneous T-cell lymphoma (CTCL), suggest that such patients may be at increased risk of a second primary malignancy. To our knowledge, AML has been reported in 8 MF patients including the present one. Attention should be given to the possibility of MF terminating in AML.     

Mycosis fungoides in a hemodialysis patient with intractable pruritus

A 69-year-old Japanese man developed pruritus 3 years after beginning hemodialysis. Although eczema was not apparent at first, erythematous patches and plaques developed gradually on the affected skin. Secondary hyperparathyroidism was considered to be a main cause of this patient's pruritus, but skin lesions worsened even after parathyroidectomy had markedly decreased parathyroid hormone concentrations. Two months later, he developed an antibiotic-refractory fever of unknown origin and cervical, axillary, and inguinal lymphadenopathy. Elevations of soluble interleukin-2 receptor with 7410 U/mL and IgE with 24 600 U/mL in serum were noted, as was eosinophilia. The skin showed multiple slightly scaly patches and infiltrative plaques, which were reddish brown and distributed widely over the body surface except for the scalp and face. Mycosis fungoides, a cutaneous T-cell lymphoma, was diagnosed from biopsy specimens findings in skin and lymph node. Mycosis fungoides has not been documented as a cause of pruritus in hemodialysis patients. However, if skin lesions steadily worsen in hemodialysis patients, malignant diseases such as mycosis fungoides should be considered.     

Mycosis fungoides as a cause of severe obstructive sleep apnea

We report the case of a 38-year-old woman, affected by a cutaneous form of mycosis fungoides (MF) who presented with a history of loud snoring associated with sleep apnea. A polysomnographic study confirmed the presence of severe obstructive sleep apnea syndrome (OSAS). Cranial and neck MRI revealed a neoplastic infiltration of the tongue base and the posterior pharynx wall. Upper airway neoplastic infiltration is rarely reported as a cause of OSAS and extra-cutaneous localizations of MF are uncommon. This is the first case in the literature of a patient with nocturnal polysomnogram documented OSAS caused by a mucosal involvement of MF.     

Immunogold detection of CD3 and CD4 antigens in patients with Mycosis fungoides

In this study, we analyzed the distribution of CD3 and CD4 antigens at the ultrastructural level in tissue samples from mycosis fungoides patients using double-immunogold labeling. We observed clusters composed of CD3 and CD4 antigens on the plasma membrane and intracellular. There were also clusters only of one type of the antigen and we could observe more often CD4 than CD3. Labeling of CD3 and CD4 was not found in control cells incubated with non-immune serum. In conclusion, our ultrastructural studies not only visualized pattern distribution and relationship between CD3 and CD4 antigens but might also suggest that the type and form of distribution provides new clues to their possible translocation in mycosis fungoides cells.     

A retrospective comparative outcome analysis following systemic therapy in Mycosis fungoides and Sezary syndrome

Cutaneous T‐cell lymphomas (CTCL), with few exceptions, remain incurable and treatment is largely palliative. We performed a retrospective analysis of systemic treatment outcomes of patients diagnosed with MF/SS. We identified 223 patients with MF/SS evaluated at a single institution from 1997 to 2013. Disease stage at diagnosis, time of treatment, and treatments received were retrospectively analyzed using our CTCL database. The primary endpoint was time to next treatment (TTNT). Treatment outcomes were analyzed using Kaplan–Meier method and comparisons among groups were made using log‐rank analysis. A superior TTNT was associated with retinoid or interferon therapies when compared with HDAC inhibitors or systemic chemotherapy. Retinoids and interferon were associated with superior TTNT in both limited‐stage and advanced stage disease. Extracorporeal photophoresis (ECP) had a superior TTNT in Sezary Syndrome. HDAC inhibitors and chemotherapy were associated with inferior TTNT in both limited stage disease and advanced stage disease. With the exception of interferon, retinoids, or ECP, durable responses are rarely achieved with systemic therapies in MF/SS patients, particularly those with advanced‐stage disease. Therefore, clinical trial participation with novel agents should be encouraged. Am. J. Hematol. 91:E491–E495, 2016. © 2016 Wiley Periodicals, Inc.