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PubMed1985

Phenotypic characterization of lymphocyte subsets in mycosis fungoides. Comparison with large plaque parapsoriasis and benign chronic dermatoses.

Altogether, 209 skin biopsies from 103 patients with mycosis fungoides (MF), large plaque parapsoriasis (LPP), and benign chronic dermatoses (BCD) have been examined immunohistologically with the use of a panel of 21 monoclonal antibodies against lymphoid cells and their subsets. All the infiltrates contained a mixture of T-lymphocytes, Langerhans cells, and other types of HLA-DR-positive dermal macrophages. The neoplastic T-cells in MF lesions expressed proliferation-(transferrin receptor) and activation-(the OKT10 antigen) associated markers more frequently than the T-cells in LPP and BCD. In other respects, the neoplastic T-cells in plaque lesions of MF resembled those seen in LPP and BCD; and most of these cases demonstrated a clear predominance of T-cells of helper/inducer type. The neoplastic T-cells in tumor lesions of MF were much more heterogeneous in phenotype. Only eight of these cases could be classified as T-helper neoplasms. In the remaining ten tumor cases, the neoplastic cells expressed either suppressor/cytotoxic or aberrant T-cell phenotypes. There were no phenotypic differences between the "classical" tumor stages and MF d'emblee cases. The data indicate that the early lesions of MF show an immunohistologic reaction pattern common to many immune responses of the skin and that the neoplastic cells in the advanced stages are more heterogeneous in phenotype than previously recognized.

Adult↗
PubMed1991

Incidence of hairy cell leukemia, mycosis fungoides, and chronic lymphocytic leukemia in first known HTLV-II-endemic population.

Unlike human T cell leukemia-lymphoma virus type I (HTLV-I), HTLV-II has not been convincingly linked to a malignancy. In the first 10 months of serologic screening for HTLV-I/II among blood donors in New Mexico in 1988-1989, HTLV-I/II infection was found in 27 donors. HTLV-I/II infection was present in 1.0%-1.6% of American Indian and 0.16%-0.27% of Hispanic donors compared with 0.009%-0.06% of non-Hispanic white donors. HTLV-II was identified by DNA amplification in 12 of 13 samples from Indian and Hispanic seropositive donors. Despite apparent endemic HTLV-II infection in these populations, New Mexico Tumor Registry data showed that the incidences of hairy cell leukemia, mycosis fungoides, and chronic lymphocytic leukemia were comparable among the three ethnic groups. A population with endemic HTLV-II infection has been identified, and there is no evidence of increased risk for these three malignancies in the endemic groups.

Blood Donors↗
PubMed1978

Lack of functional immunoregulatory cells in a patient with mycosis fungoides and circulating Sézary cells.

Peripheral blood lymphocytes obtained at various intervals from normal individuals and from a patient with mycosis fungoides (MF) were cultured with phytohemagglutinin (PHA) for 3 days to activate suppressor cells. After being cultured, the PHA-treated cells were irradiated, washed, and then transferred to fresh medium with PHA. The PHA responsiveness of the cells from normal individuals was suppressed approximately 90% by autologous or normal allogeneic lymphocytes activated for 3 days with PHA, whereas the cells activated for 3 days with PHA from the patient with MF lacked the capacity to inhibit the mitogenic response of autologous or allogeneic lymphocytes. These data suggest that this patient lacked suppressor T-cells that have a specificity for helper T-cells.

Aged↗
PubMed1989

Atypical cutaneous lymphoproliferative disorder resembling mycosis fungoides in AIDS. Report of a case with concurrent Kaposi's sarcoma.

A variety of neoplastic disorders have been described in patients with AIDS. Kaposi's sarcoma is the most common neoplasm and is recognized as one of the diagnostic criteria for AIDS. Unusual mucocutaneous carcinomas have also been reported in these patients, as have a variety of lymphoid neoplasms, including Hodgkin's and non-Hodgkin's lymphomas (NHLs). The NHLs that occur in AIDS patients are usually of B-cell or non-B, non-T-cell phenotype. In contrast, T-cell lymphomas have only rarely been reported in this patient population. We present the clinical, morphologic, and immunologic features of an atypical, cutaneous lymphoproliferative disorder resembling mycosis fungoides that developed in a 28-year-old homosexual man with AIDS and disseminated mucocutaneous Kaposi's sarcoma.

Acquired Immunodeficiency Syndrome↗
PubMed1989

B cells and plasma cells in mycosis fungoides. A study including cases with B cell follicle formation or a monotypical plasma cell component.

B cells formed a distinctive component (greater than 5%) of the infiltrates in specimens of 28% (n = 12) of the 43 cases of mycosis fungoides studied. A large majority expressed surface immunoglobulin (Ig) D and/or IgM and a small minority IgA, similar to the proportions in inflammatory infiltrates. There was a prevalence of lambda light-chain expression. In about half of these cases, specimens revealed the formation of B cell follicles at various stages of development. Plasma cells formed a substantial number (greater than 25/mm2) in specimens from 21% (n = 9) of the patients. In six of these cases, including all four cases with advanced follicle formation, the same specimens were also rich in B lymphocytes. In most of the specimens the varying majorities of IgA or IgG producing cells and the polytypical expression of kappa and lambda light chains were similar to the patterns in inflammatory disorders. Two cases showed a striking prevalence of IgG, kappa-positive plasma cells, which was present in multiple specimens of one case and was associated with atypical plasmacytoid cells in a tumor of the other case. The pertinence of these observations for a special relationship between the abnormal T cells and the B cell component is discussed.

Adult↗
PubMed1995

Histopathologic findings in the clinically uninvolved skin of patients with mycosis fungoides.

Several of the methods of treating plaque-stage mycosis fungoides (MF) advocate treatment of the whole skin, although relatively little information exists on the histologic appearance of clinically uninvolved MF skin. Our study was performed to elucidate the histologic appearance of the clinically uninvolved skin of plaque-stage MF patients. Biopsies were taken from the clinically uninvolved skin of 18 untreated patients with plaque-stage MF, 1 cm (near) and > or = 10 cm (far) from the MF plaques, and from 23 normal, healthy controls. Eight to 12 serial sections from each specimen were examined. The most common histologic finding, which was observed in six (33%) biopsies of near skin and four (22%) biopsies of far skin compared with only one (4%) biopsy of normal control (p = 0.02 and 0.1, respectively), consisted of mononuclear-cell infiltrate around a blood vessel in the papillary dermis, with extension of some of these cells into the overlying epidermis. More diffuse and epidermotropic mononuclear cell infiltrates were seen in an additional three (17%) biopsies of near skin, whereas mild nonepidermotropic superficial perivascular or perifollicular mononuclear cell infiltrates were observed in an additional four (17%) biopsies of the normal controls. In conclusion, the most common histologic finding in our study, which was observed predominantly in the MF group, might represent an earlier stage and clinically undetectable involvement of the normal-looking skin in MF patients.

Adult↗
PubMed1996

Eccrine gland infiltration by mycosis fungoides.

After identifying prominent eccrine infiltration by atypical lymphocytes in a biopsy of tumor stage mycosis fungoides (MF), we sought to determine the pattern of eccrine epithelial infiltration in MF. The frequency, intensity, and distribution of infiltration of eccrine gland structures, including acrosyringium, duct and coil epithelium, was studied by examining 71 biopsy specimens from 42 patients with MF in which eccrine structures were present. These were obtained from a retrospective review of pathologic specimens from Duke University Medical Center from 1992 and 1993. At least focal eccrine infiltration was noted in 23 of the 71 biopsy specimens (32%). Immunohistochemical confirmation of T-lymphocyte phenotype was performed in the 23 cases with positive reaction to antibodies CD3 and CD45RO and negative reaction with CD20. Folliculosebaceous units were present in 22 of the 71 biopsy specimens and were at least focally involved by MF in 11 (50%) in this series. A control group of biopsy specimens of reactive dermatoses were characterized by more superficial location of lymphocytes, with more spongiosis and epithelial degenerative changes. These findings further illustrate the epitheliotropic behavior of MF.

Adult↗
PubMed1997

Persistent pigmented purpuric dermatitis and mycosis fungoides: simulant, precursor, or both? A study by light microscopy and molecular methods.

Mycosis fungoides (MF) can present with purpuric lesions, and rare patients who seemed to have persistent pigmented purpuric dermatitis (PPPD) have developed MF. We recently encountered two patients referred to our cutaneous lymphoma clinic who had PPPD rather than MF and two others who appeared to have both conditions, leading us to explore the histologic similarities of these diseases. We examined specimens from 56 patients with PPPD to determine the frequency of MF-like histologic configurations, namely, the psoriasiform lichenoid, psoriasiform spongiotic lichenoid, and atrophic lichenoid patterns. We also noted the degree of spongiosis, epidermotropism, papillary dermal fibrosis, lymphocytic atypia, and epidermal hyperplasia, the number of extravasated erythrocytes and siderophages, and the distribution of lymphocytic infiltrate within the epidermis. In 29 of 56 patients, there were patterns typically seen in MF. PPPD can feature lymphocytes aligned along the epidermal side of the dermoepidermal junction, with few necrotic keratinocytes, as can MF. Papillary dermal edema occurred frequently in PPPD but not in MF, while lymphocytes in MF but not PPPD had markedly atypical nuclei and had ascended into the upper spinous layer. Given these similarities, we tested for clonality of the T-cell population using a polymerase chain reaction assay for gamma-chain rearrangements. Clonal populations were present in three of three and one of two specimens from patients with both PPPD and MF, but also in 8 of 12 specimens typical of lichenoid patterns of PPPD. These findings raise the possibility that the lichenoid variants of PPPD are biologically related to MF.

Adult↗
PubMed1997

Evaluation of classic architectural criteria in non-mycosis fungoides cutaneous lymphomas.

Ninety-seven cases of non-mycosis fungoides (non-MF) cutaneous lymphoma were evaluated employing published criteria for the categorization of B- and T-cell cutaneous malignancies. Included in the study were 77 primary and secondary cutaneous B-cell lymphomas, in which the diagnosis was supported by immunohistochemical studies identifying lineage. These cases were randomized with 20 cases of non-MF and T-cell lymphoma. Hematoxylin and eosin (H & E)-stained slides from each case were reviewed by at least two dermatopathologists, who were unaware of the previous diagnoses, and a judgment regarding histologic pattern was rendered. The histologic criteria employed emphasized architectural features. For B-cell patterns, these included the presence of dense perivascular, periappendageal and/or nodular collections of lymphocytes, centering in the deep dermis, and separation from the epidermis by a grenz zone. Employed criteria for cutaneous T-cell pattern included location restricted primarily to the upper dermis, interstitial pattern, the presence of epidermotropism, and the lack of a grenz zone. Three B-cell lymphomas were judged to have indeterminate patterns. Four of 74 (5.4%) of the remaining B-cell lymphomas were incorrectly categorized as T-cell lymphomas by architectural criteria. The most striking findings included epidermotropism in rare B-cell lymphomas. Three of the four miscategorized cases were large-cell lymphomas. A preference for B-cell pattern was also confirmed in non-MF T-cell lymphomas. We conclude that most B-cell lymphomas in the skin demonstrate a recognizable B-cell pattern, but rarely a pattern more reminiscent of T-cell lymphoma may be seen. This may occur more often with B-large-cell lymphomas. In addition, this study supports previous work indicating that many non-MF T-cell lymphomas show prominent architectural features typically ascribed to B-cell lymphomas. In summary, our findings support the impression that the vast majority of non-MF lymphomas show a B-cell pattern regardless of their lineage. As such, caution is indicated with regard to pattern interpretation.

Adult↗
PubMed1999

How useful are T-cell receptor gene rearrangement studies as an adjunct to the histopathologic diagnosis of mycosis fungoides?

The diagnosis and differential diagnosis of mycosis fungoides (MF) is often difficult, clinically and histologically. Recent attempts to enhance diagnostic sensitivity have involved T-cell receptor (TCR) gene rearrangement studies, using Southern blot or polymerase chain reaction (PCR) technique. PCR is more widely used because of its increased sensitivity, lower labor- and cost-intensive analytic steps, lack of radioactive substances, and applicability to routinely processed biopsies. Several studies that use this technique detect clonal bands in 50% to 83% of the MF specimens from patch, patch/plaque, and erythrodermic stages, which often pose a diagnostic challenge. This compares with no clonality in the control groups of most of these studies, or with up to 13% in a few studies, although long-term follow-up reveal that some cases of so-called "clonal dermatitis" eventually progress into overt cutaneous T-cell lymphoma. Furthermore, retrospective studies on archival histologic material from patients with MF demonstrate similar rates of clonality in histologically "inconclusive," "borderline," and diagnostic MF biopsies. Therefore, in the proper clinicopathologic setting, and with consideration of the known limitations of this technique, TCR gene rearrangement studies on lesional skin using PCR may be helpful as an adjunct to the histopathologic diagnosis of MF.

Diagnosis, Differential↗
PubMed2000

Mycosis fungoides with CD30-positive cells in the epidermis.

Large numbers of CD30-positive tumor cells are not typically observed in mycosis fungoides (MF), but CD30 expression may occur on large cells of MF that have transformed into high-grade large cell lymphoma. Of 202 patch/plaque phase MF cases studied by immunohistochemistry, we encountered 4 patients with patch-phase MF at stage Ia or Ib, whose lesions contained a high proportion (greater than 50%) of CD30-positive tumor cells within the epidermis. The morphologic and immunohistochemical features of these four cases were otherwise similar to those of other patch-phase MF cases, and were different from those of pagetoid reticulosis. More importantly, the clinical behavior of these cases did not differ from that of other cases of early MF without CD30 expression. The mechanism underlying the high levels of CD30 expression by epidermotropic tumor cells is unknown. With increased use of the CD30 immunohistochemical stain in the diagnosis of cutaneous lymphomas, similar cases are likely to be encountered, and perhaps will be evaluated for their clinical behavior.

Adult↗
PubMed2002

A case of follicular mycosis fungoides with follicular mucinosis: a rare association.

Follicular mycosis fungoides (FMF) is a rare cutaneous T cell lymphoma characterized by an atypical lymphoid infiltrate spreading within and around hair follicles without epidermotropism or follicular mucin deposits. Its occasional presentation with minimal epidermal involvement and/or follicular mucinosis suggests the need for uniform histologic criteria. We describe a new case of FMF associated with follicular mucinosis and discuss its morphologic spectrum of presentation.

CD3 Complex↗
PubMed2003

Criterion by criterion, mycosis fungoides.

A wide variety of histologic features have been associated with mycosis fungoides. Virtually all these features can be seen at least episodically in some inflammatory conditions, and little is known regarding which histologic features are most useful in establishing a diagnosis of this challenging condition. Three recent blind histologic studies have addressed this topic, however, and their results and those of other studies are reviewed and compared herein.

Humans↗
PubMed2003

Lichen sclerosus with histopathologic features simulating early mycosis fungoides.

Mycosis fungoides (MF) is a cutaneous T-cell lymphoma characterized in its early stages by a superficial band-like infiltrate with epidermotropism of lymphocytes without particularly atypical cytologic features. Even though clinicopathologic presentation is diagnostic in typical cases, some inflammatory skin disorders can simulate the histopathologic features of early MF. In this study we present data on 9 patients affected by lichen sclerosus (LS) (M:F ratio 8:1; age range 7-75 years; mean age 31.3 years; median age 13 years), who presented with histopathologic features simulating early lesions of MF. The histopathologic picture was characterized in all cases by a dense, band-like infiltrate of lymphocytes within the superficial dermis, with exocytosis of lymphocytes within the lower part of the epidermis. The papillary dermis was expanded and showed focally coarse bundles of collagen simulating MF. The typical signs of LS were either absent or present only focally. Molecular analyses of the TCRgamma gene rearrangement performed with the polymerase chain reaction (PCR) technique revealed a polyclonal smear in eight cases, and a monoclonal band in one. Our study shows that LS can present with histopathologic features simulating early MF. Especially in cases revealing a monoclonal population of T lymphocytes by PCR, the correct diagnosis may be overlooked without proper clinical information and clinicopathologic correlation. Lichen sclerosus should be added to the list of cutaneous T-cell pseudolymphomas.

Adolescent↗
PubMedDate not supplied

Chromosomes and B and T cells in mycosis fungoides.

Because mycosis fungoides (MF) and Sézary syndrome (SS) share several features, some investigators have considered them to be different stages of the same disease. Others view them as separate entities. Cytogenetic studies in four typical MF patients showed that the chromosomal abnormalities were different from those reported in SS patients, and the abnormalities were found with almost equal frequency in both B and T cells. In addition, we found the frequencies of the T cells in six of the eight peripheral blood determinations in three patients were within normal range. These observations support the possibility that MF and SS are two separate disease entities.

Aged↗
PubMed1987

Mycosis fungoides: carcinogens and cerebral involvement.

Three patients with mycosis fungoides, who were long-term employees of a manufacturer of solid fuel propellants, were seen. Two of these patients had tumorous involvement of the central nervous system, which was successfully treated with radiation therapy. The potential relationship of carcinogens is discussed.

Brain Neoplasms↗