Value of High-Frequency Ultrasound in Accurate Staging of Mycosis Fungoides/Sézary Syndrome.

OBJECTIVES: This study was aimed to assess the ultrasound (US) characteristics of mycosis fungoides (MF)/Sézary syndrome (SS) and explore the value of high-frequency US in accuracy staging for classic mycosis fungoides (cMF). METHODS: A prospective study was designed. Twenty-six patients with histopathologically confirmed MF or SS were enrolled to undergo HF-US examinations. Both 50- and 20-MHz US images of the most prominent lesion of each patient were collected by a cutaneous diagnostic US system, and the US characteristics in different stages were analyzed by 2 physicians independently. The Fisher exact test was used for the statistical analysis. RESULTS: A total of 26 patients underwent this study, including 23 with cMF, 2 with folliculotropic mycosis fungoides (FMF), and 1 with SS. Among cMF, 16 patients with patches or plaques (the early-stage group) showed a subepidermal low-echogenic band, and only 3 lesions in the plaque stage partially extended to the superficial dermis. Seven patients with tumors (the advanced-stage group) showed lesions that infiltrated to the deep dermis or subcutaneous tissue. The infiltration depth (P < .001), clarity of the boundary (P = .002), and homogeneity of internal echoes (P = .001) were significantly different between the early and advanced stages. Additionally, the 2 FMF lesions and 1 SS lesion had characteristic manifestations, showing a well-defined subepidermal low-echogenic band with patchy hypoechoic regions around the hair follicles in the dermis. CONCLUSIONS: High-frequency US can be used to accurately detect the infiltration depth and morphologic features of MF/SS lesions and provide important information for tumor staging of cMF. Additionally, the characteristic US features in FMF and SS might be helpful for diagnosis.

Dermoscopy of Lymphomas and Pseudolymphomas.

Primary cutaneous lymphomas are a heterogeneous group that includes 2 main groups of primary T- and B-cell lymphomas, which can involve the skin with distinct variability in clinical presentation, histopathology, immunophenotypes, molecular signature, and prognosis. The authors describe the most frequent clinical forms of cutaneous lymphomas and their dermoscopic features. Even if the diagnosis of these entities is still based on a cellular level and the literature on dermoscopy in cutaneous lymphomas is limited and, for several entities it is based only on single case reports/case series, we think that know how they appear also in dermoscopy can be useful for helping in the clinical diagnosis.

[Cutaneous lymphomas]. / Lymphomes cutanés.

Cutaneous lymphomas are lymphoproliferations affecting skin only at the time of diagnosis. There are two major types, B-cell lymphomas and T-cell lymphomas, which prognosis depends of histological subtype and staging evaluation. In cutaneous B-cell lymphomas, there are two indolent subtypes (primary cutaneous marginal zone B-cell lymphoma and primary cutaneous follicle center lymphoma) and one more aggressive type (primary cutaneous diffuse large B-cell lymphoma, leg type). Classification of T-cell lymphomas distinguishes indolent subtypes such as mycosis fungoides, the most frequent of T-cell lymphomas, and CD30+ lymphoproliferations such as lymphomatoid papulosis, whereas other T-cell lymphoma subtypes have a more pejorative prognosis such as Sezary syndrome (erythrodermic and leukemic form of mycosis fungoides) and CD30- lymphomas. Staging evaluation with CT-scan of chest, abdomen and pelvis, bone marrow examination if necessary and lymph node biopsy if palpable node over 1 or 1.5 cm diameter, is necessary for therapeutic decision.

Staging accuracy in mycosis fungoides and sezary syndrome using integrated positron emission tomography and computed tomography.

OBJECTIVES: To evaluate the usefulness of integrated positron emission tomography and computed tomography (PET/CT) in staging mycosis fungoides (MF) and Sézary syndrome and to correlate PET/CT data with histopathologic diagnosis of lymph nodes (LNs). DESIGN: A single-center, prospective cohort analysis. SETTING: Academic referral center for cutaneous lymphoma. PATIENTS: Thirteen patients with MF and SS at risk for secondary LN involvement. Interventions Patients were clinically evaluated based on general physical examination, total body skin examination, and laboratory screening. They underwent integrated PET/CT followed by excisional biopsy of LNs. MAIN OUTCOME MEASURES: We used PET/CT to assess LN size and metabolic activity. Enlarged LNs were defined as axillary or inguinal LNs with a short axis 1.5 cm or larger; or cervical LN, with a short axis 1.0 cm or larger. We classified LN pathologic results according to National Cancer Institute (LN1-4) and World Health Organization (WHO 1-3) criteria. We quantified PET activity using standardized uptake value (SUV) and correlated with LN grade. RESULTS: Based on CT size criteria alone, only 5 patients had enlarged LNs, whereas PET revealed hypermetabolic LNs in all 13 patients. Six patients had LN1-3, and 7 had effacement of LN architecture by lymphoma cells (LN4). Of the 7 patients with LN4 nodes, 4 had SS, and 3 had tumorous MF. Two patients with LN4 nodes had inguinal LNs smaller than 1.5 cm and would have been assigned an N0 classification without the use of integrated PET/CT. Correlation of SUV with LN grade revealed that LN1-3 nodes were associated with a mean SUV of 2.7 (median SUV, 2.2; range, 2.0-4.7) and LN4 nodes were associated with a mean SUV of 5.4 (median SUV, 3.9; range, 2.1-11.8). Patients with large cell transformation had the highest SUVs. CONCLUSIONS: For staging MF and SS, PET/CT was more sensitive in detecting LN involved by lymphoma compared with CT data alone and thus may provide more accurate staging and prognostic information. The intensity of PET activity correlated with histologic LN grade.

Bilateral breast involvement in Sézary syndrome.

Cutaneous T-cell lymphoma is a term used for mycosis fungoides and Sézary syndrome, the distinct clinical entities where the skin is the primary organ of involvement. Sézary syndrome is the leukemic variant of mycosis fungoides, presenting with generalized erythroderma, lymphadenopathy, and atypical cells (the Sézary cells) in the peripheral blood and bone marrow. The dissemination of cutaneous T-cell lymphoma may occur with no exception of the organs; however, no prior report exists about the Sézary syndrome secondarily involving the breasts. We report the clinical and radiological findings of bilateral breast involvement in a case of Sézary syndrome.

Prognostic factors and evaluation of mycosis fungoides and Sézary syndrome.

BACKGROUND: Staging evaluations of patients with mycosis fungoides (MF) and Sézary syndrome (SS) are performed to individualize therapy and to predict survival. OBJECTIVE: Our purpose was to determine the prognostic factors in patients with MF and SS. METHODS: A retrospective study of 101 patients was performed. For inclusion in the study, patients had to have been evaluated for MF or SS within 6 months of the initial definitive histologic diagnosis. The evaluation included physical examination, chest radiograph, peripheral blood smear, lymph node biopsy, bone marrow biopsy, gallium 67 scan, liver-spleen scan and computed tomography (CT) of the chest, abdomen, and pelvis. RESULTS: The type of skin disease present at initial diagnosis was a good prognostic indicator of survival and clinical outcome. Univariate adverse prognostic features included hepatosplenomegaly or adenopathy by CT scan, abnormal liver-spleen scan, abnormal gallium scan, adenopathy, and peripheral blood, bone marrow, and lymph node involvement. Independent prognostic factors in multivariate analysis were the type of skin involvement as well as peripheral blood and visceral involvement. CONCLUSION: Our study confirms previous reports that type of skin and peripheral blood and visceral involvement are important prognostic factors in patients with MF or SS. Our results support the finding that patients with T1 stage disease have an excellent survival outlook and clinical outcome.

Diagnostic imaging in the initial staging of mycosis fungoides and Sézary syndrome.

Four imaging examinations-gallium citrate Ga 67 scintigraphy, liver-spleen scans, lymphangiography, and computed tomography-were used in the initial staging of mycosis fungoides and Sézary syndrome in 62 patients (85% with stage I or II disease). None of the imaging modalities added significantly to the information already available from physical examinations and routinely performed lymph node biopsies. The results of this investigation did not support routine performance of imaging studies in patients with early stages of cutaneous T-cell lymphoma.

Labeled cells in patients with malignancy.

The use of radioisotopes for cell labeling has been a major tool in hematology laboratory research. Chromium-51-labeling of hematologic cells and lymphocytes has been used for years to study the migration and sequestration of these cells in the spleen and other sites. The substantial recirculation of lymphocytes from blood into lymphoid tissue and back into blood is well described. Recently, new approaches for radiosotopic cell labeling have gained prominence in the investigation of various aspects of malignant diseases and in the clinical care of such patients. Isotopes such as indium-111 can be visualized with standard scanning techniques providing further information about the migration of normal and malignant cells has been discovered. In vivo studies have been performed with indium-111 in animals and humans, including comparisons of the migration of abnormal cells (malignant) and of lymphocytes to abnormal nodes. Evaluation and comparison of the migration of carcinoma cells, normal lymphoid cells, and malignant lymphoid cells in animals show markedly different patterns of distribution, which could have bearing on investigations of mechanisms of metastasis. In vivo human studies also have evaluated the migration patterns of lymphoid cells from patients with chronic lymphocytic leukemia and well-differentiated lymphoma, showing very different migrating behavior between these two polarities of a similar disease. These types of studies, while initially phenomenonologic, may provide a basis for a better understanding of these diseases. There are concerns about the use of an isotope such as indium-111 for the labeling of long-lived cells such as lymphocytes. Laboratory studies have demonstrated impaired cell function at high concentrations of radioactivity. Some workers have expressed concern about long-term changes in cells that recirculate. Others cite precedents of other long-term uses of isotopes, therapeutically, without detrimental effects. These concerns continue to be investigated. Finally, an area of much interest in the use of indium-111 is the labeling of granulocytes. This technique has been useful diagnostically, to localize infections. The major value in patients with malignancy, primarily with hematologic malignancies, is to evaluate the potential benefit of granulocyte transfusions. Many of these patients develop prolonged granulocytopenia and become infected, and granulocyte transfusions may become a therapeutic consideration.(ABSTRACT TRUNCATED AT 400 WORDS)

In-111 leukocyte scanning and partial functional asplenia in a patient with Sézary syndrome.

Functional asplenia is described in the case report of an 80-year-old woman who was admitted with Sézary syndrome. The spleen could not be visualized by a Tc-99m tin colloid (SnC) liver and spleen scan, but was visualized by a Tc-99m sulfur colloid (SC) scan, suggesting a different mechanism in accumulation of SnC and SC in the spleen. In-111 oxine labeled Sézary cells could be found in the spleen, bone marrow, and lymph nodes.

Functional asplenia in Sezary syndrome.

A case of functional asplenia revealed by radiocolloid scanning is reported in a patient suffering from Sezary syndrome, a lymphoma of cutaneous origin. Heavy, diffuse sinusoidal infiltration of the spleen by the Sezary cells appears to be responsible for the functional asplenia in this case.