CD4+ CD56+ blastic tumor cells express CD101 molecules.
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Antigens, CD↗
Biomedical subjects
T Petrella
Publications and source records attributed to T Petrella.
[Agranular CD4+ CD56+ CD123+ hematodermic neoplasm (blastic NK-cell lymphoma) revealed by cutaneous localization: 2 cases].
BACKGROUND: Agranular CD4+ CD56+ hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. OBSERVATIONS: Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of CD4, CD56, and CD123. No rearrangement of the T-cell receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutaneous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+ CD56+ cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred, followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. COMMENTS: The histological presentation of these two patients was very similar with an unusual phenotype of tumor cells expressing CD4, CD56, CD123, but not expressing CD3 and CD20. Some cases have been published under the "term of blastic NK lymphoma" which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting aggressive protocols, with bone marrow allograft in the younger patients.
Aged↗
A CD56-negative case of blastic natural killer-cell lymphoma (agranular CD4+/CD56+ haematodermic neoplasm).
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Aged↗
Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas.
The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.
Adult↗
Radiotherapy of advanced mycosis fungoides: indications and results of total skin electron beam and photon beam irradiation.
BACKGROUND: The goals of this retrospective study of advanced mycosis fungoides are (1) to describe the indications of a combination of total skin electron beam and photon beam irradiation and (2) to analyze the results of total body or segmental photon irradiation for patients with extension beyond the skin. METHODS: From January 1975 to December 1995, 45 patients with pathologically-confirmed mycosis fungoides or Sézary syndrome received a combination of TSEB and photon beam irradiation for advanced disease: 34 males and 11 females, mean age 61 years (range 27-87 years). The mean follow-up was 111 months (range 18-244 months, median 85 months). Whole-skin irradiation treatment to a depth of 3-5 mm with a 6-MeV electron beam was produced by a linear accelerator to a total dose of 24-30 Gy in 8-15 fractions, 3-4 times a week. In cases of thick plaques or tumors that were beyond the scope of low energy electron beams or for treating nodal areas (especially in the head and neck area or axilla involvement), regional irradiation (RRT) with Co-60 photon beams was followed by whole-skin electron beam irradiation (15 patients). In cases of diffuse erythrodermia, Sézary syndrome, nodal or visceral involvement, total body irradiation was delivered with a 25-MV photon beam using a split-course regimen to prevent hematological toxicity (22 patients). The first course consisted of 1.25 Gy delivered in ten fractions and 10 days. Subsequently, patients received TSEB. Four to 6 weeks after TSEB, they received a second course of 1.25 Gy. The cumulative TBI dose ranged from 2.5 to 3 Gy in about 3 months. Hemi-body irradiation (HB) with Co-60 (and a bolus) was given in cases of multiple regional tumors with large and thick infiltration of the skin to a dose of 9-12 Gy (using fractions of 1-1.5 Gy/day) which, once flattened, were boosted with whole-skin electron beam therapy (8 patients). RESULTS: At 3 months, the overall response rate was 75% with 23/45 (51%) patients in complete response and 24% in partial response; one patient had stable lesions and 1 patient presented progressive disease. The overall response rate was 81% for T3 patients, 61% for T4, 79% for N1 and 70% for N3. The complete response rate was 67% for T3 and 28% for T4. Sixty-four percent of N1 patients and 41% of N3 had a complete response. The 5-year actuarial overall survival was 37% for T3 and 44% for T4 (P = 0.84). Patients with clinically abnormal lymph nodes that were pathologically negative (N1) presented a 5-year survival of 63%. Patients with pathologically positive lymph nodes (N3) experienced a 5-year survival rate of 32% (P = 0.040). CONCLUSIONS: TSEB provides an excellent quality of life by reducing itching and discharge from the skin. Patients with more advanced disease may be treated and cured by the addition of photon beams in combination with TSEB. A selection of patients with advanced skin disease and regional extension may be cured by a combination of TSEB and photon beam irradiation. The regional treatment allows the use of electrons after the reduction of the plaques or thick tumors and a prophylactic irradiation of the adjacent nodal area.
Adult↗
Immunodetection of the microvillous cytoskeleton molecules villin and ezrin in the parasitophorous vacuole wall of Cryptosporidium parvum (Protozoa: Apicomplexa).
Microvilli - actin - villin - ezrin - Cryptosporidium parvum The sporozoites and merozoites of the Apicomplexan protozoan Cryptosporidium parvum (C. parvum) invade the apical side of enterocytes and induce the formation of a parasitophorous vacuole which stays in the brush border area and disturbs the distribution of microvilli. The vacuole is separated from the apical cytoplasm of the cell by an electron-dense layer of undetermined composition. In order to characterize the enterocyte cytoskeleton changes that occur during C. parvum invasion and development, we used both confocal immunofluorescence and immunoelectron microscopy to examine at the C.parvum-enterocyte interface the distribution of three components of the microvillous skeleton, actin, villin and ezrin. In infected cells, rhodamine-phalloidin and anti-villin and anti-ezrin antibodies recognized ring-like structures surrounding the developing parasites. By immunoelectron microscopy, both villin and ezrin were detected in the parasitophorous vacuole wall surrounding the luminal and lateral sides of the intracellular parasite. In contrast, anti-beta and anti-gamma actin antibodies showed no significant labelling of the vacuolar wall. These observations indicate that the parasitophorous vacuole wall contains at least two microvillus-derived components, villin and ezrin, as well as a low amount of F-actin. These data suggest that C.parvum infection induces a rearrangement of cytoskeleton molecules at the apical pole of the host cell that are used to build the parasitophorous vacuole.
Actins↗
CD4+ CD56+ cutaneous neoplasms: a distinct hematological entity? Groupe Français d'Etude des Lymphomes Cutanés (GFELC).
We report seven cases of particular cutaneous tumors selected from the register of the French Study Group on Cutaneous Lymphomas. The patients (three men, four women) were aged 37-86 years. They initially presented with cutaneous nodules or papules. Three cases presented with regional lymph nodes. Stagings were negative, except for one patient with bone marrow involvement. Histological features were relevant with pleomorphic medium T-cell lymphoma, but these cells exhibited a distinguishing phenotype. They were positive for CD4, CD56, and also CD45, CD43, and HLA-DR. All other T-cell and B-cell markers were negative. The myelomonocytic markers (CD13, CD14, CD15, CD33, CD117, myeloperoxidase, and lysozyme) were negative excepted CD68, which was clearly positive in four cases and weakly in two cases. Others natural killer cell markers (CD16, CD57, TiA1, granzyme B), TdT, and CD34 were negative. Polymerase chain reaction studies did not detect any B or T clonal rearrangement. The cytogenetic studies, performed in five cases, showed a del(5q) in two cases. All patients were treated successfully by polychemotherapy, but relapsed quickly in the skin, between 4 and 28 months. Five patients developed bone marrow involvement, with leukemia in three cases, and they died in 5-27 months. One patient died at 17 months with skin progression. The seventh patient is alive at 33 months, with cutaneous progression. The origin of these cells is unclear. Despite expression of CD4 or CD56, we failed to demonstrate a T-cell, natural killer cell origin. However, CD4 and CD56 are not specific for T or natural killer lineages. Although these two markers are also known to be expressed by monocytic cells, classic myeloid antigens were negative. These seven cases, together with other rare similar cases already reported, seem to represent a distinct entity likely developed from hematological precursor cells.
Adult↗
Prognostic significance of a polymerase chain reaction-detectable dominant T-lymphocyte clone in cutaneous lesions of patients with mycosis fungoides.
Although mycosis fungoides (MF) is considered to be an indolent lymphoma, survival is highly influenced by TNM stage. At diagnosis, most MF patients present with early stage disease and a high probability of long-term survival. Treatment is generally directed towards skin lesions, and achievement and duration of complete responses are variable. A dominant T-cell clone is detectable in the cutaneous lesions of 60% of patients. The aim of this study was to determine whether the presence of a T-cell clonal population influences the clinical course of the disease after topical therapy. Cutaneous biopsies from 68 patients were histologically diagnosed as MF and T-cell clonality was analyzed by in vitro amplification of TCR-gamma chain gene rearrangements (polymerase chain reaction gamma [PCRgamma]). After a median follow-up of 48 months, response to treatment was clinically assessed. Age, sex, duration of symptoms before diagnosis, type of cutaneous lesions (T stage), TNM stage, and PCRgamma were evaluated as predictive factors of response to treatment in univariate and multivariate analyses. Univariate analysis demonstrated that T1 cutaneous lesions (P = .05) and PCRgamma negativity (P = .007) were associated with a higher complete remission rate. Using multivariate analysis, T stage (relative risk, 3.13; P = .06) and PCRgamma (relative risk, 4.4; P = .01) remained independent significant predictive parameters of response. In conclusion, T stage and cutaneous PCRgamma at diagnosis are the two predictive parameters of treatment response for MF. Therefore, the cutaneous PCRgamma findings should be considered in the analysis of future therapeutic trials.
Administration, Cutaneous↗
Value of clonality studies of cutaneous T lymphocytes in the diagnosis and follow-up of patients with mycosis fungoides.
Histological features of early mycosis fungoides (MF) can simulate numerous inflammatory lesions and histological confirmation of MF is often delayed, compared with clinical diagnosis. Recently, using molecular techniques, the detection of a dominant T-lymphocyte clone has been reported in cutaneous lesions of MF. The aim of the present study was to determine the diagnostic value of a dominant T-lymphocyte clone as assessed by PCR-DGGE in early MF. Histopathological and molecular analyses were performed on cutaneous lesions from 104 patients clinically suspected as having MF. In this population, the positive predictive value of a PCR gamma(+) was 0.86. In addition, four of six patients whose lesions were PCR gamma(+) (detectable dominant T-cell clone) but not histologically MF progressed to MF within 2-48 months. In order to evaluate the relevance of PCR gamma-DGGE in MF follow-up, serial biopsies were performed in 24 patients. In 89 per cent of cases, the presence or absence of a PCR gamma(+) was constant during the course of the disease. When present, the DGGE imprint of PCR products was case-specific. These data demonstrate the diagnostic value in MF of T-lymphocyte clonality assessed by PCR gamma-DGGE on cutaneous lesions and show that the technique can be used in MF follow-up to evaluate residual disease with high specificity.
Clone Cells↗
Primary anaplastic large-cell lymphoma in adults: clinical presentation, immunophenotype, and outcome.
Anaplastic, CD30+, large-cell lymphoma is now a well-recognized pathologic entity that accounts for 2% to 8% of all lymphomas. Recent progress has been made in the understanding of certain biologic features found in anaplastic large-cell lymphoma, but information about its clinical behavior, in comparison to other large-cell lymphomas, is limited. The pathologic review of a large multicenter study of the treatment of aggressive lymphoma identified 146 cases of anaplastic large-cell lymphoma (ALCL) on the basis of morphology and CD30 expression. We compared initial presentation, immunophenotype, and clinical outcome of these cases with those of the 1,695 nonanaplastic diffuse large-cell lymphomas (non-ALCL) included in the same trial. Patients with ALCL were more likely to be male (P = .018) and were younger (P < .0001) than those with non-ALCL. B symptoms were more frequent in ALCL (P = .006). Skin (P < .0001) and lung (P < .05) involvement was also more frequent in ALCL, but frequency of bone marrow involvement was identical (P = . 5). Tumor cell phenotype was B in 56 cases (38%), T in 49 cases (34%), and null in 33 cases (22%). Response to chemotherapy (P = . 001), event-free survival (P = .006), and overall survival (P = . 0004) were better for ALCL than for non-ALCL. Multivariate analyses identified anaplastic character as an independent factor that predicted a longer survival. Tumor cell phenotype did not influence event-free survival (P = .72) or overall survival (P = .83). ALCL in adults is a clinicopathologic entity which, independent of its phenotypic characteristics, has a better outcome than other diffuse large-cell lymphomas.
Adult↗
Hepatoid adenocarcinoma of the lung: report of a case of an unusual alpha-fetoprotein-producing lung tumor.
We report on a rare tumor of the lung characterized by its morphologic hepatoid features and alpha-fetoprotein production. This unusual neoplasm arose in the left lung of a 36-year-old man in whom clinical and radiologic examinations did not reveal any other tumor. The serum level of alpha-fetoprotein was measured at 6,090 ng/mL and was parallel to the evolution of the tumor. Despite treatment, the patient died 7 months after the diagnosis. The microscopic appearance of the tumor was the same as observed in hepatocarcinoma and hepatoid adenocarcinoma of the ovary or the stomach, with a tubular, papillary, or trabecular pattern. Periodic acid-Schiff-positive hyaline globules were numerous, and tumor cells showed immunohistologic positivity for alpha-fetoprotein and carcinoembryonic antigen. This lung adenocarcinoma was first described by Ishikura et al. in 1990 and was named hepatoid lung adenocarcinoma. Like the rare hepatoid carcinoma of the gallbladder, the pancreas, the ampulla of Vater, the renal pelvis, and the bladder, the exact histogenesis and the prognosis of this type of lung tumor are not yet known.
Adenocarcinoma↗
Improved management of invasive pulmonary aspergillosis in neutropenic patients using early thoracic computed tomographic scan and surgery.
PURPOSE: The prognosis of invasive pulmonary aspergillosis (IPA) occurring in neutropenic patients remains poor. We studied whether new strategies for early diagnosis could improve outcome in these patients. PATIENTS AND METHODS: Twenty-three histologically proven and 14 highly probable IPAs in 37 hematologic patients (neutropenic in 36) were analyzed retrospectively. RESULTS: The most frequent clinical signs associated with IPA were cough (92%), chest pain (76%), and hemoptysis (54%). Bronchoalveolar lavage (BAL) was positive in 22 of 32 cases. Aspergillus antigen test was positive in 83% of cases when tested on BAL fluid. Since October 1991, early thoracic computed tomographic (CT) scans were systematically performed in febrile neutropenic patients with pulmonary x-ray infiltrates. This approach allowed us to recognize suggestive CT halo signs in 92% of patients, compared with 13% before this date, and the mean time to IPA diagnosis was reduced dramatically from 7 to 1.9 days. Among 36 assessable patients, 10 failed to respond (amphotericin B [AmB] plus fluorocytosyne, n = 2; itraconazole + AmB, n = 8) and died of aspergillosis. Twenty-six patients were cured or improved by antifungal treatment (itraconazole with or without AmB, n = 22; voriconazole, n = 4). In 15 of 16 cases, surgical resection was combined successfully with medical treatment. Achievement of hematologic response, early diagnosis, unilateral pulmonary involvement, and highest level of fibrinogen value < 9 g/L were associated with better outcome. CONCLUSION: In febrile neutropenic patients, systematic CT scan allows earlier diagnosis of IPA. Early antifungal treatment, combined with surgical resection if necessary, improves IPA prognosis dramatically in these patients.
Antigens, Fungal↗
Systemic mast cell disease associated with hairy cell leukaemia.
Systemic mast cell disease (SMCD) can be regarded as a tumorous proliferation of tissue mast cells involving various organs. The frequency with which SMCD is found in patients with haematological disorders suggests that the association is non-random. The association includes primarily, myeloid disorders such as myelodysplastic syndromes and acute or chronic myeloproliferative disorders. Lymphoproliferative disorders may also occur but more rarely, mostly non-Hodgkin's low grade B cell lymphomas. In this report a case is described in which SMCD occurred in a patient with hairy cell leukaemia.
Humans↗
Changing trends in the incidence of non-Hodgkin's lymphoma in Europe. Biomed Study Group.
Non-Hodgkin's lymphoma (NHL) is not a uniform disease entity, and in order to investigate the reported changes in incidence we have set up a study in seven population-based cancer registries in Europe. The study is designed to look at changes in the incidence of total NHL and disease subgroups using standard definitions and methodology. The registries are based in Leeds, Dijon, Kuopio, Odense, Florence, Eindhoven, and Ragussa. The classification system we have used is based on the REAL classification and has utility for epidemiological studies. We have used it to convert data sets which have utilized both local cases and the ICD-O classification. In order to improve data reproducibility, CLL/LL, myeloma/MGUS, lymphoblastic disease, and Hodgkin's disease have been excluded because of the difficulty in defining incident cases accurately. The preliminary results of this study show that there is still an upward trend in incidence rate and that in Yorkshire this is 3% per annum in total NHL. The subgroups which are increasing are extranodal and nodal peripheral T-cell lymphoma. Similar increases in incidence have been reported for the other registries. We conclude that there is a continued upward trend in incidence of NHL, the causes of which are uncertain.
Europe↗
CD5-CD56+ T-cell receptor silent peripheral T-cell lymphomas are natural killer cell lymphomas.
Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms. The existence and the clinical relevance of lymphomas derived from the third lymphocyte lineage, ie, natural killer (NK) cells are still controversial. NK cells are lymphocytes that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics: they express the NK-related antigen CD56, T-cell markers such as CD2 and CD7, but do not express CD5 and T-cell receptor (TCR) proteins, and their TCR locus is not rearranged. Therefore, if NK cell lymphomas exist, they should express some T-cell markers, but not alpha beta or gamma delta TCR proteins. Such lymphomas are actually called TCR silent peripheral T cell lymphomas (PTCL). To detect and characterize NK cell lymphomas, we investigated the immunophenotype and immunogenotype of 35 patients with TCR silent PTCL. The first group included 16 patients with a lymphoma of CD5-CD56+ phenotype, which is identical to normal NK cells. These patients had either a nasal/nasopharyngeal lymphoma (11 cases) or a lymphoma with predominant non-nasal/non-nodal initial involvement (five cases). Eight of the nine cases for which immunogenotypic data were available lacked clonal rearrangement of the TCR gamma genes. Thus, these tumors are likely to be NK cell lymphomas. The second group of 15 cases had a CD5+ phenotype (14 were CD56-, and 1 was CD56+) and clonal rearrangement of TCR gamma genes, indicating that they were true PTCL with unproductive TCR rearrangement. The four remaining cases were CD5- CD56- lymphomas and disclosed either a clonal (two cases) or no clonal (two cases) rearrangements of the TCR gamma genes. Altogether these findings show that CD5-CD56+ so-called "TCR silent PTCL" bear the immunophenotype and immunogenotype of normal NK cells and display peculiar clinical features distinct from true PTCL.
Adolescent↗
Translocation (21;22)(q22;q12) in a Ewing sarcoma. New case characterized by cytogenetic analysis and fluorescence in situ hybridization of a fresh tumor.
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Adolescent↗
Nasopharyngeal lymphomas: further evidence for a natural killer cell origin.
The authors report four cases of sinonasal lymphomas with immunophenotypic (four cases) and genotypic (three cases) studies. These lymphomas are frequent in Oriental countries, but unusual in Western populations, particularly in France. Although they have been originally considered as T-cell lymphomas, their origin remains unclear, and several authors have recently suggested that they are a natural killer (NK)-cell proliferation. The tumor cells of the four cases reported here showed characteristics of NK cells. On cryostat sections, they were CD3 negative, but CD2 and CD56 positive. The cytoplasm of the tumor cells exhibited azurophilic granulations. Using polymerase chain reaction (PCR), no clonal rearrangement of the T-cell receptor gamma gene was present. Furthermore, clinically, two presented a pharyngeal involvement, and two were also characterized by hepatosplenic involvement at the time of the diagnosis. Thus, the present cases provide additional evidence toward the NK-cell origin of these rare lymphomas. Many sinonasal lymphomas, including the present cases, are positive for Epstein-Barr virus (EBV) (LMP-1 antibody and EBER-messenger RNA probes). This may suggest an important role of EBV as a local factor in their pathogenesis.
Adult↗
Epidemiological characteristics of myelodysplastic syndrome in a well-defined French population.
Data on myelodysplastic syndromes (MDS) are seldom collected by cancer registries and unbiased findings from population-based studies remain rare. We report detailed information on MDS in a well-defined French population in the period 1980-1990. The crude incidence rate was 3.2 per 100000 per year and no significant change in incidence was noted in the study period. The sex ratio was 1.9 and the male predominance was present in all age groups. We observed a rise in incidence after 60 years of age but no significant change in incidence of MDS as a whole was observed over the period studied. Refractory anaemia with excess of blasts (RAEB) was the most frequent subtype. Overall 5 year transformation rate of MDS was 31% (+/- 4%) but it was 100% in RAEB in transformation. The observed 5 year survival rate was 23% +/- 3% and the corresponding corrected rate was 33%. The prognosis of RAEB in transformation was worse than the prognosis of other subtypes (P < 0.01). Discrepancies with epidemiological data from other European countries are discussed.
Adult↗