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Werner Kempf

Publications and source records attributed to Werner Kempf.

At least 37 records · Page 2Linked to original sources
PubMed2004

Follicular lymphoma of the skin and superficial soft tissues associated with a prominent follicular dendritic cell proliferation: an unusual pattern which may represent a diagnostic pitfall.

We describe two elderly patients with follicular lymphoma (FL) involving the skin and superficial soft tissues, with a striking proliferation of follicular dendritic cells (FDC). In addition, one patient had bone marrow involvement by FL. Histopathologically, the most remarkable feature in both cases seen at low magnification was a striking pallor of the constituent cells, which were arranged in fascicles, whorls, and round islands. The majority of the cells had the typical cytologic features of FDCs. They were intimately intermingled with centroblasts and centrocytes. A large amount of the clear cytoplasm and the pale nuclei of FDCs, which predominated in the tumors, caused the striking overall pallor of the lesions. Small reactive lymphocytes were scattered between the fascicles. A vague follicular growth pattern was seen only focally. The mantle zones were markedly reduced or absent so that the follicles were seen lying unseparated. The close intermixture of the FDCs and the germinal center cells was responsible for the FDCs appearing to be decorated with B-associated marker, and the germinal center cells seemed to be stained to some degree with FDC-markers. The tumor bulk demonstrated a diffuse and strong reaction with CD10, CD20, CD21, CD35, and stained weakly with CD79a. Fascin and CD23 showed only a weak and focal staining pattern. Bcl-2 decorated large centroblasts and small reactive T-cells. The tumor bulk was negative for actin, EMA, cytokeratins, vimentin, desmin, and factor XIIIa. The proliferative index was rather low; MIB-1 mainly decorated large centroblasts. No monoclonal rearrangement of IgH genes was detected. Epstein-Barr virus was not identified. Electron microscopy revealed typical features of FDCs intermingled with germinal center cells. Such cases may represent a diagnostic pitfall, as FDC overgrowth can mask FL and give the neoplasm the appearance of FDC sarcoma/tumor. We believe that, in both cases, the FDC proliferation had a reactive character.

Aged↗
PubMed2004

The anti-MAGE antibody B57 as a diagnostic marker in melanocytic lesions.

The differentiation of melanoma from certain benign melanocytic lesions on histologic grounds alone may sometimes be difficult. The anti-MAGE antibody 57B was suggested to be a useful adjunct in differentiating melanoma from nevi. Our aim was to study MAGE immunoreactivity with B57 in benign melanocytic lesions that have not been investigated to this end so far. One hundred six benign melanocytic lesions were stained with the monoclonal antibody 57B. They included deep-penetrating nevus (n = 6), desmoplastic nevus (n = 9), halo nevus (n = 10), persistent melanocytic nevus (n = 12), common blue nevus (n = 17), cellular blue nevus (n = 8), cellular blue nevus with microalveolar pattern (n = 3), desmoplastic cellular blue nevus (n = 6), epithelioid blue nevus (n = 2), sclerotic blue nevus (n = 3), and clonal nevus (n = 30). Fifty-two lesions (49%) demonstrated various patterns of MAGE immunoreactivity, with clonal nevi and deep-penetrating nevi showing the most consistent staining. In conclusion, MAGE immunoreactivity detected by the monoclonal antibody 57B in formalin-fixed, paraffin-embedded tissue can be observed in benign melanocytic lesions, and therefore this antibody cannot be used in the differential diagnosis between melanoma and nevi.

Adolescent↗
PubMed2004

Melanoma with prominent pigment synthesis (animal-type melanoma): a case report with ultrastructural studies.

Melanoma with prominent pigment synthesis or animal-type melanoma (ATM) is a very rare type of melanoma. Its histogenesis has not been elucidated and ultrastructural features have not been described in human beings. We present an additional case of ATM in a 28-year-old woman with positive sentinel node biopsy and provide the results of electron microscopic studies. Histopathologically, the skin lesion was composed of heavily pigmented neoplastic cells mostly arranged as large sheets, focally also in a nodular growth pattern. After bleaching, the neoplastic cells demonstrated round nuclei with 1 or rarely 2 conspicuous nucleoli and a prominent nuclear membrane and abundant, gray, slate-like cytoplasm. Some cells demonstrated round cytoplasmic inclusions. There was no nuclear pleomorphism, and only a few mitotic figures could be found after extensive search. Multiple areas of necrosis en masse of tumor cells were seen. The lymph node biopsy revealed a complete effacement of the lymph node architecture by the extensive proliferation of hyperpigmented cells in the parenchyma. Immunohistochemically, the same pattern of staining was seen on the bleached and unbleached slides both in the skin and in the lymph node. The neoplastic cells stained positively with MiTF (nuclei), NSE, NKI/C3, tyrosinase (weak), p53, and CD68. S-100 protein, HMB45, Melan A, Mac367, and lysozyme reacted negatively. Occasional cells (<1%) reacted with MIB-1. Ultra-structural studies revealed that the neoplastic cells possessed a large, indented nucleus with a prominent nuclear membrane, a single (para) centrally located nucleolus, and peripherally marginated chromatin. The cytoplasm was abundant and contained numerous single melanosomes and rare compound melanosomes. The melanosomes were in stages II to IV of maturation, with a marked predominance of stage II and stage III melanosomes. There was a high number of aberrant melanosomes with a wide variety of configurations. Melanophages were a minor component of the lesion. Our ultrastructural studies provide unequivocal evidence that ATM is a neoplasm of melanosome-producing cells. We also review the literature on ATM.

Adult↗
PubMed2004

Low-grade trichoblastic carcinosarcoma of the skin.

We present an unusual case of cutaneous carcinosarcoma with the epithelial component closely resembling nodular basal cell carcinoma, and the mesenchymal component composed of cells constituting extended follicular papillae. A solitary tumor was excised in an 80-year-old man. Histologic sections revealed an ulcerated, asymmetric, poorly circumscribed neoplasm composed of epithelial cells arranged in lobules with peripheral palisading or in a cribriform pattern. The epithelial cells were darkly basophilic with scant cytoplasm and round or oval nuclei with an indistinct chromatin pattern and nucleoli. Nuclei crowding and mitotic figures were observed. Some lobules contained melanin. There were no shadow cells, sebaceous or apocrine glandular differentiation. Each epithelial nodule was surrounded by multiple rows of cells with pale vesicular nuclei and scant cytoplasm. Smaller epithelial aggregations were encircled by these cells concentrically; in larger ones these cells were aligned across a broad front resembling so-called "continuous papillae". Additionally, numerous small follicular germ-like structures associated with papillae were seen. The cells composing "continuous papillae" showed nuclear pleomorphism, numerous mitotic figures including atypical ones, and nuclear crowding. At foci, the transition from the multilayered arrangement of these cells into their diffuse proliferation in the stroma was seen. There were no transitions between the epithelial and stromal component; both were intermingled as though being mutually dependent, with no areas revealing a high-grade tumor or dedifferentiation. Immunohistochemically, the epithelial cell component stained with cytokeratins. The cells of the mesenchymal component tested positive for vimentin and negative for desmin and cytokeratins. The proliferation index (Ki-67) was high in both components. There were also a high number of p53-positive cells in both compartments. We propose the term "low-grade trichoblastic carcinosarcoma" for this neoplasm. We are not aware of a similar tumor published in the English literature.

Aged↗
PubMed2004

Castleman disease of the subcutis and underlying skeletal muscle: report of 6 cases.

Castleman disease of the subcutis and skeletal muscle is rare. We have collected a series of 6 cases of extranodal Castleman disease, located in the subcutis and skeletal muscles of the extremities and trunk. Tumors from mediastinal and retroperitoneal soft tissue and those histologically involving peripheral or truncal lymph nodes were excluded. There were four females and two males; ages ranged from 18 to 37 years (mean, 26 years). Locations included thigh (n = 3), anterior chest (n = 2), and upper arm (n = 1). Sizes ranged from 4.0 to 6.0 cm (mean, 5.2 cm). All patients presented with localized disease. One patient had involvement of the mediastinum 1 year prior to the appearance of his soft tissue lesion. None of the patients demonstrated systemic involvement or signs of the POEMS syndrome. Histopathologically, all cases were classified as hyaline-vascular type (HVCD). In 3 cases, follicular dendritic cell dysplasia was observed. In 1 case, the dysplasia was marked. The subcutaneous lesion of 1 patient revealed a maze of capillaries set in a lipomatous background with occasional lymphoid follicles possessing hyalinized lymphocyte-depleted centers. This lesion probably represented incipient HVCD. Molecular biologic studies did not reveal the presence of Epstein-Barr virus or human herpesvirus-8 DNA in the lesional tissue. There also were no monoclonal rearrangements of IgH. Four patients with follow-up included 2 patients with no evidence of disease at 10 and 13 years, respectively, and 2 patients with local recurrence at 2 and 6 months, respectively. In conclusion, soft tissue Castleman disease is a disease of young patients with a female predominance and a propensity to involve the trunk and limbs. It can be of large size and is generally solitary. There may be mild to marked follicular dendritic cell dysplasia. The HVCD predominates in this location. These lesions are usually unassociated with POEMS, Epstein-Barr virus, human herpesvirus-8, or monoclonal rearrangements of IgH.

Adolescent↗
PubMed2004

Multilineage progression of genetically unstable tumor subclones in cutaneous T-cell lymphoma.

Molecular analysis of solid malignant tumors has suggested multilineage progression of genetically unstable subclones during early stages of tumorigenesis as a common mechanism of tumor cell evolution. We have investigated whether multilineage progression is a feature of cutaneous T-cell lymphoma (CTCL). To identify individual tumor cell subclones, we determined the pattern of mutations within microsatellite DNA obtained from multiple histomorphologically confined tumor cell nests of mycosis fungoides (MF) and lymphomatoid papulosis (LyP) lesions. Tumor cells were isolated by laser microdissection, and allelotypes were determined at microsatellite markers D6S260, D9S162, D9S171, D10S215, TP53.PCR15, and D18S65. Nine cases of MF and one patient with anaplastic large cell lymphoma (ALCL) originating from LyP were analyzed at 277 different microdissected areas obtained from 31 individual lesions. Three specimens of cutaneous lichen planus microdissected at 26 areas served as the control tissue. Microsatellite instability in microdissected tissue [MSI(md-tissue)] was detected in tumor tissues of all CTCL patients. One hundred and fifty-seven of 469 analyzed polymerase chain reaction (PCR) amplifications contained mutated microsatellite alleles (34%). In lichen planus, MSI(md-tissue) was seen in only four of 76 PCR products (5%) (P < 0.0001). The distribution of allelotypes in tumor cells from different disease stages was consistent with multilineage progression in five MF cases, as well as in the LyP/ALCL patient. Our results suggest that CTCL may evolve by multilineage progression and that tumor subclones in MF can be detected in early disease stages by mutation analysis of microsatellite DNA obtained from multiple microdissected areas.

Alleles↗
PubMed2004

Micronodular Kaposi's sarcoma - a new variant of classic-sporadic Kaposi's sarcoma.

Kaposi's sarcoma (KS) in its classic-sporadic form is a rare vascular neoplasm affecting predominantly elderly men of Jewish or Mediterranean origin. Women are very rarely affected by KS. The tumor manifests itself most commonly with brownish macular or infiltrated oval lesions on the lower legs in both genders. Human herpesvirus 8 (HHV-8) has recently been demonstrated to be etiologically linked to KS. We report a 51-year-old HIV-seronegative Caucasian woman with an unusual, previously not described variant of KS which presented with small (3 mm in diameter) scattered firm red papules on her right arm. The histology with nodular spindle cell tumor expressing endothelial markers, the demonstration of serum antibodies against HHV-8 and the presence of viral nucleic acids in the lesional tissue proved the diagnosis of the nodular stage of KS. An indolent course characterized by clinically identical seasonal recurrences, even after surgical treatment and cryosurgery, was observed during the follow-up period of 9 years. We propose the term 'micronodular KS' for this unusual clinical variant of KS mimicking capillary hemangioma.

Antibodies, Viral↗
PubMed2004

Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis.

Nephrogenic fibrosing dermopathy (NFD) is a rare fibrosing skin disease of unknown etiology occurring in patients with terminal renal disease. It was first described in the year 2000. The histology of NFD shows an increased number of dendritic cells, fibroblasts and thickened collagen fibers resembling scleromyxedema. It can be distinguished from scleromyxedema by a different distribution pattern of the skin lesions with indurated plaques mainly on the extremities and the absence of paraproteinemia. As yet, no treatment for NFD has been proven to be uniformly efficient. We describe the case of a 40-year old patient with renal insufficiency who was treated with hemodialysis and who had undergone kidney transplantation. Two years after transplantation, she developed sclerodermiform brownish plaques on her extremities. The induration improved significantly after 4 cycles of extracorporeal photopheresis.

Adult↗
PubMed2004

Successful treatment of recalcitrant chronic actinic dermatitis with tacrolimus.

Therapeutically, chronic actinic dermatitis is a problematic condition. Frequently applied sunscreen usually fails to mitigate the clinical symptoms, and steroids--while efficient--exert many undesired side-effects with prolonged usage. We present a case of chronic actinic dermatitis responding well to topically applied tacrolimus (Protopic) in combination with chemical and physical UV protection.

Administration, Topical↗
PubMed2003

Superficial radiotherapy for patients with basal cell carcinoma: recurrence rates, histologic subtypes, and expression of p53 and Bcl-2.

BACKGROUND: The histologic subtype of a basal cell carcinoma (BCC) may be an important factor for the success of a certain treatment modality. In the current article, the authors report recurrence rates among patients with BCC after superficial radiotherapy as well as Bcl-2 and p53 expression levels stratified by BCC subtype. METHODS: The authors performed a retrospective study of 175 BCCs in 148 patients (64 female patients and 84 male patients; mean age, 69 years) who were treated with radiotherapy. According to their histologic patterns, BCCs were classified as nodular (n = 103), superficial (n = 25), and sclerosing (n = 47). In addition, six patients with metatypic BCC were reviewed. Bcl-2 and p53 protein expression was examined on a tissue microarray of 60 BCC samples (18 nodular tumors, 12 superficial tumors, and 30 sclerosing tumors). RESULTS: The estimated 5-year recurrence rate for all patients with BCC was 15.8%: 8.2% for patients with the nodular subtype, 26.1% for patients with the superficial subtype, and 27.7% for patients with the sclerosing subtype (Kaplan-Meier analysis: P = 0.055). The median follow-up was 48 months. The mean time to recurrence was 20 months, and 86.4% of all recurrences occurred within 3 years after treatment. No gender-specific differences were observed. In addition, one of six metatypic BCCs recurred. Nuclear p53 immunoreactivity and low Bcl-2 expression were significantly correlated with the sclerosing subtype. Overall, 61.5% of patients developed additional neoplasms during follow-up (76 developed additional BCCs, 15 developed squamous cell carcinomas, and 6 developed Bowen disease). CONCLUSIONS: The sclerosing subtype of BCC was a risk factor for recurrence after radiotherapy. In contrast, excellent results were achieved for patients with predominant nodular subtype. Nevertheless, radiotherapy may be the therapy of choice for patients with all BCC subtypes, depending on the individual patient's characteristics. Expression analyses confirmed that p53 and Bcl-2 levels may be used as indicators for the aggressiveness of a BCC subtype. Due to the high incidence of additional skin malignancies, patients with BCC need careful follow-up.

Adult↗
PubMed2003

Anti-inflammatory effects of exercise training in the skeletal muscle of patients with chronic heart failure.

OBJECTIVES: The aim of this study was to assess the effects of regular physical exercise on local inflammatory parameters in the skeletal muscle of patients with chronic heart failure (CHF). BACKGROUND: Inflammatory activation with increased serum cytokine levels and expression of inducible nitric oxide synthase (iNOS) in the myocardium and peripheral skeletal muscles has been described in CHF. METHODS: Twenty male patients with stable CHF (left ventricular ejection fraction 25 +/- 2%; age 54 +/- 2 years) were randomized to a training group (n = 10) or a control group (n = 10). At baseline and after six months, serum samples and vastus lateralis muscle biopsies were obtained. Serum tumor necrosis factor (TNF)-alpha, interleukin (IL)-6, and IL-1-beta levels were measured by enzyme-linked immunosorbent assay, local cytokine, and iNOS expression by real-time polymerase chain reaction. RESULTS: Exercise training improved peak oxygen uptake by 29% in the training group (from 20.3 +/- 1.0 to 26.1 +/- 1.5 ml/kg. min; p < 0.001 vs. control group). While serum levels of TNF-alpha, IL-6, and IL-1-beta remained unaffected by training, local skeletal muscle TNF-alpha decreased from 1.9 +/- 0.4 to 1.2 +/- 0.3 relative U (p < 0.05 for change vs. control group), IL-6 from 71.3 +/- 16.5 to 41.3 +/- 8.8 relative U (p < 0.05 vs. begin), and IL-1-beta from 2.7 +/- 1.1 to 1.4 +/- 0.6 relative U (p = 0.02 vs. control group). Exercise training also reduced local iNOS expression by 52% (from 6.3 +/- 1.2 to 3.0 +/- 1.0 relative U; p = 0.007 vs. control group). CONCLUSIONS: Exercise training significantly reduced the local expression of TNF-alpha, IL-1-beta, IL-6, and iNOS in the skeletal muscle of CHF patients. These local anti-inflammatory effects of exercise may attenuate the catabolic wasting process associated with the progression of CHF.

Biopsy↗
PubMed2003

Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop.

BACKGROUND: Cutaneous lymphomas expressing a cytotoxic or natural killer (NK) cell phenotype represent a group of lymphoproliferative disorders for which there is currently much confusion and little consensus regarding the best nomenclature and classification. METHODS: This study analyzes 48 cases of primary cutaneous lymphoma expressing cytotoxic proteins and/or the NK cell marker, CD56. These cases were collected for a workshop of the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Task Force, to better clarify the clinical, morphologic, and phenotypic features of these uncommon tumors. RESULTS: Several categories with different clinical and pathologic features were delineated: 1) aggressive, CD8+, epidermotropic, cytotoxic T-cell lymphoma; 2) mycosis fungoides, cytotoxic immunophenotype variant; 3) subcutaneous panniculitis-like T-cell lymphoma; 4) NK/T-cell lymphoma, nasal type; 5) CD4+, NK cell lymphoma; 6) blastoid NK cell lymphoma; (7) intravascular NK-like lymphoma; and 8) cytotoxic, peripheral T-cell lymphoma. CONCLUSIONS: Our data show that primary cutaneous cytotoxic/NK cell lymphomas include distinct groups of diseases, clinically, histologically, and biologically. Because the finding of a cytotoxic phenotype often has prognostic significance, the routine use of cytotoxic markers in the diagnosis and classification of cutaneous lymphomas should be expanded.

CD4-Positive T-Lymphocytes↗
PubMed2003

Topical and systemic retinoid therapy for cutaneous T-cell lymphoma.

Because curative therapies for CTCL are not yet available, short of TSEB in patients who have early-stage disease and allogeneic bone marrow transplantation in patients who have more advanced disease, the goal of current therapies is to prevent progression of MF and to preserve quality of life. The overall conclusion drawn from the studies reported in the literature, is that retinoids as monotherapy, or in combination with other nonaggressive treatment modalities, represent a low-risk treatment alternative that is especially suitable for controlling early stages of MF and other CTCL. A combination of therapies may be more effective in controlling CTCL as shown with IFN-alpha plus retinoids, and, recently, IFN-alpha with bexarotene and other modalities. For example, isotretinoin, followed by TSEB (for stage I to II disease) or preceded by chemotherapy (for stage II and IV disease) and bexarotene plus PUVA or photopheresis plus IFN, gave overall response rates of 82% and 69% in patients who had MF and SS, respectively. Retinoids as monotherapy may induce complete remissions, but usually these responses are of short duration and relapses are common. Clinical response is not identical to histologic clearance. Even in cases with clinically complete clearance of skin lesions, lymphoid infiltrates persisted, which are most likely the source of recurrences. The new generation of retinoids, the RXR selective agonists like bexarotene, represent a promising approach for refractory or persistent MF that is unresponsive to first-line therapies. Individual differences in response to retinoids may be due to different expression of retinoid receptors, functional polymorphisms in metabolizing retinoids, or resistance to some retinoids. In the future, pharmacogenomic studies are needed to clarify the mechanisms that underlie the differing response rates of patients who have CTCL to retinoids. In addition, new agonists of RAR and RXR, either selective or pan agonists, will become available and will enlarge the spectrum of vitamin A analogs that have antitumoral properties.

Adjuvants, Immunologic↗
PubMed2003

Hepatitis C and G viruses in B-cell lymphomas of the skin.

BACKGROUND: The etiology of B-cell lymphoproliferative disorders (LPDs) of the skin has still to be elucidated. So far, Borrelia sp. has been identified as the causative agent of some cases of B-cell LPDs of the skin. Apart from bacterial pathogens, recent studies suggested that also flaviviruses, in particular hepatitis C (HCV) and G (HGV) viruses, may be involved in the pathogenesis of B-cell non-Hodgkin's lymphomas (NHLs). Most studies were performed in patients with known HCV infection, but the overall frequency of HCV- and HGV-RNA in tumoral tissue of primary cutaneous B-cell lymphomas (CBCLs) is unknown. METHODS: We examined 23 tumor biopsies of various forms of CBCLs by reverse transcriptase-polymerase chain reaction (RT-PCR) and sequencing for the presence of HCV and HGV. RESULTS: HCV-RNA sequences were detected in seven of 23 (30%) of the tumor biopsies. In contrast, RNA sequences of HGV were not detected in any of the tumors. CONCLUSIONS: Interestingly, the presence of HCV in our series of primary CBCLs was not restricted to a distinct clinicopathologic subform. HCV which can infect B cells, may play a role in pathogenesis of one-third of CBCLs, whereas HGV is not involved in CBCLs. Further molecular studies and therapeutic trials are needed to clarify the putative pathogenetic role of HCV in CBCLs.

DNA Primers↗
PubMed2003

CO2 laser treatment of warts in immunosuppressed patients.

BACKGROUND: A large proportion of chronically immunosuppressed patients suffer from recalcitrant viral warts. Furthermore, the incidence of nonmelanoma skin cancer is clearly elevated in this patient group and human papillomavirus is most likely to play a causal role in these neoplasms. OBJECTIVE: To analyze the effectiveness of treatment of viral warts with the CO(2) laser in immunosuppressed patients. METHODS: Multiple viral warts of 13 patients with immunosuppression, a majority of them renal allograft recipients, were treated with CO(2) laser in local or regional anesthesia. In case of recurrences, the lesions were treated again after 3 months. RESULTS: 12 of 13 patients experienced dramatic improvement after 1-3 treatment sessions, 6 of these patients showed full remission. Side effects such as postoperative pain or scarring were minimal. CONCLUSION: CO(2) laser treatment of recalcitrant warts in immunosuppressed patients shows promising results which are comparable to those in immunocompetent hosts. This offers a welcome enlargement of the array of therapies in this otherwise difficult-to-treat group of patients and may contribute to the reduction of risk factors for nonmelanoma skin cancer.

Adolescent↗
PubMed2003

Imiquimod induces complete clearance of a PUVA-resistant plaque in mycosis fungoides.

Imiquimod is a topical immune response modifier that binds to Toll-like receptors 7 and 8 and induces alpha-interferon. We locally applied imiquimod 5% cream (Aldara) daily for 2 weeks on a PUVA- and retinoid-resistant plaque on the face of a patient with mycosis fungoides. The diagnosis was based on clinical appearance, histology and molecular studies. Most of the disease manifestations showed a clear remission during PUVA therapy combined with low-dose retinoids. However, the plaque on the face was PUVA resistant. Local imiquimod treatment resulted in the complete clearance of the plaque. The patient has now been in complete remission for 12 months.

Adjuvants, Immunologic↗