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Biomedical subjects

H K Müller-Hermelink

Publications and source records attributed to H K Müller-Hermelink.

At least 19 recordsLinked to original sources
PubMed2007

[Peripheral NK/T-cell lymphoma].

Peripheral T-cell lymphomas comprise 8% of the malignant lymphomas in Germany. About 25% of these cases present primarily in extranodal localizations. Such localizations are typical for the respective disease and form the basis for the classification of extranodal peripheral T-cell lymphoma. The morphology, immunophenotype and lineage specificity of the tumor cells (originating from T- or NK-cells) is only secondary for the classification. Extranodal NK/T-cell lymphomas of the nasal type are characterized by an angiocentric growth pattern and large confluent areas of necrosis. In addition, there is a clonal infection by Epstein-Barr virus in the T-lymphocytes. In the differential diagnosis, B-cell lymphomas are more frequent at all localizations than T- or NK-cell lymphomas.

Diagnosis, Differential↗
PubMed2007

[Cutaneous B-cell lymphoma. Classification and diagnostics].

Primary cutaneous B-cell lymphomas include cutaneous follicle centre lymphoma (PCFCL), cutaneous marginal zone B-cell lymphoma (PCMZL) and cutaneous diffuse large B-cell lymphoma (PCLBCL) "leg type" which are the three main types in the new WHO-EORTC classification for cutaneous lymphomas. PCFCL and PCMZL are indolent lymphomas with an excellent prognosis while PCLBCL shows an aggressive clinical course. All three types must be distinguished from a secondary skin involvement by systemic lymphomas. Since histological and immunohistochemical findings are not decisive, making this distinction requires appropriate staging procedures. In contrast, the pathologist can make an important contribution to the differential diagnosis between neoplastic and reactive cutaneous lymphoproliferations.

Humans↗
PubMed2007

[Enteropathy type T-cell lymphomas: pathology and pathogenesis].

Enteropathy type T-cell lymphomas (ETL) are the most frequent T-cell lymphomas arising in the gastrointestinal tract. Commonly, the neoplasm is clinically associated with symptoms of malabsorption, and it frequently manifests as a spontaneous bowel perforation. Among ETL, two types can be distinguished by morphology, immunophenotype and, possibly, by pathogenesis. A total of 80% of ETL are characterized by a close association with celiac disease, pleomorphic cytomorphology and the rare expression of CD8 and CD56. In contrast, 20% of ETL show a monomorphic small to medium size cytomorphology and frequent expression of CD8 and CD56, an association with celiac disease is rare in the latter cases. Genetically, ETL is characterized by frequent and recurrent chromosomal gains of 9q33-q34.

Chromosome Aberrations↗
PubMed2006

Nodal peripheral T-cell lymphomas correspond to distinct mature T-cell populations.

Peripheral T-cell lymphomas (PTCL) have not been successfully correlated with specific developmental stages of reactive T-cells. Mature T-cells pass through distinct stages upon antigen encounter. Naïve T-cells are CD45RA(+)/CD45R0(-)/CD27(+)/CCR7(+). After antigen contact they replace CD45RA expression with CD45R0. The mature T-cells differentiate to central memory cells, which retain CD27 and CCR7, or to effector memory cells, which lose expression of both molecules depending on the strength of the antigen interaction. In this study, we evaluated lymph node biopsies from eight PTCL-not otherwise specified (PTCL-NOS), seven angioimmunoblastic T-cell lymphomas (AILT), and 15 anaplastic large cell lymphomas (ALCL). Detection of tumour cells with antibodies that recognize specific rearranged T-cell receptor Vbeta segments allowed us to investigate the expression of various differentiation-associated molecules. Results were analysed by hierarchical cluster analysis. All AILT and ALCL showed a homogeneous effector cell phenotype (CD45RA(-)/CD45R0(+)/CD27(-)), but differed in the cytotoxic and activation markers expressed. Several (5/8) PTCL-NOS clustered together; these cases all exhibited a CD4(+) central memory cell phenotype (CD45RA(-)/CD45R0(+)/CD27(+)) and four expressed the lymph node homing receptor CCR7. In conclusion, AILT and ALCL tumour cells correspond to different subsets of effector cells, while a subset of PTCL-NOS correlates with a non-effector T-cell population.

CD4-Positive T-Lymphocytes↗
PubMed2006

[Optimized RT-PCR based detection of specific genetic abnormalities within malignant hematopoietic disorders].

The moleculargenetic detection of specific genetic abnormalities within malignant hematopoietic disorders is an important diagnostic tool with relevance for the differential diagnosis, therapy and prognosis.According to a modified and optimized RT-PCR based technique native bone marrow aspirates and peripheral blood samples of 183 patients were investigated for the presence of specific genetic abnormalities. The histological diagnosis of all CMLs t(9;22) and variants of AMLs M3 t(15;17) and t(11;17) were moleculargenetically confirmed. Additionally one t(5;12) positive chronic myelomonozytic leukemia, one t(8;21) positive AML M1, one t(9;22) positive B-ALL and in each case one t(6;9) and one t(3;21) positive myelodysplastic syndrome were detected. The hereby described method is a simple, specific and reliable technique for the rapid moleculargenetic detection of specific genetic abnormalities within malignant hematopoietic disorders with implication for the diagnosis/differential diagnosis, prognosis and therapy.

Gene Expression Regulation, Neoplastic↗
PubMed2005

Extended field radiotherapy, combined modality treatment or involved field radiotherapy for patients with stage IA lymphocyte-predominant Hodgkin's lymphoma: a retrospective analysis from the German Hodgkin Study Group (GHSG).

BACKGROUND: Since there are no randomized studies, the treatment of choice for patients with early stage lymphocyte-predominant Hodgkin's lymphoma (LPHL) remains unclear. We thus reviewed all LPHL cases registered in the database of the German Hodgkin Study Group (GHSG) and compared the different treatment approaches, such as extended field (EF), involved field (IF) radiation and combined modality (CM) treatment for LPHL stage IA patients. PATIENTS AND METHODS: One hundred and thirty-one patients with LPHL in clinical stage IA without risk factors were analyzed. Forty-five patients were treated with EF radiotherapy, 45 patients with IF radiation and 41 patients received CM treatment. The median follow-up was 78 months in the EF group, 40 months after CM and 17 months after IF, respectively. RESULTS: A total of 129 patients achieved complete remission (CR and CRu): 98% after EF radiotherapy, 100% after IF radiation and 95% after CM. With a median follow-up of 43 months there were 5% relapses and only three patients died. Toxicity of treatment was generally mild with most events observed after CM. CONCLUSION: In terms of remission induction IF radiotherapy for stage IA LPHL patients is as effective as EF or CM treatment. However, longer follow-up is needed before final conclusion as the optimal therapy.

Adolescent↗
PubMed2005

A prospectively randomized trial carried out by the German Hodgkin Study Group (GHSG) for elderly patients with advanced Hodgkin's disease comparing BEACOPP baseline and COPP-ABVD (study HD9elderly).

In contrast to younger patients, the prognosis of elderly patients with advanced Hodgkin's disease (HD) has not improved substantially over the last 20 years. We thus carried out a prospectively randomized study (HD9(elderly)) to compare the BEACOPP regimen in this setting against standard COPP-ABVD. Between February 1993 and 1998, 75 patients aged 66-75 years with newly diagnosed HD in advanced stages were recruited into the HD9 trial as a separate stratum (HD9(elderly)). Patients were assigned to eight alternating cycles of COPP and ABVD or eight cycles of BEACOPP in baseline doses. Radiotherapy was given to initial bulky or residual disease. In total, 68 of 75 registered patients were assessable: 26 were treated with COPP-ABVD and 42 with BEACOPP baseline. There were no significant differences between COPP-ABVD and BEACOPP in terms of complete remission (76%), overall survival (50%) and freedom from treatment failure (FFTF) (46%) at 5 years. At a median follow-up of 80 months, a total of 37 patients died: 14/26 patients (54%) treated with COPP-ABVD and 23/42 patients (55%) with BEACOPP. Two patients (8%) treated with COPP-ABVD and nine patients (21%) treated with BEACOPP died of acute toxicity. Hodgkin-specific FFTF at 5 years was 55% after COPP-ABVD and 74% after BEACOPP (P=0.13). Thus, there are no differences in survival between these regimens in elderly patients.

Administration, Oral↗
PubMed2005

Composite marginal zone B-cell lymphoma and classical Hodgkin's lymphoma: a clinicopathological study of 12 cases.

AIMS: Classical Hodgkin's lymphoma (cHL) rarely coexists as composite lymphoma with B-cell non-Hodgkin's lymphoma (B-NHL). We characterized 12 cases of composite marginal zone B-cell lymphoma (MZBL) and cHL by immunohistochemistry and molecular biology. METHODS AND RESULTS: Eight patients had gastric MZBL of mucosa-associated lymphoid tissue (MALT)-type, in five cases with a diffuse large B-cell lymphoma component. Concurrent cHL was observed either in the stomach wall, regional, or distant lymph nodes. One patient each had composite pulmonary/thyroid MZBL of MALT-type and cHL. In two cases, nodal composite MZBL and cHL was observed. cHL displayed features of mixed cellularity type in 10 cases, while in two cases only scattered Hodgkin- and Reed-Sternberg (H/RS) cells were noted. H/RS cells expressed CD30, multiple myeloma oncogene 1 protein (MUM1P), p53 (100%), CD15 (58%), CD20 (58%) and Epstein-Barr virus-associated LMP1 (50%). No t(11;18)(q21;q21) was detected in composite MZBL of MALT-type and cHL. CONCLUSIONS: MZBL and cHL may occur as composite lymphoma, possibly reflecting clonal lymphoma progression. Derivation from extranodal MZBL of MALT-type should be excluded in cases in which a diagnosis of primary extranodal cHL is considered.

Aged↗
PubMed2005

Extrafollicular proliferation of B cells in the absence of follicular hyperplasia: a distinct reaction pattern in lymph nodes correlated with primary or recall type responses.

AIMS: Extrafollicular activation of B cells is rarely observed in human lymph nodes. The aim of this study was to extensively analyse the expression of surface molecules and transcription factors in four such cases, comparing them with follicular B cells and medullary cord plasma cells. METHODS AND RESULTS: Various combinations of B-cell-related surface markers and transcription factors were studied by triple immunofluorescence. While in the germinal centre, reactive immunoglobulin production occurred exclusively in non-proliferating cells, in extrafollicular activation proliferation of B cells and immunoglobulin production coexisted. In two of these cases proliferating cells were mainly IgG+CD27+, i.e. derived from class-switched postgerminal centre memory B cells. Some of these cells expressed CD30. In the other two cases, immunoglobulin-forming cells were non-class-switched IgM+CD27- B cells, representing a primary expansion of naive B cells. CONCLUSIONS: Extrafollicular B-cell activation is the morphological correlate of rapid B-cell responses that do not involve the germinal centres. It is pathogenetically heterogeneous, comprising primary responses that occur prior to, or independent of, germinal centre reaction or memory cell activation in recall responses.

Antigens, CD↗
PubMed2005

Chronic non-bacterial osteomyelitis in children.

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) in children is a chronic non-suppurative inflammation involving multiple sites. Some children affected by chronic non-bacterial osteomyelitis (CNO) do not have multiple lesions or a recurrent course. OBJECTIVE: To characterise the long term outcome of children with the full spectrum of CNO. METHODS: 30 children diagnosed with CNO were followed up for a mean of 5.6 years and their disease assessed using a clinical score, multiple imaging, and a diagnostic biopsy, including extensive microbial analysis. RESULTS: 9 patients had unifocal non-relapsing disease, 3 unifocal lesions with relapses, 9 multifocal lesions without relapses, and 9 multifocal lesions with relapses (CRMO). Granulocytes were present significantly more often in CRMO than in unifocal and non-recurrent lesions. Pustulosis was more common in multifocal cases regardless of recurrence. Mean duration of treatment in 15 children with a single occurrence was 9.2 months. Naproxen treatment was generally effective. Naproxen treatment in 12 patients with relapses lasted 25 months. However, 7 of these were not effectively treated with naproxen alone. Five were treated with oral glucocorticoids for 27 days in addition to naproxen, which induced remission in four, lasting for at least 1.5 years. Longitudinal growth of affected bones was not altered, except for the development of hyperostosis. CONCLUSION: CNO is a spectrum of inflammatory conditions, with CRMO being the most severe. Most children with CNO have a favourable outcome of the disease. Oral glucocorticoids may be necessary in severe recurrent cases.

Adolescent↗
PubMed2004

[Clarification of tissue contamination in a suspected adenocarcinoma of the spermatic cord by microsatellite analysis].

Malignant tumors of the spermatic cord are rare with primary neoplasms being mostly sarcomas. Adenocarcinomas of the spermatic cord are found extremely seldom and are predominantly metastases of extragonadal tumors. We present an asymptomatic 47-year-old patient who was referred to our clinic after a vasectomy had been performed and the histological examination had surprisingly shown adenocarcinoma of the left spermatic cord. Extensive laboratory analysis and diagnostic imaging did not demonstrate any testicular, spermatic cord, or extragonadal tumor. To exclude a mix-up of specimens, we performed DNA analysis. Microsatellite PCR clearly demonstrated the different origins of the tumor and the vasectomy specimen. Specimen mix-up or contamination may happen in any phase of surgical or pathological processing. In any case of clinical uncertainty concerning the identity of a tissue specimen, this possibility has to be taken into consideration. Microsatellite PCR can clearly identify the origin of tissue samples even on embedded material.

Adenocarcinoma↗
PubMed2004

[Isolated Rosai-Dorfman disease].

A case of extranodal sinus histiocytosis with massive lymphadenopathy (ENSHML; Rosai-Dorfman disease) is reported. The patient presented with a history of intracranial tumour and exophthalmus. Clinical examination found a large mass in the left orbit and paranasal sinuses. Excisional biopsy showed a dense fibrous tissue with an infiltrate rich in macrophages. Further evaluation revealed a retroperitoneal mass with consecutive ureteral stenosis. Further histological and immunohistochemical investigation of the orbital mass, now in suspicion of a systemic disease showed an infiltrate of S-100-positive histiocytes and emperipolesis allowing the diagnosis of extranodal sinus histiocytosis. The correct histologic diagnosis was delayed due to the unusual and isolated extranodal localisation of the disease. The literature concerning extranodal manifestations of Rosai-Dorfman disease is reviewed. We suggest the additional evaluation of such rare and unusual cases in experienced reference centers.

Biopsy↗
PubMed2004

Hepatoid thymic carcinoma: report of a case.

We describe the clinicopathologic findings in a so far unrecognized thymic tumor. The tumor occurred in a 70-year-old woman with respiratory distress but neither myasthenia gravis nor other symptoms. Metastases or another primary tumor were absent. The well-circumscribed neoplasm was located in the thymic region, measured 18 x 12 x 8 cm, and showed a homogeneous, tan-colored, soft cut surface. By histology, the tumor lacked a true capsule and a lobular growth pattern, was almost devoid of stroma, and infiltrated among remnant thymus lobules. The polygonal tumor cells formed solid sheets, trabeculae, or occurred as single cells that resembled hepatocytes. Proliferative activity was low. Portal structures, sinuses, and bile were absent as were areas of conventional thymoma, adenocarcinoma, or germ cell tumor. The tumor expressed cytokeratins 7 and 19, alpha1-antitrypsin, alpha1-antichymotrypsin, and hep-Par-1. Alpha-fetoprotein (AFP), human beta-chorionic gonadotropin (beta-HCG), placental alkaline phosphatase, CD5, CD30, CD31, CD34, CD45, CD68, CD99, S-100, HMB45, desmin, actin, or neuroendocrine markers were not expressed, and intratumorous CD1a+ or TdT+ immature T cells were absent. AFP was repeatedly undetectable in the blood. Mediastinal tumor recurrence was detected 6 months after surgery. Following radiochemotherapy, the patient has remained free of disease for 26 months. We conclude that this tumor is a thymic carcinoma (WHO type C thymoma). A diagnosis of hepatoid yolk sack tumor appears unlikely considering absence of a bona fide germ cell component, lack of AFP expression, and the patient's female gender. Because of its morphologic and immunohistochemical features, we propose the term "hepatoid thymic carcinoma" for this new type of thymic carcinoma.

Aged↗
PubMed2004

[The spectrum of lymphoproliferations and malignant lymphoma after organ transplant].

Lymphoproliferative disease in patients with organ transplantation has increasingly been diagnosed in recent years. It is caused by immunosuppression after organ transplantion. A large percentage of these tumors is associated with Epstein-Barr virus infection. The classification differentiates between early lesions, polymorphic and monomorphic lymphoproliferations. Whereas the early lesions are often polyclonal, the remaining groups are mostly monoclonal. Polymorphic lymphoproliferative disease shows a wide spectrum of B-cell differentiation, whereas monomorphic proliferations resemble sproadic malignant lymphomas. The latter also show genetic abberations of the c-myc-, Ras-, p53- and Bcl6-genes. With reference to the germinal center reaction, posttransplant lymphoproliferations have to be assigned to the post-follicular activated B-cell type. While until 1991 only 2% of the cases of PTLD had an EBV-association, the EBV-associated PTLD have been frequently observed in recent times and presently constitute about 20% of the cases. EBV-negative PTLD constitute a negative prognostic factor. Posttransplant lymphomas might also represent a model for sporadic lymphoproliferative disease, that for example also occurs at elderly patients.

B-Lymphocytes↗
PubMed2004

[The overexpression of NCAM (CD56) in human hearts is specific for ischemic damage].

The main reason for myocardial dysfunction is chronical myocardial ischemia. Recently we could show, that NCAM (CD56), a neural cell adhesion molecule and member of the immunoglobuline superfamily, and the transcription factor AML1 (RUNX1) are overexpressed in chronic ischemic human heart failure compaired to normal hearts. Here we demonstrate, that the overexpression of NCAM (CD56) is specific for ischemic damage as compaired to other heart diseases including congestive cardiomyopathy, hypertrophic obstrutive cardiomyopathy, myocarditis and sarcoidosis. Concerning the transcriptional regulation of NCAM (CD56) by AML1 (RUNX1) we isolated 3 novel isoforms of AML 1 (RUNX1) with different transactivating function, that might be a regulatory element of the NCAM (CD56) overexpression in chronical myocardial ischemia.

Adult↗
PubMed2003

14-day variant of the bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone regimen in advanced-stage Hodgkin's lymphoma: results of a pilot study of the German Hodgkin's Lymphoma Study Group.

PURPOSE: This multicenter pilot study assessed the feasibility and efficacy of a time-intensified bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP) regimen given in 14-day intervals (BEACOPP-14) with granulocyte colony-stimulating factor (G-CSF) support in advanced Hodgkin's lymphoma. PATIENTS AND METHODS: From July 1997 until March 2000, 94 patients with Hodgkin's lymphoma stage IIB, III, and IV were scheduled to receive eight cycles of BEACOPP-14. Consolidation radiotherapy was administered to regions with initial bulky disease or residual tumor after chemotherapy. RESULTS: All patients were assessable for toxicity and treatment outcome. Eighty-six patients received the planned eight cycles of BEACOPP-14. Consolidation radiotherapy was given in 66 patients. Chemotherapy could generally be administered on schedule. Dose reductions varied among drugs but were generally low. Acute toxicity was moderate, with World Health Organization grade 3/4 leukopenia in 75%, thrombocytopenia in 23%, anemia in 65%, and infection in 12% of patients. A total of 88 patients (94%) achieved a complete remission. Four patients had progressive disease. At a median observation time of 34 months, five patients have relapsed, one patient developed a secondary non-Hodgkin's lymphoma, and three deaths were documented. The overall survival and freedom from treatment failure rates at 34 months were 97% (95% confidence interval [CI], 93% to 100%) and 90% (95% CI, 84% to 97%), respectively. CONCLUSION: Acceleration of the BEACOPP baseline regimen by shortening cycle duration with G-CSF support is feasible and effective with moderate acute toxicity. On the basis of these results, the German Hodgkin's Lymphoma Study Group will compare the BEACOPP-14 regimen with BEACOPP-21 escalated in a prospective multicenter randomized trial.

Adolescent↗
PubMed2003

Malignant thymoma: current status of classification and multimodality treatment.

Classification, understanding of the pathophysiology, and treatment options of thymoma have changed during recent years. It is hoped that novel strategies will lead to a survival benefit in these patients. It has become clear that patients with thymoma are best treated with multimodality therapy. In this review, a pathologist, an immunologist, a surgeon, a radiotherapist, a pneumologist, and oncologists discuss the current status of classification and strategies for the treatment of patients with thymoma.

Clinical Trials as Topic↗