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Abstract Background In a recent study we showed that in patients with primary biliary cirrhosis (PBC) being positive or negative for anti-M2 antibodies reacting with the 2-oxoacid-dehydrogenase complex (ODC) also antibodies to the beta- and gamma-subunits of F1F0-ATPase (anti-β, anti-γ) occur. This is a mitochondrial enzyme but parts are also expressed on plasma membranes of endothelial cells. Here we wanted to analyse in more detail their clinical relevance. Methods Fifty-nine untreated and histologically defined PBC patients who had been followed for at least five years were included into the study (51 anti-M2 positive, 8 anti-M2 negative). Twenty-three of them were treated in the follow up with ursodeoxycholic acid (UDCA), eight received during a trial methotrexate (MTX). In 13 patients orthotopic liver transplantation (OLT) had to be performed. Serum samples before and during therapy were available. Patients were analysed with respect to laboratory parameters, disease activity and histological stages. Patients’ sera were tested by ELISA for IgG- and IgM-antibodies against the beta- and gamma-subunits which had been recombinant expressed in E.coli and highly purified by electro-elution from SDS-gels after electrophoresis. Results Fifty-nine percent of the anti-M2 positive and 50% of the anti-M2 negative PBC patients had anti-β- and/or anti-γ-antibodies. There were no differences between anti-β- and/or anti-γ-antibody positive or negative patients with respect to biochemical parameters, immunoglobulins, histological stages or disease activity. Antibody reactivity significantly decreased during UDCA and MTX-treatment and also after OLT. Conclusions Antibodies to the β- and γ-subunits of F1F0-ATPase occur in anti-M2 positive and –negative PBC but do not have any relevance with respect to clinical activity or prognosis. However, in contrast to the anti-M2 antibodies they decrease during UDCA and immunosuppressive therapy.

Background: In a recent study we showed that in patients with primary biliary cirrhosis (PBC) being positive or negative for anti-M2 antibodies reacting with the 2-oxoacid-dehydrogenase complex (ODC) also antibodies to the beta- and gamma-subunits of F1F0-ATPase (anti-β, anti-γ) occur. This is a mitochondrial enzyme but parts are also expressed on plasma membranes of endothelial cells. Here we wanted to analyse in more detail their clinical relevance. Methods: Fifty-nine untreated and histologically defined PBC patients who had been followed for at least five years were included into the study (51 anti-M2 positive, 8 anti-M2 negative). Twenty-three of them were treated in the follow up with ursodeoxycholic acid (UDCA), eight received during a trial methotrexate (MTX). In 13 patients orthotopic liver transplantation (OLT) had to be performed. Serum samples before and during therapy were available. Patients were analysed with respect to laboratory parameters, disease activity and histological stages. Patients' sera were tested by ELISA for IgG- and IgM-antibodies against the beta- and gamma-subunits which had been recombinant expressed in E.coli and highly purified by electro-elution from SDS-gels after electrophoresis. Results: Fifty-nine percent of the anti-M2 positive and 50% of the anti-M2 negative PBC patients had anti-β- and/or anti-γ-antibodies. There were no differences between anti-β- and/or anti-γ-antibody positive or negative patients with respect to biochemical parameters, immunoglobulins, histological stages or disease activity. Antibody reactivity significantly decreased during UDCA and MTX-treatment and also after OLT. Conclusions: Antibodies to the β- and γ-subunits of F1F0-ATPase occur in anti-M2 positive and -negative PBC but do not have any relevance with respect to clinical activity or prognosis. However, in contrast to the anti-M2 antibodies they decrease during UDCA and immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

T‑follicular helper (TFH) cell lymphoma (TFHL) is a lymphoma of mature T cells with phenotypic characteristics and gene expression signature of TFH cells. The lymphoma harbors recurrent mutations of RHOA G17V , IDH2 R172 , TET2 and DNMT3A. Whereas RHOA G17V and IDH2 R172 are almost exclusively found in this entity, TET2 and DNMT3A mutations occur in a broad variety of hematological neoplasms and are the most frequently affected genes in clonal hematopoiesis (CH). CH in humans shows a progression rate to overt hematologic neoplasia of about 0.5 to 1% per year, depending on clone size, number of mutations and affected genes. In 2018, the first case was described in which a lymphoid (TFHL) and myeloid (acute myeloid leukemia) neoplasm arose from a common mutated progenitor cell with shared mutations and additional private mutations. In recent years, further studies showed in up to 70% of patients with TFHL the occurrence of identical mutations of TET2 and/or DNMT3A in the myeloid cells, irrespective of bone marrow involvement, indicating a prominent role of CH in the pathogenesis of TFHL. In up to 18%, these patients show also additional synchronous or metachronous overt myeloid neoplasms, often with private myelodysplastic-type mutations, most often myelodysplastic syndrome, chronic myelomonocytic leukemia and acute myeloid leukemia. Recently, there is also evidence for two distinct lymphoid neoplasms arising from CH. TFH lymphoma cases with antecedent or concomitant hematologic neoplasm often show high variant allelic frequencies of TET2 and often more than one mutation, suggesting a role for surveillance in these patients., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

History and Admission Findings: We report on a 58-year-old patient with weight loss, weakness and polyarthralgia. She reported of recurrent drops, painful joints and muscle weakness., Investigations: The patient had swollen joints, clubbing and cachexia. The radiogram of the hands and feet showed a hypertrophic oseoarthropathy. In further investigations we found a tumor in the upper left lung. The histology findings showed a adenocarcinoma of the lung., Diagnosis, Treatment and Course: The diagnosis of a secondary hypertrophic osteoarthropathy (Pierre Marie-Bamberger syndrome) due to a adenocarcinoma of the lung was made. The patient refused a radiochemotherapy and decided to go on vacation. Further course is unknown., Conclusions: Arthralgia is a common symptom for medical consultation. Pierre Marie-Bamberger syndrome is a rare cause of paraneoplastic arthritis. Notice that unspecific symptoms like weakness, weight loss and arthritis may result from paraneoplastic tumor disease., (© Georg Thieme Verlag KG Stuttgart · New York.)