Patients can develop malignancies due to various reasons including genetic factors, chemical carcinogens, radiation, and defects in their immune system. The immune system is postulated to carry out routine surveillance for malignancy. Patients who have defective immune responses may be susceptible to malignancies due to complicated underlying mechanisms. These include defective immune response to cancer-causing bacteria, transforming viruses, and concomitant molecular, cellular and immunoregulatory defects. Common variable immune deficiency (CVID) is characterized by hypogammaglobulinemia, impaired antibody responses and an increased susceptibility to infections. A disorderly immune response, or immune dysregulation, may also lead to autoimmune complications and possibly to malignancy. The treatment of CVID involves infusion of replacement doses of immunoglobulin, either intravenously (IGIV) or subcutaneously (SCIG). However, it is unclear whether adequate replacement of immunoglobulins is sufficient to prevent the increased risk of malignancy seen in this disease. We present two cases of unusual solid tumors complicating CVID treated with adequate doses of intravenous immunoglobulins. In this study we review the occurrence of malignancy in patients with CVID and postulate mechanisms that may be involved indigent to this disease. We will also review the role of replacement immunoglobulin and discuss cancer screening in these high risk individuals.

京都大学 / 0048 / 新制・課程博士 / 博士(医学) / 甲第17780号 / 医博第3806号 / 新制||医||999(附属図書館) / 30587 / 京都大学大学院医学研究科医学専攻 / (主査)教授 妻木 範行, 教授 武藤 学, 教授 松田 道行 / 学位規則第4条第1項該当 / Doctor of Medical Science / Kyoto University / DFAM

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Although intestinal involvement occurs in more than half of the cases with KS that are HIV positive, it is uncommon in the classical form, as it occurs in approximately 10% of the patients. We present the case of a 60-year-old male patient with a one-year disease time characterized by having violaceous lesions on the feet and the hands, slightly pruritic and 2 months of epigastralgia and constipation with weight loss of approximately 12 percent of his total body weight. In the physical examination multiple violaceous papule-like lesions are shown on the hands and the feet, some coalescing to form plaques. Laboratory tests revealed a mild normocytic normocytic anemia, the serology for viral hepatitis B and C was negative, HIV negative and ELISA test too. An upper endoscopy was performed and multiple maculopapular and erythematous-violaceous lesions were observed in the esoph-agus, the stomach and the duodenum. In the colonoscopy, multiple lesions with similar characteristics in the ileum, throughout the colon and in the rectum were recognized. The biopsy result was compatible with the KS in all lesions and it was confirmed with the positive HVV-8 immunohis-tochemistry. This case highlights the likelihood of presenting GI SK in elderly patients with gastrointestinal compromise and cutaneous findings, HIV negatives as well as the need to realize an adequate discarding by performing endoscopic studies with the biopsies to optimize treatment. / Revisión por pares

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The Procollagen-Lysine, 2-Oxoglutarate 5-Dioxygenase (PLOD) family contains three members: PLOD1, PLOD2 and PLOD3. The PLOD family catalyze the lysyl hydroxylase (LH) which plays a crucial role in the synthesis of collagen. As one of the most important components in ECM, collagen plays a crucial role in normal tissues. Soft tissue sarcomas (STS) are malignant tumors with more than 100 subtypes which origin from mesenchymal tissue. Although the STS is a rare malignancy accounting for less than 1% of all adult tumors, it has been reported to be responsible for 20% of all cancer-related deaths in childhood and adolescence. In this study, the relation between PLOD family and STS was analyzed. The overexpression of PLOD family is associated with poor prognosis and the PLOD family seems to be a regulator in TME. The PLOD family could serve as strong novel biomarkers and may be used as a therapy target for personalized treatment in STS.

Changwu Wu's doctoral dissertation with the date of the award decision on the title page. Changwu Wu defended his dissertation on March 21, 2023 and was awarded the Dr.rer.med by the University of Leipzig School of Medicine on March 28, 2023. The dissertation is entitled 'RNA-sequencing-based risk stratification and individualized immunotherapy strategies for soft tissue sarcoma'.

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