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Primary central nervous system lymphomas (PCNSLs) classically remain confined within the CNS throughout their evolution for unknown reasons. Our objective was to analyse the rare extracerebral relapses of PCNSL in a nationwide population-based study. We retrospectively selected PCNSL patients who experienced extracerebral relapse during their follow-up from the French LOC database. Of the 1968 PCNSL included in the database from 2011, 30 (1.5%, median age 71 years, median KPS 70) presented an extracerebral relapse, either pure (n = 20) or mixed (both extracerebral and in the CNS) (n = 10), with a histological confirmation in 20 cases. The median delay between initial diagnosis and systemic relapse was 15.5 months [2-121 months]. We found visceral (n = 23, 77%), including testis in 5 (28%) men and breast in 3 (27%) women, lymph node (n = 12, 40%), and peripheral nervous system (PNS) (n = 7, 23%) involvement. Twenty-seven patients were treated with chemotherapy, either with only systemic targets (n = 7) or mixed systemic and CNS targets (n = 20), 4 were consolidated by HCT-ASCT. After systemic relapse, the median progression-free survival and overall survival (OS) were 7 and 12 months, respectively. KPS > 70 and pure systemic relapses were significantly associated with higher OS. Extracerebral PCNSL relapses are rare, mainly extranodal, and frequently involve the testis, breast, and PNS. The prognosis was worse in mixed relapses. Early relapses raise the question of misdiagnosed occult extracerebral lymphoma at diagnostic workup that should systematically include a PET-CT. Paired tumour analysis at diagnosis/relapse would provide a better understanding of the underlying molecular mechanisms., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The infiltration of tumors by lymphocytes is a prognosis factor in colorectal cancer (CRC). The magnitude and quality of this infiltration have emerged as important component of the clinical outcome in these patients. Specifically, markers associated with functional cell-mediated immunity, i.e., a Th1 immune response, are independent markers of better prognosis, whereas Th17-associated components are deleterious and correlate with poorer survival. Mucosal-associated invariant T (MAIT) cells are a recently described T cell subset with tissue-homing properties. They display a restricted TCR repertoire specific for widely conserved microbial ligands, and display anti-bacterial properties upon release of Th1-like, Th17-like, and/or cytotoxic granules. MAIT-cell-specific transcripts have been found in kidney and brain cancer, but have not been studies in other sites. In this study, we retrospectively analyzed by confocal microscopy the presence of MAIT cells within colorectal tumors as compared with paired healthy tissues. We observed a significant although variable increase, both in density and in proportion of overall tumor-infiltrating T lymphocytes inside the tumors. Importantly, survival curves as well as multivariate analysis showed that patients displaying a higher recruitment of MAIT cells in their tumor, as compared with the neighboring healthy tissue, showed a less favorable clinical outcome. This study suggests that including MAIT-cell-specific markers or transcripts in the analysis of tumor-infiltrating lymphocytes could be a benefit to the diagnosis and follow-up of CRC patients.

Crohn's disease (CD) has been occasionally reported to be associated with myelodysplastic syndromes (MDS) or acute myeloid leukaemia (AML). From October 1991 to June 1993, four AML patients with a previous history of CD but no history of MDS were referred to our institution. Three presented AML with a monoblastic proliferation (one AML4 and two AML5). Cytogenetic analysis revealed a normal karyotype in two patients, an inv(16) in another and a del(11)(q24-25) in the fourth. Interestingly, the three patients with AML4/AML5 had not received high doses of immunosuppressive agents, prior CD therapy ranging from 1 to 4 months sulfasalazine with additional azathioprine in one case.

In the present paper, we report two cases of normal pregnancy after high dose chemotherapy and autologous stem cell transplantation (ASCT) in two of the 72 women belonging to a group of 188 patients transplanted in our unit for advanced malignant lymphoma. These pregnancies occurred 19 and 33 months after high dose therapy in two women aged 27 and 28, neither of whom had received previous total body irradiation or pelvic radiotherapy. There are several reasons for the relatively low frequency of pregnancies after ASCT. In particular, the median age of the patients is 35 years, most of these women are transplanted in relapse and have received previous pretreatment with alkylating agents and the disease free survival rate does not exceed 40%. The preservation of long term fertility should be taken into account when deciding therapeutic options for young women with curable disease.

Following treatment for mediastinal lymphoma, residual masses are defined as a mass greater than 2 cm observed on the CT scan in the absence of other evolutive signs of lymphoma. In this study, we examined 55 patients with residual mediastinal mass after optimal therapy, using gallium scan (37 cases) or magnetic resonance imaging (MRI, 44 cases). The group comprised 41 subjects with Hodgkin's disease and 14 with non Hodgkin's lymphoma, stages I and II (32 cases) and stages III and IV (23 cases); 35 subjects (64%) having bulky mediastinal involvement at diagnosis. A negative gallium scan or fibrotic signal on MRI was correlated with complete remission in 48 cases (87%). In seven of these 48 patients, MRI was not conclusive with a high signal of indeterminate tissue following radiotherapy, but four of the seven had fibrotic tissue on biopsy and none relapsed. Positive gallium scan was observed in six cases. In conclusion, we suggest performing a gallium scan at the end of induction chemotherapy and when it is negative, treatment may be continued without surgical biopsy or salvage therapy. MRI is of value when it shows fibrotic tissue but can be inconclusive.

In a recent prospective study of allogenic bone marrow transplantation we reported that decreases in factor VII and protein C were predictive markers for high risk of veno-occlusive disease (VOD). In order to determine the relative involvement of endothelial and hepatocyte injury in the genesis of VOD, 34 consecutive patients undergoing autologous bone marrow transplantation (BMT) were studied. Conditioning was performed by chemotherapy alone or associated with total body irradiation (TBI). Protein C and factor VII, the endothelial markers Von Willebrand factor (vWF and t-PA, fibrinogen and fibronectin were measured weekly before and after BMT. Protein C and factor VII were within the normal range before BMT, decreased significantly on day 7 to 73 and 64% respectively (p < .01) and then returned to normal values. Fibrinogen increased to 7 g/l (p < .001) on day 7 but then returned to normal levels. Fibronectin was abnormally high (p < .001) before BMT and decreased thereafter, while vWF increased (p < 0.001) for three consecutive weeks. t-PA was low (p < 0.001) before conditioning but increased thereafter. These results demonstrate the presence of endothelial lesions before BMT and acute hepatic and endothelial lesions after conditioning. Although VOD was never observed in our patients, this complication could well arise from preexisting vascular lesions due to previous chemotherapy and/or from acute hepatocytic injury, which could also be of endothelial origin, after conditioning.

Extra medullary plasmocytoma (EMP) is a plasma cell proliferation of low frequency as compared to multiple myeloma which develops in the upper digestive and respiratory tracts and sometimes in the digestive tract. Other sites are possible but pancreatic or hepatic localizations are unusual with few cases being reported in the literature. This study describes an exceptional observation of pancreatic followed by hepatic plasmocytomas occurring after a long evolution of recurrent EMP. A review of the literature shows that the usual forms of treatment are surgery and radiotherapy. In our patient, this was possible for the pancreatic plasmocytoma, whereas the hepatic lesion was first treated with general polychemotherapy because of the tumour size and adverse prognostic factors. Surgery was then followed by recombinant interferon alpha therapy to obtain complete and hopefully prolonged remission.

Despite combination of chemotherapy and radiotherapy, some patients with refractory or relapsed Hodgkin's disease (HD) have a bad prognosis. We performed high dose chemotherapy (HDCT) (BCV regimen) with autologous bone marrow transplantation (ABMT) in 26 patients with advanced HD. 11 patients were considered refractory after at least two non cross-resistant chemotherapy regimens and radiotherapy in 8 cases. 15 patients had a poor prognosis relapse (in visceral or irradiated site) after complete remission and they received chemotherapy before ABMT. At the time of transplantation 4 patients had progressive disease with B symptoms and did not respond to HDCT; they all died 3 to 12 months post-transplantation from persistent HD. For the remaining 22 patients, 3 months after HDCT 19 patients (73%) were in complete remission, 2 (8%) in partial remission and one patient died from toxicity. After complete remission 4 patients relapsed at 12 to 18 months and 15 patients are in persistent remission 24 to 46 months after transplantation. These results are encouraging in patients with bad prognostic factors but able to respond to chemotherapy.

A man was admitted for severe autoimmune hemolytic anemia with a mediastinal mass. He required corticosteroid treatment, which altered the first histological examination. Laparosplenectomy was performed and all histological sections were negative, but the hemolytic anemia was not improved. Finally, thoracotomy was performed when the mediastinal mass increased after reducing the corticosteroid treatment. Pathological examination revealed Hodgkin's disease associated with Castleman's disease. After chemotherapy and radiotherapy, the patient remains in complete remission.