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Major Digestive and Endocrine Glands
Published in George W. Casarett, Radiation Histopathology: Volume II, 2019
Reed and Cox222 likened the appearance of the altered hepatic lobules to that of hepatic veno-occlusive disease in man arising under other conditions. They concluded that the vascular lesion was an early result of irradiation and that the hyperemia and hepatic cell loss or atrophy were secondary to the vascular obstruction in the small branches of the hepatic veins. Dose size permitting, there may be sufficient recovery of an effective circulatory system in the damaged regions to lead to considerable restoration of hepatic cells and hepatic architecture, probably by means of the development of collateral circulatory channels. In addition to the progressive fibrous obliteration of the small branches of the hepatic veins, there may also be some thickening of the small portal vein branches and focal intimai thickening of the small arteries.
Approaches to patients with variants in RAG genes: from diagnosis to timely treatment
Published in Expert Review of Clinical Immunology, 2019
Adeeb A. Bulkhi, Joseph F. Dasso, Catharina Schuetz, Jolan E. Walter
With the introduction of NBS for SCID via TRECs that is offered in the US and other countries, early detection of SCID became more prevalent. Several studies have highlighted that HSCT at an early age (<3.5 months) and prior infection exposure have the greatest success rate [35,74]. There is an ongoing discussion whether and which conditioning regimen should be used for these asymptomatic patients. If a matched family donor is available, HSCT can be performed without graft manipulation and conditioning. This approach circumvents toxicity but usually does not result in B-cell engraftment. With alternative donors (MUD or haploidentical), conditioning is highly recommended to avoid graft failure and ensure full immune reconstitution including B cell immunity and long-lasting T cell production. Toxicity in this very young group of SCID patients includes both acute (e.g. hepatic veno-occlusive disease) and long-term toxicities.
Comorbidities, age, and other patient-related predictors of allogeneic hematopoietic cell transplantation outcomes
Published in Expert Review of Hematology, 2018
Verena Wais, Donald Bunjes, Florian Kuchenbauer, Mohamed L. Sorror
Historically, a corrected diffusion capacity of the lung for carbon monoxide (Dlco) of ≥60% was a requirement for eligibility for allogeneic HCT. In a large study including 1297 recipients of HCT (82% allogeneic and 12% autologous), Dlco values <60% were associated with a 1.5-fold increase in post-transplantation mortality [19]. A subsequent study has shown Dlco values of <70% to be associated with a 2.5-fold occurrence of severe post-transplantation hepatic veno-occlusive disease (VOD). However, a subset of patients with a corrected Dlco of <60% could still benefit from allogeneic HCT. Those are typically patients with autoimmune diseases that frequently involve the lungs, e.g. scleroderma [20]. It is challenging to evaluate the functional capacity of the lung in older or medically unfit patients, as there are no specific laboratory parameters except arterial blood gas analyses.
Outcome of two patients with bilateral nephroblastomatosis/Wilms tumour treated with an add-on 13-cis retinoic acid therapy – Case report
Published in Pediatric Hematology and Oncology, 2018
Waltraud Friesenbichler, Wolfgang Krizmanich, Karoly Lakatos, Andishe Attarbaschi, Michael Dworzak, Gabriele Amann, Rhoikos Furtwängler, Norbert Graf, Leo Kager
Patient 2, a 6-months old girl, presented to our hospital with an asymptomatic abdominal mass in June 2009. Imaging studies revealed massively enlarged kidneys with numerous nephrogenic rests bilaterally (Figure 2A). According to the sonographic and MRI appearance, bilateral NBL was diagnosed.11 Initially, NBL was associated with acquired factor VIII deficiency resulting in an increased bleeding tendency. According to the SIOP 2001/GPOH protocol, vincristine and actinomycin-D were administered over 15 months. The patient twice presented with symptoms of hepatic veno-occlusive disease, the second time accompanied by characteristic ultrasound findings. As a consequence, the dose of the chemotherapy was reduced (vincristine 66%, actinomycin-D 45%).