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  • Eriksen Barbee posted an update 1 year, 6 months ago

    Whilst Trisomy 6 is associated with low birth weight and perinatal death, few studies have reported these outcomes in 10q26 deletion syndrome. Our findings therefore contribute to the evidence base regarding unbalanced translocations and may improve the clinical management of such patients.A 62-year-old woman, who had a 16-year history of JAK2V617F-mutated myeloproliferative neoplasm (MPN), developed Burkitt leukemia (BL) 16 months after treatment with ruxolitinib to control hydroxyurea-refractory conditions. BL cells were CD10+, CD19+, CD20-, CD34-, cytoplasmic CD79a+, and TdT+, and lacked surface immunoglobulins but expressed the cytoplasmic μ heavy chain. In the bone marrow, nuclear MYC+ BL cells displaced the MPN tissues. t(8;14)(q24;q32) occurred at a CG dinucleotide within MYC exon 1 and at the IGHJ3 segment, and an N-like segment was inserted at the junction. The V-D-J sequence of the non-translocated IGH allele had the unmutated configuration. DNA from peripheral blood at a time of the course of MPN exhibited homozygous JAK2V617F mutation, while that at BL development included both JAK2V617F and wild-type DNAs. Although the association between JAK1/2 inhibitor therapy for MPN and secondary development of aggressive B-cell neoplasm remains controversial, this report suggests that, in selected patients, close monitoring of clonal B-cells in the BM is required before and during treatment with JAK1/2 inhibitors.Necrotizing lymphadenitis (NEL) is a self-limited systemic disease exhibiting characteristic clinical features. The pathogenesis of the disease remains unclear, but it may be associated with viral infection. In lymph nodes affected by this disease, innumerable plasmacytoid dendritic cells produce interferon-α when triggered by certain viral stimuli. selleck IFN-α presents antigens causing the transformation of CD8+ cells into immunoblasts and apoptosis of CD4+ cells. From the perspective of innate immunity, UNC93B1, an endoplasmic reticulum (ER)-resident protein, associates more strongly with TLR9 than TLR7. Homeostasis is maintained under normal conditions. However, in NEL, TLR 7 was observed more than TLR 9, possibly because mutant type UNC93B1 associates more tightly with TLR7. The inhibitory effects against TLR7 by TLR9 were reported to disappear. It is likely that more TLR7 than TLR9 is transported from the ER to endolysosomes. In conclusion, overexpression of TLR7, an innate immune sensor of microbial single-stranded RNA, is inferred. Consequently, NEL may be induced.Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) has a significantly poor prognosis among secondary HLH. We describe the rare case of a 74-year-old female with secondary HLH presenting with a rapidly fatal course. Post-mortem examination revealed Epstein-Barr virus (EBV) -positive diffuse large B-cell lymphoma (DLBCL). We were unable to make a definite antemortem diagnosis because the patient did not exhibit lymphadenopathy and bone marrow biopsy demonstrated hemophagocytosis without evidence of lymphoma. She died of multiple organ failure on the twelfth day of hospitalization despite a temporary response to steroids. Autopsy revealed diffuse lymphoma cell infiltration of the bone marrow, liver and spleen, suggesting “bone marrow-liver-spleen” (BLS)-type large B-cell lymphoma (LBCL). BLS-type LBCL is a rare and clinically aggressive lymphoma, usually associated with fever, cytopenia and HLH. The disease has a high mortality rate due to the delay in diagnosis and a highly aggressive clinical course. Further studies are required to improve our understanding of this rare extranodal DLBCL.This study aimed to identify the causes of death in dogs that were buried at pet cemeteries. Using data obtained from a questionnaire completed by 5,118 dog owners from 1 April 2016 to 31 March 2017, we estimated the life expectancy and the odds ratio for principal causes of death using multiple logistic regression analysis with potential risk factors as independent variables. The life expectancy determined at birth was 13.98 years. The commonest cause of death was senility. The odds ratios of dying of senility being significantly higher in Labrador Retrievers and Shiba breeds and aged dogs, and significantly lower in Chihuahuas, dogs living indoors and neutered dogs.Leptospirosis is an exacerbating factor responsible for the drastic decline of sloth bear population in India. In this study, a multipronged approach based on antigen detection using Polymerase Chain Reaction (PCR) employing G1/G2 and LigBF/LigBR primers, antibody detection using Microscopic Agglutination Test (MAT) and recombinant LigBCon1-5 antigen based Latex Agglutination Test (rLigBCon1-5 LAT), serum biochemistry using hepatic (serum glutamate oxalo acetic transaminase (SGOT) and serum glutamate pyruvic transaminase (SGPT) and renal biomarkers (blood urea nitrogen (BUN) and Creatinine) and gross/histopathological evidence in liver and kidneys were employed to investigate leptospirosis in captive sloth bears. A total of 133 serum samples collected from Agra (n=113) and Bannerghatta (n=20) sloth bear rescue centers were screened using MAT and rLigBCon1-5 LAT. A total of 87 and 78 sera tested positive by MAT and LAT respectively. Pyrogenes was the leading serovar obtained using MAT followed by Icterohaemorrhagiae, Javanica, Grippotyphosa, Canicola and Tarassovi. The relative sensitivity, specificity and accuracy of rLigBCon1-5 LAT in comparison to MAT were 89.66%, 100% and 93.23% respectively. PCR performed on hepatic and renal tissues showed amplicon of 285 and 219 base pairs for G1/G2 and LigBF/LigBR primers respectively. Gross evidence (icteric liver, severely engorged hepatic sinusoids, congested kidneys with necrotic white spots on sub capsular surface), histopathology (severe hepatic degeneration and tubulointerstitial nephritis) and elevated hepatic/renal biomarkers were suggestive of leptospirosis. This study suggests that rLigBCon1-5 LAT can be employed as a pen-side test for detecting leptospirosis in sloth bears.Pinnipeds have evolved enlarged ocular bulbs to catch fish in the dark. However, their large protruding eyes are easily damaged, which increases the risk of blindness and death in these animals. In captivity, ophthalmic disorders, manifested as keratitis and cataracts, are common among pinnipeds. In this study, we investigated symptoms of ophthalmic disorders in pinniped species using a questionnaire distributed to 32 zoos and aquariums throughout Japan. We conducted this study in cooperation with the Japanese Association of Zoos and Aquariums. The survey included 295 pinnipeds from four otariid species, five phocid species, and one odobenid species. Of these, 43.1% of the pinnipeds had diseases affecting the lens, cornea, and/or other eye parts. Age was positively associated with lens disorders in California sea lions, South American sea lions, and spotted seals. Conflicts and public appearances were also associated with corneal and/or lens disorders in California sea lions. Treatments were evaluated as effective for corneal disorders and conjunctivitis.