A 10-year-old boy had chronic diarrhea, abdominal pain, severe weight loss and hepatomegaly; multiple enlarged para-aortic and mesenteric lymph nodes. Mycobacterium fortuitum-chelonae complex was identified in the culture of the lymph nodes. Interleukin-12 receptor beta 1 expression could not be observed in phytohemagglutinin-driven T cell blasts. A homozygous missense interleukin-12 receptor beta 1 mutation was found (R173P).
Leydig cell tumors of the testis are rare, mostly presenting as a testicular mass or as endocrinological symptoms. Here, three patients who were admitted for investigation of primary infertility and one patient presenting with a testicular mass are reported. The histological features were reviewed and an immunohistochemical study was done using a panel of antibodies against cytokeratin, vimentin, inhibin A, S-100, Ki-67, follicle-stimulating hormone, luteinizing hormone, prolactin, p53, bcl-2, and c-erbB2. The latter case (lost during follow up of metastatic disease) demonstrated massive tumor necrosis, extension through the tunica albuginea, and a high mitotic activity and MIB-1 score. Only this malignant case was bcl-2 positive. Of the two oncogenic markers studied, none of the cases were positive for c-erb2, while p53 was positive in more than 50% of cells in the malignant case and in one case of infertility with a large tumor, hemorrhage, focal necrosis and atypical cytological features. We recommend the evaluation of infertile men for Leydig cell tumors, and we believe that a panel of antibodies, including Ki-67, p53 and bcl-2, used for immunohistochemical analysis could be of diagnostic value in the identification of malignant and borderline cases of Leydig cell tumor.
Various racial and geographic differences have been observed in studies questioning the role of Epstein-Barr virus (EBV) infection in the etiology of T-and NK-cell lymphomas. The aim of this study was to evaluate the relationship of EBV with nodal or extranodal (skin excluded) T-and NK-cell lymphoma subtypes encountered in our geographic area. Sixty-two cases of peripheral T-cell lymphoma were included in the study. EBV-encoded early RNA (EBER) was detected by in situ hybridization. The distributions of T-and NK-cell lymphoma subtypes were as follows: 32 peripheral T-cell lymphomas, unspecified (PTCL, NOS), 13 anaplastic large-cell lymphomas (ALCL), 8 angioimmunoblastic T-cell lymphomas (AIT-CL), 4 extranodal NK/T-cell lymphomas, nasal type (NKTCL), 3 enteropathy-type T-cell lymphomas (ETTCL), 1 hepatosplenic T-cell lymphoma (HSTCL), and 1 subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Using a cut-off value of > 25% of EBER-positive neoplastic lymphoid cells, EBV was positive in 22.6% of all cases. According to subtype, the neoplastic cells of 31.3% of PTCL, NOS and 100% of extranodal NKTCL, nasal type were EBER positive, whereas some cases of ALCL, AITCL, and ETTCL presented EBER-positive non-neoplastic cells, and all cells of HSTCL and SPTCL were EBV negative. Extranodal NKTCL, nasal type, presented the strongest association with EBV, followed by PTCL, NOS.
Nasopharyngeal carcinomas (NPC) are epithelial neoplasms which show a distinct geographical distribution and have a characteristic histology. These tumors have multifactorial etiology, including virological, environmental, and genetic components. The aim of the present study is to assess the relation between Epstein-Barr-virus (EBV) and subtypes of NPC in Aegean Turkish patients. In the present study, nasopharyngeal biopsies of 84 cases diagnosed as nasopharyngeal carcinoma, between 1998 and 2004, were reevaluated. In situ hybridization with the fluorescein-conjugated EBV-encoded small nuclear RNA (EBER) oligonucleotide probe was performed on paraffin-embedded tissue sections using an automated slide stainer system. Of 84 patients, 55 were men and 29 were women with ages ranging between 7 and 77 years (median 50, mean 46.73). Seventy-three of 84 cases were EBER positive. All of 62 cases (100.0%) with undifferentiated carcinoma, 8 of 16 (50.0%) with differentiated nonkeratinizing carcinoma, and three of six (50.0%) with keratinizing squamous cell carcinoma were EBV positive. EBER positivity was statistically significantly higher in undifferentiated carcinomas, compared to the other morphological subtypes (p = 0.000). Our results showed that all morphological subtypes of NPC are highly associated with EBV latent infection in our region, and a higher prevalence was found for the undifferentiated subtype.
Objective To evaluate, in patients with unobstructive azoospermia, the heterogeneity of spermatogenesis within the testes and thus whether there is any region of advanced spermatogenesis. Patients and methods Seventy infertile men (mean age 34 years, SD 5.01) with no varicoceles or testicular atrophy had bilateral open testicular biopsies taken from six different sites. For each biopsy specimen the number of seminiferous tubules and of tubules with sperm maturation were counted (by light microscopy at r 400). The ratio of tubules with active spermatogenesis to the total number was calculated for each biopsy sample. Results The mean (SD) right and left testicular volumes were 19.82 (7.8) and 18.84 (7.89) mL, respectively; the patients' follicle-stimulating hormone level was 8.34 (1.17) IU/mL. On sextant biopsy spermatozoa were detected in 42 of the 70 patients (60%). The mean (SD) ratio of tubules with spermatozoa was 5.23 (0.8)% for the right and 5.37 (0.76)% for the left testes. There was no statistically signi®cant difference in the ratio of seminiferous tubules positive for spermatozoa at the different biopsy sites in either the right or left testis. Spermatozoa were identi®ed in only one to three biopsy sites in almost half of those with maturation arrest; this ratio increased to 74% in patients diagnosed as having Sertoli-cell-only syndrome with focal spermatogenesis. Conclusion There is no region of the testis that is rich or advanced in spermatogenesis in patients with unobstructive azoospermia. Without multiple testicular biopsy it is possible to miss advanced spermatogenesis in some unobstructed patients. The sextant testis biopsy is a reliable method for detecting the presence and exact location of seminiferous tubules with spermatozoa in patients with unobstructive azoospermia.
Little is understood about the basic biological mechanisms that underlie the reasons for acute transformation in chronic myeloid leukemia (CML). Progression of disease may include inactivation of one or more tumor suppressor genes (TSGs). A widely used methodology for indirectly detecting somatic inactivation of TSGs is searching loss of heterozygosity (LOH) for polymorphic loci located in or near the gene(s) of interest. We aimed to analyze DNA of chronic phase and blastic phase archive material of 15 CML patients for LOH using D1S430, D2S123, D3S1611, D11S29, D14S65, D17S520, BAT 40 markers, the dinucleotide repeat located in the ABL gene and the trinucleotide repeat located in the BCR gene (amplification of the trinucleotide in the BCR gene could not be succeeded). LOH was identified by a %50 lost of one of the alleles intensity. LOH was detected with the ABL dinucleotide repeat and D2S123 marker in two patients and with the D14S65 marker in three patients. The three patients exhibiting LOH at the D14S65 locus, all proceeded through lymphoid blast crisis. The D14S65 marker is located at the 14q32 locus which contains the immunoglobulin heavy chain gene and the TCL1 oncogene. 14q32 abnormalities at the molecular level, may be predictive for lymphoid blast crisis, whether or not they are detectable cytogenetically.
Human amnion, which has been used experimentally and clinically as an ideal bioprosthesis, was used in the present study to wrap the experimentally injured spleens and the technique was compared to splenorrhaphy. Standard splenic injuries were made on 30 male albino rats. 15 of the spleens were repaired by splenorrhaphy and the other 15 were wrapped with sterile human amniotic membrane. Isotope uptakes of the spleens calculated at the 30th postoperative day were similar in both groups (p less than 0.05). But the histopathological examination revealed a better healing in the amnion group with better preservation of the splenic architecture. It was concluded that in heavily injured spleens, amnion wrapping may be an effective alternative to splenorrhaphy.
Myelofibrosis with myeloid metaplasia, or agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by aniso‐ and poikilocytosis, leukoerythroblastosis and hepatosplenomegaly with extramedullary hematopoiesis. Agnogenic myeloid metaplasia is very rare in children. In this report, two cases of AMM in whom the onset of the illness were at 3 and 12 months of age, are presented. Both had severe anemia, hepatosplenomegaly and bone marrow fibrosis. Lymph node biopsy of the first patient and liver biopsy of the second revealed extramedullary hematopoiesis. They were treated with an intravenous high dose of methylprednisolone (daily 30 mg/kg for 3 days, 20 mg/kg for 4 days, 10 mg/kg for 1 week, 5 mg/kg for 1 week). A complete improvement of hematological and clinical findings was observed.
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