Burkitt Leukemia With Precursor B-Cell Immunophenotype and Dual Translocation of t(14;18) and t(8;14) in a Child: Case Report and Review of the Literature.

BACKGROUND: Burkitt leukemia (BL) with the precursor B-cell immunophenotype is a rarely reported condition. The prognosis of such patients is similar to that of classic BL. However, the combination of chromosomal translocations associated with bcl-2 and c-myc rearrangement has a poor prognosis. OBSERVATIONS: An 11-year-old child presented with fever and weakness. Bone marrow aspiration showed morphologically L1 type blasts and flow cytometry analysis was compatible with precursor B-cell immunophenotype. Cytogenetic analysis revealed a combination of t(8;14) and t(14;l8). CONCLUSIONS: The combination of t(8;14) and t(14;l8) can exhibit different immunophenotypical and morphologic features in leukemias.

Follicular lymphoma generating in a patient with non-small cell lung cancer following nivolumab discontinuation.

INTRODUCTION: Immune checkpoint inhibitors have demonstrated the benefit for many cancer types, melanoma, non-small cell lung cancer, urothelial cancer, renal cell cancer, etc. Especially in advanced non-small cell lung cancer, significant improvement in survival results has been shown. CASE REPORT: Here, we report a 66-year-old man with lung adenocarcinoma who received nivolumab for 80 cycles.Management and Outcome: Two months after discontinuing nivolumab, he developed follicular lymphoma. Pneumonitis was also accompanied, which was treated with metilprednisolon, but he died due to progressive respiratory failure. DISCUSSION: Our clinical knowledge with checkpoint inhibitors is increasing day by day, and to the best of our knowledge, this paper presents the first case in the English literature who developed follicular lymphoma after discontinuing nivolumab in non-small cell lung cancer patient.

A clinicopathological experience in acute myeloid leukemia: Effects of clinical data and status of FLT3, CEBPA and NPM1 on prognosis.

Background: The purpose of the study was to analyze the expression of nucleophosmin (NPM1), CCAT/enhancer-binding protein alpha (CEBPA), and FMS-like tyrosine kinase 3 (FLT3) with immunohistochemistry and evaluate the relationship with clinicopathologic data with special emphasis on prognosis in bone marrow biopsy specimens diagnosed with acute myeloid leukemia (AML). Materials and Methods: Bone marrow biopsies of 104 patients who were diagnosed with AML were re-evaluated for diagnosis and subclassification. Immunohistochemically, anti-NPM1, anti-CEBPA, and anti-FLT3 antibodies were applied to slides prepared from formalin-fixed paraffin-embedded tissues. Sixty-three of these patients had their follow-up in our institutional hematology clinic and these patients' clinical, biochemical, and radiological data were obtained and analyzed from patient files. These data were analyzed with survival times statistically. Results: Except for age, no significant effect of clinical data on prognosis was detected. Immunohistochemical results were also statistically compared with clinical data. No correlation was found between overall survival and disease-free survival with the expression of anti-CEBPA or anti-NPM1 antibodies. However, immunohistochemical reactivity for anti-FLT3 antibody was found to be a poor prognostic factor and statistically significant. Also, when the expression of FLT3 was analyzed with that of NPM1 or CEBPA, a correlation (dependent on the expression of FLT3) was found with disease-free survival. Conclusions: FLT3 is an independent prognostic factor for AML. CEBPA and NPM1 should be considered as good prognostic factors only in the absence of FLT3 abnormalities.

Treatment of pediatric Burkitt lymphoma in Turkey.

This study aimed to assess the demographic data and treatment results of children who were diagnosed with Burkitt lymphoma and treated according to the Berlin-Frankfurt-Münster-95 (BFM) protocol in a single institution. A total of 48 patients (37 boys, 77%) with a median age of 8 years (range 2 to 16 years) at diagnosis, were evaluated. Primary tumor sites were abdomen (70.8%), head and neck (22.9%), peripheral lymph node (2%), bone (2%), and testis (2%). The 5-year overall survival (OS) and event-free survival (EFS) were 78.1±4% and 76.6±6%, respectively. In univariate analysis, hemoglobin level less than 10 g/dL, cerebrospinal fluid (CSF) positivity and dialysis requirement at diagnosis were found to be important reverse predictor factors for EFS (P; 0.001, 0.001, 0.004, respectively). In multivariate analysis, hemoglobin level less than 10 g/dL and dialysis at diagnosis were found to be important reverse predictor factors for EFS (P; 0.0001). The EFS of our patients was lower than the values achieved with BFM-95 protocol in other centers. This study provides evidence that low hemoglobin level, CSF positivity and dialysis at diagnosis were important predictor factors for EFS in children with Burkitt lymphoma.

Comparative study of adhesion molecule expression in nodular lesions of Behçet syndrome and other forms of panniculitis.

Adhesion molecules have a role in many vasculitic disorders. Our aim was to evaluate the status of adhesion molecules in nodular lesions of Behçet syndrome (BS) and compare them with results for the 2 most common types of panniculitis, erythema nodosum (EN) and nodular vasculitis (NV). We included the data for 28 patients with nodular lesions of BS, 24 with EN, and 22 with NV. A panel of monoclonal antibodies against E-selectin, P-selectin, vascular cell adhesion molecule-1, platelet endothelial cell adhesion molecule-1, and intercellular adhesion molecule (ICAM)-1 were applied. The distribution and intensity of adhesion molecules were assessed. There were no statistically significant differences between the BS and control groups in regard to these adhesion molecules except for ICAM-1. The percentage of strongly ICAM-1-stained endothelial cells in subcutaneous fat tissue in relation to the total number of endothelial cells was the lowest in BS (P= .0208). Because many lesions of BS were related to an enhanced inflammatory response, the lower percentage of ICAM-1 expression seems counterintuitive.

The effects of somatostatin and ursodeoxycholic acid in preventing the ischemic injury of the liver following Pringle maneuver in obstructive jaundice-rat model.

BACKGROUND/AIMS: In our study, the effects of somatostatin (SS) and ursodeoxycholic acid (UDCA) on ischemic liver injury were studied in (obstructive) jaundice-rat model. METHODOLOGY: For this purpose, jaundice was produced in the first four groups by binding of their choleducts. We performed just laparotomy to the other four groups of animals. To groups 1 and 5, SS was given 15 mcg/kg/day intraperitoneally, and to groups 2 and 6, UDCA was given 20 mg/kg/day enterally. No drugs were given to any other group. At the end of one week, a procedure with ischemia of the liver for 60 minutes followed by reperfusion for 2 hours, was performed to each rat except for groups 4 and 8. Following this procedure, they were sacrificed. The blood samples were taken to measure SGOT, SGPT, ALP, LDH, total and direct bilirubin levels, while liver biopsies were taken for histopathological evaluation. RESULTS: Under normothermic conditions, following 60-minute liver ischemia period, no irreversible histopathological changes were detected. However, increases in liver necrosis parameters were noted biochemically. SS and UDCA were thought to be effective in preventing the injury by decreasing the liver enzymes levels to a significant degree. The damage of the hepatic ischemic injury was found to be more meaningful and prominent in liver with jaundice. CONCLUSIONS: In this study, it was noted that SS and UDCA decrease the effects of cholestatic hepatic injury especially and improve the condition.

Bronchus Associated Lymphoid Tissue Lymphoma Presenting with Immunodeficiency and Multiple Pulmonary Nodules.

Bronchus Associated Lymphoid Tissue Lymphoma (BALTOMA) is a rare subgroup of pulmonary non-Hodgkin's lymphomas (NHLs) comprising less than 1% of all cases. It constitutes 3.6% of all extranodal lymphomas and only 0.5-1% of primary pulmonary malignancies. They are usually low grade B-cell lymphomas and are considered to originate from the mucosa associated lymphoid tissue (MALT) of the bronchi. Here, we represent a rare case of BALTOMA presenting with immunodeficiency and multiple pulmonary nodules.

Coexistence of early onset sarcoidosis and partial interferon-γ receptor 1 deficiency.

Pediatric sarcoidosis comprises a spectrum of childhood granulomatous inflammatory conditions. Pathological hallmark of the disease is granuloma formation that is seen in the affected tissues and almost any organ or system can be involved. There are two forms of pediatric sarcoidosis. One is seen in older children and the clinical picture is very similar to that of adult sarcoidosis and the other one is seen in early childhood. Sarcoidosis in early childhood can be divided as Blau syndrome (familial form) and early onset sarcoidosis (sporadic form). In both of the diseases there is a defect in the NOD2/CARD15 gene. The typical triad of early onset sarcoidosis is polyarthritis, dermatitis and uveitis. Interferon-γ receptor 1 deficiency is caused by defects in the IFNγR1 gene and non-tuberculosis mycobacterial pathogens are the leading causes of infections that start in early childhood. Herein we report a patient who presented with the symptoms of early onset sarcoidosis and also had partial interferon-γ receptor 1 deficiency that presented with BCG-osis. In addition to anti-mycobacterial treatment, methotrexate and prednisolone were used in therapy.

T-cell-rich B-cell lymphoma: a clinicopathologic study of 21 cases and comparison with 43 cases of diffuse large B-cell lymphoma.

Clinicopathologic features of 21 patients with T-cell-rich B-cell lymphoma (TCRBCL) were reviewed and compared to 43 patients with diffuse large B-cell lymphoma (DLBCL) to determine if there were distinguishing clinical characteristics and differences in response or survival to CHOP therapy. For the diagnosis of TCRBCL, the current WHO criteria was used. In all of our cases, the majority of cells are non-neoplastic T cells and <10% large neoplastic B cells are present. The initial pathologic diagnosis was nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) in two cases. Patients with TCRBCL were significantly younger (median: 46 years) and had a significantly higher incidence of B symptoms (62%), hepatomegaly (33%) and marrow infiltration (33%) at presentation when compared to DLBCL (P<0.03). The CR rate after treatment was 48% for TCRBCL patients versus 79% for the DLBCL (P<0.003). Although the CR rates in between the two groups are significant, the difference in 3 years survival rates in each CR groups was insignificant (80% versus 77%). The overall survival time in the two groups was 17 months. Event-free survival time in TCRBCL was 12 months, compared with 17 months in the DLBCL (P>0.05). The frequency of patients with TCRBCL achieving CR was 52.6% whereas that of patients with DLBCL was 79% (P<0.003). The TCRBCL 3 years event-free survival 48% and overall survival 64% were 63 and 72% for DLBCL, respectively.

Typhoid fever as a rare cause of hepatic, splenic, and bone marrow granulomas.

During the course of typhoid fever, the usual histologic finding of the liver is "nonspecific reactive hepatitis." Hepatic granuloma (HG) is a rare complication of typhoid fever. We present two cases of typhoid fever with HG and review the relevant literature. Case 1 (a 53-year-old female) was found to have both hepatic and splenic granulomas. This is the first case of typhoid fever with splenic granulomas in the English language literature. Case 2 (a 66-year-old male) developed granulomas in the bone marrow in addition to HG. It should be considered that typhoid fever may lead to granulomas in several organs.

Expression of MMP-1, MMP-9 and TIMP-2 in prostate carcinoma and their influence on prognosis and survival.

PURPOSE: Matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) participate in tumorigenesis, and their association with disease outcome is highly controversial. The present study investigates the influence of MMP-1, MMP-9 and TIMP-2 on different clinicopathologic variables and disease-free survival (DFS) of patients with prostate carcinoma. METHODS: Hundred and forty-five cases are included in the study, and levels of MMP/TIMP expressions are assessed in three tissue compartments (i.e., tumor, stroma and normal glands) with immunohistochemistry. RESULTS: Matrix metalloproteinase-1 expression in tumor cells was associated with lower Gleason scores, pretreatment prostate-specific antigen levels and lower incidence of vascular, perineural and extracapsular invasions. Moreover, MMP-9 positivity and TIMP-2 expression in normal glands were correlated with lower Gleason patterns and early stage at presentation. Expression of MMP in tumor cells and the presence of TIMP-2 in normal glands were associated with better DFS. CONCLUSION: Variability of MMP/TIMP expressions from case to case makes it difficult to evaluate their impact on clinical outcome. However, these proteins might be new and promising targets for prostate cancer therapy in the future.

Primary central nervous system lymphoma in immunocompetent individuals: a single center experience.

UNLABELLED: Primary central nervous system lymphoma (PCNSL) is defined as the involvement of brain, leptomeninges, eyes or spinal cord by non-Hodgkin lymphoma. The role of various prognostic markers in predicting adverse outcome is debated. OBJECTIVES: To investigate the clinical and immunohistochemical findings of immunocompetent PCNSL cases (39 cases) diagnosed at the study center, and evaluate the influence of potential prognostic factors on overall survival (OS) of patients. METHODS: Data regarding patient characteristics, neuroimaging, pathological and immunohistochemical features and follow-up were obtained from patient records. The influence of potential prognostic parameters on OS was investigated by log-rank test and Cox regression analysis. RESULTS: Patients who received combined chemotherapy and radiotherapy had a significantly better OS when compared to chemotherapy alone. Other variables included in this study were not associated with a significant survival advantage. CONCLUSION: In this study, we failed to demonstrate a relationship between different clinicopathological variables and OS of patients. Prospective studies with large patient series are needed to investigate other potential prognostic factors.

Synchronous Detection of Hairy Cell Leukemia and HIV-Negative Kaposi's Sarcoma of the Lymph Node: A Diagnostic Challenge and a Rare Coincidence.

Hairy cell leukemia (HCL) is an uncommon chronic lymphoproliferative disorder and accounts for around 2% of all forms of leukemias. The association of HCL with other neoplasms, mainly non-Hodgkin's lymphomas, is well known. However, the simultaneous diagnosis of HCL and Kaposi's sarcoma is rare, with only few cases of such an association having been reported. We describe a 42-year-old male patient with a well characterized HCL and in whom HIV-negative Kaposi's sarcoma of the lymph node was detected.

Radiological features in paediatric primary gastric MALT lymphoma and association with Helicobacter pylori.

Primary malignant tumours of the stomach are very rare in children, most being lymphomas and sarcomas. The majority of primary gastric lymphomas are high-grade non-Hodgkin's lymphomas and are of B-cell origin. However, a significant number are low-grade B-cell lymphomas that are derived from mucosa-associated lymphoid tissue (MALT) that is not found in the normal stomach. Helicobacter pylori infection predisposes to the development of MALT in the stomach and provides the pathogenic background for MALT-type lymphomagenesis. To our knowledge, only eight paediatric cases of primary gastric lymphoma have been described. The diagnosis and follow-up of gastric lymphoma are mainly made by endoscopy. Nevertheless, radiologists must be aware of this disease because it may be observed on radiological examinations that are performed for non-specific upper digestive symptoms in children.

Primary granulocytic sarcoma of the urinary bladder: case report and review of the literature.

We report a case of granulocytic sarcoma of the urinary bladder, with no evidence of hematologic involvement. The patient was initially misdiagnosed and was treated with chemotherapy for transitional carcinoma grade 3. Despite this treatment, the clinical features of the patient progressed, and a repeated biopsy yielded the correct diagnosis. Three cases of granulocytic sarcoma of the urinary bladder have been reported in published studies, with only one of these primary. To our knowledge, ours is the second case of granulocytic sarcoma of the urinary bladder presenting with urologic symptoms but without hematologic findings.