ABSTRACT
Background: Differential diagnosis of chronic lymphoproliferative disorders (CLDs) has remained challenging due to the highly variable morphology features and immunophenotyping. Currently, the development of multiple-marker panel analyses by flow cytometry has opened a broad way for diagnosis of CLDs. Methods: We analyzed the peripheral blood and bone marrow samples of 131 patients with B-cell CLDs (including 91 chronic lymphocytic leukemia (CLL), 15 atypical CLL, 14 mantle cell lymphoma (MCL), and 11 CD5-/CD10-lymphoma patients) from April 2018 to April 2019, using a panel of specific markers by flow cytometry. Results: Our results indicated that the expression pattern of CD22, CD23, FMC-7, and CD5 allowed us to accurately and differentially diagnose the B-CLL, MCL, and CD5-/CD10- lymphoma, while it was not capable of differentiating MCL from atypical CLL. We, however, found that the expression patterns of CD38 and immunoglobulin light chain differed significantly between atypical B-CLL and MCL. CD38 and lambda light chain were remarkably expressed in MCL patients (92.8% and 85%, respectively) compared to the atypical CLL (1.1% and 0% respectively), with the p value less than 0.001 for both markers. In contrast to MCL patients, all the patients with atypical CLL, expressed kappa light chain. The immunohistochemistry method used for cyclin D1 confirmed that the flow cytometry detection of kappa and lambda light chains could provide a new approach with high sensitivity (91%) and moderate specificity (50%) to distinguish MCL patients from atypical B-CLL. Conclusion: Expression of CD5, CD20 (bright), CD22, FMC-7, CD38, and lambda light chain with no expression of CD23 can accurately detect MCL and differentiate it from atypical B-CLL
Subject(s)
Leukemia, Lymphocytic, Chronic, B-Cell , Lymphoma, Mantle-Cell , Adult , Humans , Leukemia, Lymphocytic, Chronic, B-Cell/diagnosis , Leukemia, Lymphocytic, Chronic, B-Cell/metabolism , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Lymphoma, Mantle-Cell/diagnosis , Lymphoma, Mantle-Cell/metabolism , Lymphoma, Mantle-Cell/pathology , Diagnosis, Differential , Flow Cytometry/methods , Immunohistochemistry , ImmunophenotypingABSTRACT
Background: Primary cytoreduction surgery followed by chemotherapy is the cornerstone treatment for epithelial ovarian cancer (EOC). In patients with a low probability of optimal primary surgical debulking, neoadjuvant chemotherapy (NACT) followed by interval debulking increases the chance of optimal surgery. The aim of this study was to develop a model to identify preoperative predictors for suboptimal cytoreduction. Methods: Medical records of patients with EOC who underwent primary cytoreductive surgery in a referral tertiary gyneco-oncology center were reviewed from 2007 to 2017. Data were collected on a range of characteristics including demographic features, comorbidities, serum tumor markers, hematologic markers, preoperative imaging, surgical procedures, and pathologic reports. Univariate and multivariate analyses were performed to clarify the ability of preoperative factors to predict suboptimal primary surgery. Results: The majority of patients (71.3%) who underwent primary cytoreductive surgery were optimally debulked. Based on the Youden index, the best cut-off point for the serum CA125 level to distinguish suboptimal debulking was 420U/ml with 0.730 (95%CI:0.559 to 0.862) sensitivity and 0.783 (0.684 to 0.862) specificity. Multiple logistic regression results showed that serum CA125 level >420 U/ ml (p value <0.001), the presence of liver metastasis on preoperative imaging (p value: 0.041) and ascites (p value: 0.032) or massive ascites (p value:0.010) significantly increased the risk of suboptimal debulking (logit p = 2.36 CA125 level +1.85 Liverinvolvement +1.68 presence of Ascites+ 2.28 Massive Ascites). Conclusion:The present study suggests that a serum CA125 level >420 U/ml, the presence of ascites or massive ascites and liver metastasis are strong predictors of suboptimal primary surgery in cases of EOC. Based on the constructed model, with any of these 4 factors, the probability of suboptimal debulking in EOC is more than 80%.
Subject(s)
Ascites/pathology , Biomarkers, Tumor/analysis , Cytoreduction Surgical Procedures , Models, Statistical , Neoadjuvant Therapy , Neoplasm Recurrence, Local/pathology , Neoplasms, Glandular and Epithelial/pathology , Ovarian Neoplasms/pathology , Ascites/metabolism , CA-125 Antigen/metabolism , Carcinoma, Ovarian Epithelial , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Middle Aged , Neoplasm Recurrence, Local/metabolism , Neoplasm Recurrence, Local/therapy , Neoplasms, Glandular and Epithelial/metabolism , Neoplasms, Glandular and Epithelial/therapy , Ovarian Neoplasms/metabolism , Ovarian Neoplasms/therapy , Prognosis , ROC CurveABSTRACT
Fanconi syndrome is a metabolic disorder involving dysfunction of the renal proximal tubules, resulting in excessive urinary excretion of several metabolites. Various factors may lead to Fanconi syndrome, as it may be a genetic disease with primary or secondary etiologies, or may be acquired. In this study, we report a unique case of Fanconi syndrome with development of a relatively rare acute leukemia, a condition that has not been reported before. The case was an 8-year-old boy with familial occurrence of Fanconi syndrome, presenting with pallor, asthenia, recurrent infections, growth failure, and a variety of biochemical and hematological abnormalities. After physical examination, radiographic studies, and comprehensive laboratory analyses, Fanconi syndrome associated with bilineal acute leukemia, of myeloid and T-lymphoid lineages, was diagnosed.
