FIP1L1-PDGFRA Clonal Hypereosinophilic Syndrome With Eosinophilic Myocarditis and Intracardiac Thrombus.

A 45-year-old man from El Salvador with no past medical history presented with cough and chest pain. Investigations revealed 60% peripheral eosinophilia (absolute count 12.3 K/uL). Cardiac imaging was consistent with myocarditis with intracardiac thrombus formation. Endomyocardial biopsy confirmed eosinophilic infiltration of the myocardium, and bone marrow biopsy showed hypercellular marrow with 28% eosinophils. Cytogenetics/fluorescence in situ hybridization (FISH) confirmed positive FIP1L1-PDGFRA rearrangement. The patient was treated for FIP1L1-PDGFRA clonal hypereosinophilic syndrome with associated eosinophilic myocarditis and intracardiac thrombus. The treatment regimen consisted of a steroid taper, imatinib, and anticoagulation. Treatment was followed by normalization of the eosinophil count. At two-year follow-up, the patient was without recurrence of eosinophilia on maintenance imatinib and indefinite anticoagulation with warfarin.

Mortality rate in rheumatoid arthritis-related interstitial lung disease: the role of radiographic patterns.

BACKGROUND: To assess mortality rate (MR) and standardized mortality rate (SMR) of rheumatoid arthritis-related interstitial lung disease (RA-ILD) patients and to evaluate the role of radiographic patterns in mortality. METHODS: A longitudinal multicentric study was conducted in RA-ILD patients from 2005 to 2015 and followed-up until October 2018 in Madrid. Patients were included in the Neumologia-Reumatología y Enfermedades Autoinmunes Registry, from diagnosis of ILD. The main outcome was all-cause mortality. The radiographic pattern at baseline [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), or others] was the independent variable. Covariables included sociodemographic and clinical data. Survival techniques were used to estimate MR, expressed per 1000 persons-year with their 95% confidence intervals [CI]. Cox multiple regression model was run to examine the influence of radiographic patterns on survival. SMR [CI] was calculated comparing MR obtained with MR expected in the general population of Madrid by indirect age-gender standardization. RESULTS: 47 patients were included with a follow-up 242 patients-year. There were 16 (34%) deaths, and most frequent causes were acute ILD exacerbation and pneumonia. MR was 64.3 [39.4-104.9], and 50% of the patients died at 8.3 years from ILD diagnosis. After adjusting for confounders, (UIP compared to NSIP was associated with higher mortality risk. The overall SMR was 2.57 [1.4-4.17]. Women of 60-75 years of age were the group with the highest SMR. CONCLUSIONS: RA-ILD is associated with an excess of mortality compared to general population. Our results support that UIP increases the risk of mortality in RA-ILD, regardless other factors.

Lower 24-Month Relative Survival among Black Patients with Non- Hodgkin's Lymphoma: An Analysis of the SEER Data 1997-2015.

Background: Recent progress in the therapies used in patients with Non- Hodgkin's lymphoma has improved survival. The incidence has been reported to be decreasing in the last few years, accounting for 4% of all cancers. This study analyzed time trends for incidence, mortality, and prevalence of NHL. Methods: We analyzed the SEER Cancer Database from 1997 to 2015. Join point regression analysis was used to determine age-adjusted incidence rates, 24-month relative survival rate, and to identify racial/ethnic groups with a lower survival. Results: The trend in incidence of NHL decreased between 2008 and 2011 at an annual percentage change rate of 3.74%. The male predominance among NHL patients between 1997-2015 was 57%. The number of male patients affected with NHL has been similar in the last 20 years. Female predominance with NHL was higher in 1998 at 46 %, and lower in 2010 at 42.85%. The 24-month relative survival rate was higher among white patients as compared to black patients with NHL. Conclusions: Our analysis demonstrated that the incidence of Non-Hodgkin's Lymphoma has decreased among minorities; however, the outcomes are inferior in terms of survival. This analysis showed an inferior 24-month relative survival rate among black patients compared with white patients. This analysis demonstrates the need for further research in NHL to determine the biological differences and social factors that influence the lower survival among black patients with NHL.

Serum microRNAs as Tool to Predict Early Response to Benralizumab in Severe Eosinophilic Asthma.

Severe eosinophilic asthma poses a serious health and economic problem, so new therapy approaches have been developed to control it, including biological drugs such as benralizumab, which is a monoclonal antibody that binds to IL-5 receptor alpha subunit and depletes peripheral blood eosinophils rapidly. Biomarkers that predict the response to this drug are needed so that microRNAs (miRNAs) can be useful tools. This study was performed with fifteen severe eosinophilic asthmatic patients treated with benralizumab, and serum miRNAs were evaluated before and after treatment by semi-quantitative PCR (qPCR). Patients showed a clinical improvement after benralizumab administration. Additionally, deregulation of miR-1246, miR-5100 and miR-338-3p was observed in severe asthmatic patients after eight weeks of therapy, and a correlation was found between miR-1246 and eosinophil counts, including a number of exacerbations per year in these severe asthmatics. In silico pathway analysis revealed that these three miRNAs are regulators of the MAPK signaling pathway, regulating target genes implicated in asthma such as NFKB2, NFATC3, DUSP1, DUSP2, DUSP5 and DUSP16. In this study, we observed an altered expression of miR-1246, miR-5100 and miR-338-3p after eight weeks of benralizumab administration, which could be used as early response markers.

Retrospective Analysis of Patients with Gynecological Uterine Sarcomas: Leiomyosarcomas and other Histological Subtypes at a Single Institution from 1996 to 2015.

Uterine sarcomas are a rare group of malignancies that account for less than 10% of all uterine malignancies. They are histologically diverse and fall into two broad groups: mesenchymal and epithelial tumors. The treatment in both these groups is marked by high failure rates and quick progression of disease. Patients with stage I to II with resectable disease benefit from operative cytoreduction. Those with advanced stages, benefit from chemotherapy with or without external beam radiation therapy. Our research in this paper looks at the number of LMS cases at our institution, Wyckoff Heights Medical Center in Brooklyn, NY for a period of 20 years from 1996 until 2015 and assesses our cohort's age at diagnosis and their survival in accordance to grade and stage of diagnosis. Our findings suggest that disease stage is a strong prognostic factor with good survival rates in stage I and II, with higher incidence in African-American women. All LMS patients with distant metastasis died within five years.

Prospective evaluation of a transfusion policy of D+ red blood cells into D- patients.

BACKGROUND: Although D- patients should receive red blood cells (RBCs) from D- donors, the scarcity of D- blood components in certain situations makes the transfusion of D+ RBCs unavoidable. Therefore it is recommended that guidelines be developed in order to standardize transfusion policy in these scenarios. STUDY DESIGN AND METHODS: We have prospectively evaluated a policy for the use of D+ RBCs in 905 D- patients. The amount of D- RBCs saved as well as the incidence of hemolytic reactions and anti-D alloimmunization were assessed. RESULTS: 554 patients received D- RBCs while 351 received a total of 1032 D+ RBCs, all of them within our criteria for the acceptable use of D+ RBCs. This strategy allowed us to save 25.6 percent of D- RBCs (1032 out of 4024 RBCs requested). No hemolytic reactions were reported. The incidence of alloimmunization was 21.4 percent. Most patients who developed anti-D did so within the first 2 or 4 RBCs transfused (64% after the first 2 RBCs transfused and 88% after the first 4). In multivariate analysis the age of less than 77 years was the only predictor for alloimmuization (HR = 2.48 [95% CI = 1.21-3.81]; p = 0.014). CONCLUSION: The use of D+ RBCs in selected D- patients does not induce adverse reactions and allows the saving of a significant number of D- RBCs.

The composition of leukapheresis products impacts on the hematopoietic recovery after autologous transplantation independently of the mobilization regimen.

BACKGROUND: Effects of mobilization regimen on the composition of leukapheresis products (LPs) and on hematopoietic reconstitution after autologous peripheral blood progenitor cell transplantation (PBPCT) are not well known. STUDY DESIGN AND METHODS: The effects of three different mobilization regimens--stem cell factor (SCF) plus granulocyte colony stimulating factor (G-CSF) plus cyclophosphamide (CCP), G-CSF alone, and G-CSF plus CCP--on the composition of LPs from patients with nonhematologic PBPC malignancies compared to LPs from G-CSF-mobilized healthy donors and normal marrow (BM) samples were analyzed. The impact of LP composition on both short- and long-term engraftment after autologous PBPCT was also evaluated. RESULTS: The most effective regimen for mobilization of CD34+ hematopoietic progenitor cells (HPCs) into peripheral blood was SCF, G-CSF, and CCP, providing the highest numbers of all CD34+ HPCs subsets analyzed. Patients mobilized with SCF plus G-CSF plus CCP showed the highest numbers of neutrophils and monocytes, whereas the highest numbers of lymphocytes and NK cells were observed in LPs from G-CSF-mobilized patients. The overall number of CD34+ HPCs was the strongest factor for predicting recovery of platelets, whereas the number of myelomonocytic-committed CD34+ precursors was the most powerful independent prognostic factor for WBC and neutrophil recovery. The overall number of CD4+ T cells returned showed an independent prognostic value for predicting the occurrence of infections, during the first year after transplant. CONCLUSIONS: The use of different mobilization regimens modifies the overall number of CD34+ HPCs obtained during leukapheresis procedures, and also affects both the absolute and the relative composition of the LPs in different CD34+ and CD34- cell subsets.