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Objectives Determination of bone marrow cellularity is a key part of bone marrow examination because it provides a small window into a patient's current state of hematopoietic well-being. Traditionally, bone marrow cellularity is estimated semiquantitatively through microscopic examination of core biopsy specimens harvested from the iliac crest of the pelvic bone. Bone marrow cellularity is then designated as hypercellular, normocellular, or hypocellular based on the patient's age. This assessment can have significant clinical impact, but the variation in the age-adjusted normocellularity range is not sufficiently characterized because of a lack of study data, especially in older patients (those older than 70 years of age). This study further established the normal range of bone marrow cellularity, particularly in older adults. Methods In this study, 570 benign staging and healthy donor bone marrows from patients 1 year to 93 years of age were analyzed for cellularity. Results Linear regression modeling demonstrates that cellularity in adults declines approximately 3% per decade, including after the seventh decade of life. The 90% reference interval for normocellularity in United States is 30% to 75% for those aged 18 to 90 years. Conclusions The findings revealed a more stable and slower rate of decline in cellularity with age in adults than the widely used linear model of "100% minus the patient age in decades." Normocellularity is better modeled based on age group. In those younger than 20 years of age, normocellularity ranges from 45% to 85% (mean [SD], 65% [20%]), as defined by Friebert et al in 1998. Based on our study finding of a little less than 3% decline per decade of age, the following is our recommendation for normocellularity range: For individuals 20 to 40 years of age, it ranges from 40% to 70% (mean [SD], 55% [15%]); for individuals 40 to 60 years of age, it ranges from 35% to 65% (mean [SD], 50% [15%]); and for individuals older than 60 years of age, it ranges from 30% to 60% (mean [SD], 45% [15%]). Interestingly, those older than 70 years of age do not show a significant decrease from those aged 60 to 69 years. [ABSTRACT FROM AUTHOR]

Chimeric antigen receptor (CAR) T-cell therapy presents a promising treatment for hematologic malignancies, displaying high efficacy but not being exempt from toxicity. In this observational study, we assessed adverse events (AEs) reported to the Food and Drug Adverse Event Reporting System (FAERS) including any of the six approved CAR T-cell therapies. A total of 5249 reports mentioning a CAR T-cell as a suspect product were retrieved from the FAERS database, containing a total of 24333 AEs, of which 3236 (13.3%) were cytopenias. The highest number of AEs mentioned by the report was observed for tisagenlecleucel (mean = 6.7), with the lowest for ciltacabtagene (mean = 1.3). Among all reports, hematopoietic leukopenia was the most frequently reported AEs ( n  = 1386, 5.7%), with hematopoietic erytropenia the least reported ( n  = 291, 1.2%). Tisagenlecleucel showed a high reporting odds ratio for hematopoietic erythropenia (27.28, 95%CI 14.04-53.00), leukopenia (4.04, 95%CI 3.52-4.64), and thrombocytopenia (4.01, 95%CI 3.19-5.03). Cytopenias represent one of the most frequently reported AEs in FAERS, a CAR T-cell therapy is indicated, with haematopoetic leukopenia being the most common. When comparing different CAR-T cell therapies, the cytopenias' reporting odds ratio was particularly high for tisagenlecleucel, especially in relation to hematopoietic erythropenia .

Background: MDS FISH was routinely ordered together with chromosome analysis for patients with cytopenia in our hospital. The utility of MDS FISH in the pediatric population is unknown. Objective: To analyze the utility of fluorescence in situ hybridization panel for myelodysplastic syndrome (MDS FISH) in the management of patients with cytopenia. Methods: We performed a retrospective review over a 5-year period, from 2009 to 2014 to determine whether chromosome analysis (CA) plus MDS FISH added useful information compared to chromosome analysis alone. Both CA and MDS FISH were performed on 253 bone marrow biopsies from 182 patients. Results: CA was highly correlated with MDS FISH (P < .0001) and detected all of the abnormalities seen by MDS FISH in 93.7% of the cases. CA is less expensive and detects additional chromosomal abnormalities not tested in the myelodysplastic syndrome panel. We propose MDS FISH should be ordered when CA fails to give adequate results. [ABSTRACT FROM AUTHOR]

Rationale: Granulomatous-lymphocytic interstitial lung disease (GLILD) has emerged as a major cause of morbidity in patients with common variable immunodeficiency (CVID). While GLILD is among the most serious noninfectious pulmonary complications of CVID, risk factors for this condition have not been reported.Objectives: To identify clinical, physiologic, and serologic risk factors for GLILD in adults with CVID.Methods: Of 345 consecutive adult patients with CVID, we identified 34 in the National Jewish Health research database who had a radiographic-pathologic diagnosis of GLILD evaluated between 2002 and 2014. Each case was age and sex matched to 52 CVID control subjects. We used logistic regression to determine independent predictors of GLILD. A mixed effects model was used to estimate the longitudinal change in percent predicted FVC.Measurements and Main Results: The mean time from CVID diagnosis to GLILD detection was 7.8 years. Compared with matched control subjects, cases were more likely to have a history of autoimmune cytopenia, hypersplenism, polyarthritis, lower marginal zone and switched memory B cells, and restrictive lung function. Multivariate analysis revealed that hypersplenism (odds ratio [OR], 24; 95% confidence interval [CI], 4.5-179.1), polyarthritis (OR, 19; 95% CI, 2.3-206.8), and percent predicted FVC (OR, 0.93; 95% CI, 0.87-0.98) were independently associated with the development of GLILD. The rate of change of percent predicted FVC (slope, P = 0.48) did not vary significantly in patients with GLILD over a mean follow-up of 7 years after diagnosis.Conclusions: Hypersplenism and polyarthritis are strong risk factors for GLILD in patients with CVID. Percent predicted FVC remained stable over time in patients with GLILD. [ABSTRACT FROM AUTHOR]

Background: Polycythemia vera (PV) is associated with increased blood cell counts, risk of thrombosis, and symptoms including fatigue and pruritus. National guidelines support the use of hydroxyurea (HU) in high-risk patients or those with some other clinical indication for cytoreduction., Patients and Methods: REVEAL is a prospective, observational study designed to collect data pertaining to demographics, disease burden, clinical management, patient-reported outcomes, and health care resource utilization of patients with PV in the United States. In this analysis, HU treatment patterns and outcomes were assessed from 6 months prior to enrollment to the time of discontinuation, death, or data cutoff., Results: Of the 1381 patients who received HU for ≥ 3 months, the median HU exposure was 23.6 months (range, 3.1-38.5 months). The most common maximum daily HU doses were 1000 mg (30.6%) and 500 mg (30.1%); only 6.4% received ≥ 2 g/d HU. Approximately one-third (32.3%) of patients had dose adjustments, 23.8% had dose interruptions, and 257 (18.6%) discontinued HU. The most common reasons for HU discontinuations and interruptions were adverse events/intolerance (37.1% and 54.5%, respectively) and lack of efficacy (35.5% and 22.1%, respectively). Of those who received HU for ≥ 3 months, 57.1% had hematocrit values > 45% on ≥ 1 occasion, 33.1% continued to receive phlebotomies, and 27.4% had uncontrolled myeloproliferation., Conclusion: The results of this analysis emphasize the need for active management of patients with PV with appropriate HU dose titration to maintain blood count control while monitoring for signs and symptoms of HU intolerance., (Copyright © 2019 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background Human Immunodeficiency Virus (HIV) patients are at increased risk of opportunistic infections and malignancies. Evaluation for the etiology of fever and/or cytopenia with conventional means such as cultures and serology can remain negative. Bone marrow aspiration or biopsy (BMAB) has the advantage of diagnosing disseminated infections, hematological abnormalities and oncological malignancies in HIV patients. Methods We performed a retrospective descriptive study of HIV patients with fever and/ or cytopenia who underwent Bone marrow aspiration or biopsy (BMAB). Patients with a diagnosis of HIV, 18 years and older who underwent BMAB in University Health (UH) Hospital or in UH clinics from January 2012 to February 2018 were included. Results There were a total of 42 patients who underwent Bone Marrow Aspiration or Biopsy. The median age was 41.5 years. Twenty-eight patients were Male and 14 were female. Preexisting Hematological malignancy was present in 10 patients at the time of BMAB. Average CD4 count at the time of BMAB was 92. 8 patients were compliant with ART and 12 patients were compliant with clinic appointments. White Blood Cell (WBC) count below 4.4 cells / L was present in 30 patients at the time of BMAB. Disseminated Mycobacterium Avium Complex infection (2 patients),Disseminated Histoplasmosis (2 patients), Disseminated Cryptococcus (1 patient) and Parvovirus B19 (based on Immunohistochemistry, 1 patient) were diagnosed from BMAB. CD4 count of these 6 patients range from 0 to 12 at the time of BMAB. All 6 patients presented to the hospital with fever for evaluation. Average WBC count, Hemoglobin and platelet count in these patients are 4.1 cells / liter, 8.7 g/dL and 74.8 k/micro liter, respectively. All 6 patients were non compliant to HIV medications and clinic appointments. Conclusion Since the advent of Anti Retroviral drugs with excellent efficacy and early diagnosis of HIV patients, incidence of disseminated fungal and mycobacterial infections have decreased in the United States. But patients with low CD4 count and cytopenias warrant a Bone Marrow aspiration or Biopsy to make a clear and early diagnosis. Disclosures All authors: No reported disclosures. [ABSTRACT FROM AUTHOR]