Hematology Commons^™

Health-Related Quality Of Life (Hr-Qol) And Chronic Health Conditions In Survivors Of Childhood Acute Myeloid Leukemia (Aml) With Down Syndrome (Ds): A Report From The Children's Oncology Group., Kris Ann P. Schultz, Lu Chen, Alicia Kunin-Batson, Zhengjia Chen, William G. Woods, A S. Gamis, Toana Kawashima, Kevin C. Oeffinger, H Stacy Stacy Nicholson, Joseph P. Neglia

Manuscripts, Articles, Book Chapters and Other Papers

Survival rates for children with Down syndrome (DS) and acute myeloid leukemia (AML) are high; however, little is known regarding the health-related quality of life (HR-QOL) of these survivors. Individuals who survived ≥5 years following diagnosis of childhood AML were invited to complete parent or patient-report surveys measuring HR-QOL and chronic health conditions. In total, 26 individuals with DS had a median age at diagnosis of 1.8 years (range, 0.77 to 10.9 y) and median age at interview of 15 years (range, 8.3 to 27.6 y). Participants with DS and AML were compared with AML survivors without DS whose caregiver …

Novel Hla-Dp Region Susceptibility Loci Associated With Severe Acute Gvhd., Rakesh K. Goyal, S J. Lee, T Wang, M Trucco, M Haagenson, S R. Spellman, M Verneris, R E. Ferrell

Manuscripts, Articles, Book Chapters and Other Papers

Despite HLA allele matching, significant acute GvHD remains a major barrier to successful unrelated donor BMT. We conducted a genome-wide association study (GWAS) to identify recipient and donor genes associated with the risk of acute GvHD. A case-control design (grade III-IV versus no acute GvHD) and pooled GWA approach was used to study European-American recipients with hematological malignancies who received myeloablative conditioning non-T-cell-depleted first transplantation from HLA-A, -B, -C, -DRB1, -DQB1 allele level (10/10) matched unrelated donors. DNA samples were divided into three pools and tested in triplicate using the Affymetrix Genome-wide SNP Array 6.0. We identified three novel susceptibility …

Protein Profiling Analysis Of Platelets In Hypercoagulable State Of Β-Thalassemia/Hbe Patients, Puangpaka Chanpeng

Chulalongkorn University Theses and Dissertations (Chula ETD)

β-thalassemia/HbE is an inherited hemolytic anemia caused by defect in β-globin synthesis resulting in accumulation of excess α-globin chains in red blood cells. A hypercogulable state leading to high risk of thromboembolic event is one of the most common complications observed in this disease, particularly in patients with splenectomy. Previous studies suggested that increased platelet activation and coagulation factors in β-thalassemia/HbE intermediate patients promote the hypercoagulable state. However, the hypercoagulable state as well as the molecular mechanism regarding this pathogenesis in β-thalassemia/HbE is not yet well understood. This study aimed to identify proteins related to platelet activation and to hypercoagulable …

The Hemostatic Effects Of Acute Exposure To Colored Cornstarch Powder During A 5k Run, Robert C. Allsbrook

Senior Honors Projects, 2010-2019

PURPOSE: To examine the acute hemostatic effects of particulate matter (PM) in the form of colored corn starch powder during a 5 kilometer race. METHODS: 10 recreationally active adults completed two 5k runs, one with color and one without color. 10 mL blood samples were taken 6 hours prior to the trial and immediately following the trial. PAI-1 activity, FVIII antigen, and tPA activity were measured using an ELISA. RESULTS: No significant main effects or interaction effects (P<0.05) were observed among any of the variables although a trend (P = 0.082) was observed for increased PAI-1 activity during exercise in the color condition. CONCLUSIONS: There is a trend towards increased PAI-1 activity levels during exercise during color runs that could be due to increased inflammation. Furthermore, the lack of increased FVIII:ag and tPA activity suggests that the intensity of the exercise may not have been adequate and further research should be conducted in this area.

Stars In Cytoplasm: Pediatric Neuroblastoma, Monazza Chaudhary, Shahzad Sarwar, Natasha Bahadur Ali

Section of Haematology/Oncology

We report a case of a 2-year-old female child who presented with abdominal distention and fever. On examination, there was a palpable mass in the right hypochondrium. Computed tomography of the abdomen revealed a large mass in the right suprarenal area. Biopsy of the mass revealed malignant round blue cell neoplasm. Bone marrow examination showed diffuse infiltration with atypical mononuclear cells and cytogenetic studies showed positivity for NMyc translocation.

A Trial Of Unrelated Donor Marrow Transplantation For Children With Severe Sickle Cell Disease., Shalini Shenoy, Mary Eapen, Julie A. Panepinto, Brent R. Logan, Juan Wu, Allistair Abraham, Joel Brochstein, Sonali Chaudhury, Kamar Godder, Ann E. Haight, Kimberly A. Kasow, Kathryn Leung, Martin Andreansky, Monica Bhatia, Jignesh Dalal, Hilary Haines, Jennifer Jaroscak, Hillard M. Lazarus, John E. Levine, Lakshmanan Krishnamurti, David Margolis, Gail C. Megason, Lolie C. Yu, Michael A. Pulsipher, Iris Gersten, Nancy Difronzo, Mary M. Horowitz, Mark C. Walters, Naynesh Kamani

Manuscripts, Articles, Book Chapters and Other Papers

Children with sickle cell disease experience organ damage, impaired quality of life, and premature mortality. Allogeneic bone marrow transplant from an HLA-matched sibling can halt disease progression but is limited by donor availability. A Blood and Marrow Transplant Clinical Trials Network (BMT CTN) phase 2 trial conducted from 2008 to 2014 enrolled 30 children aged 4 to 19 years; 29 were eligible for evaluation. The primary objective was 1-year event-free survival (EFS) after HLA allele-matched (at HLA-A, -B, -C, and -DRB1 loci) unrelated donor transplant. The conditioning regimen included alemtuzumab, fludarabine, and melphalan. Graft-versus-host disease (GVHD) prophylaxis included calcineurin inhibitor, …

Splenic Infarction: An Uncommon Presentation Of Acute Infectious Mononucleosis, Madhulika Urella, Roma Srivastava, Waseem Ahmed, Yehuda Lebowicz

Marshall Journal of Medicine

Splenic infarction is a relatively uncommon diagnosis. It occurs when the splenic artery or one of its sub-branches is occluded with an infected or bland embolus or clot. Splenic infarction may be caused by atrial fibrillation, bacterial endocarditis, sickle cell disease, antiphospholipid syndrome, and trauma whereas an infectious etiology is uncommon. It is considered a rare presentation of acute infectious mononucleosis. Currently, its pathogenesis is still unclear. We describe a 24-year-old African American female who was admitted for evaluation of left-sided chest pain. Chest imaging, abdominal ultrasound, and initial laboratory data were normal, followed by a negative hypercoagulability panel. Signs …

Primary Malignant Pleural Effusion With A Profound Type B Lactic Acidosis, Rachel Kinney Do, Christine Saraceni Do, Eliot L. Friedman Md

Department of Medicine

No abstract provided.

Feasibility Of A Community-Based Sickle Cell Trait Testing And Counseling Program, Ashley Housten, Regina Abel, Terianne Lindsey, Allison King

Journal of Health Disparities Research and Practice

Background: Sickle cell trait (SCT) screening is required at birth in the United States; however, adults rarely know their SCT status prior to having children.

Purpose: Assess feasibility of a community-based SCT education and testing intervention.

Methods: Participants were recruited from eight community sites to complete an educational program and offered a hemoglobin analysis. A genetic counselor met individually with participants to discuss lab results.

Results: Between July 14, 2010 and May 31, 2012, 637 participants completed the educational program. Five hundred seventy (89.5%) provided a blood sample, and 61 (10.9%) had SCT or other hemoglobinopathies. The genetic counselor met …

Hemophagocytic Lymphohistiocytosis, An Overlooked Culprit Of Disseminated Intravascular Coagulation, Shivtaj Mann Do

Department of Medicine

No abstract provided.

Prevalence Of Stroke In Children Admitted With Sickle Cell Anaemia To Mulago Hospital, Samden Lhatoo, Martha Sajatovic

Faculty Scholarship

Background: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. Methods: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in …

Adrenal Incidentaloma In Young – A Gray Zone?, Ranjit R. Nair Md, Stacey Smith Md, Facp

Department of Medicine

No abstract provided.

A Predictive Model For Intensive Chemotherapy Outcomes In Newly Diagnosed Elderly Patients With Aml, Sameer Tolay Md, David F. Claxton Md, Junjia Zhu Md

Department of Medicine

No abstract provided.

Peritoneal Lymphomatosis: The Failure Of Occam’S Razor, Ranjit R. Nair Md, Nicole M. Agostino Do, Shereen M F Gheith Md, Usman Shah Md

Department of Medicine

No abstract provided.

Mutations Affecting Epigenetic Regulators And Their Role In Peripheral T-Cell Lymphomas, Chao Wang

Theses & Dissertations

Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of generally aggressive lymphoid malignancies, accounting for 10-15% of all non-Hodgkin lymphomas. Angioimmunoblastic T-cell lymphoma (AITL) represents approximately 20% of all PTCLs and is recognized as a distinct entity. Accurate diagnosis and classification of PTCL remain challenging. With the exception of ALK+ ALCL, patients with PTCL generally have a poor prognosis with standard chemotherapy and even with the availability of many novel drugs, including HDAC inhibitor (romidepsin and belinostat), gemcitabine, and bortezomib. Therefore, deciphering the pathogenesis of this group of diseases is needed to identify novel treatable targets for better therapeutic intervention. …

Pattern Of Relapse In Paediatric Acute Lymphoblastic Leukaemia In A Tertiary Care Unit, Emaduddin Siddiqui, Sayyeda Ghazala Kazi, Muhammad Irfan Habib, Khalid Mehmood Ahmed Khan, Nukhba Zia

Department of Emergency Medicine

OBJECTIVE:

To determine the frequency, site and time to relapse from diagnosis, and to see the relationship of relapse with important prognostic factors.

METHODS:

The prospective descriptive observational study was conducted at the National Institute of Child Health, Karachi, June 2005 to May 2007, and comprised newly-diagnosed cases of acute lymphoblastic leukaemia. Bone marrow aspiration was done on reappearance of blast cells in peripheral smear and cerebrospinal fluid. Detailed report was done each time when intra-thecal chemotherapy was given or there were signs and symptoms suggestive of central nervous system relapse. SPSS 12 was used for data analysis.

RESULTS:

Of …

Immunoglobulin G4-Related Disease: A Rare Disease With An Unusual Presentation, Muhammad Waqas Khan, Terrance Hadley, Melissa Kesler, Zartash Gul

Internal Medicine Faculty Publications

IgG4-RD can also present in the skeletal muscle, mimicking several other diseases. It is unusual for this relatively new classification of diseases to present in the muscles and can be mistakenly diagnosed as other autoimmune diseases rendering a delay in the appropriate management and progression of the disease.

Age- And Sex-Related Changes In Fasting Plasma Glucose And Lipoprotein In Cynomolgus Monkeys, Feng Yue, Guodong Zhang, Rongping Tang, Zhouquan Zhang, Liqiong Teng, Zhiming Zhang

Neuroscience Faculty Publications

Background: The age-related dysfunction of glucose and lipid metabolism has a long-standing relationship with cardiovascular and neurodegenerative disease. However, the effects of metabolic dysfunction on men and women are different. Reasons for these sex differences remains unclear. Cynomolgus monkeys have been used, in the past, for the study of human metabolic diseases due to their biologically proximity to humans. Nevertheless, few studies to date have focused on both age- and sex-related differences in glucose and lipid metabolism. The present study was designed to specifically address these questions by using a large cohort of cynomolgus monkeys (N = 1,399) including …

Total Versus Partial Splenectomy In Pediatric Hereditary Spherocytosis: A Systematic Review And Meta-Analysis, Leonardo Guizzetti

Epidemiology and Biostatistics Publications

To compare the clinical effectiveness of total (TS) or partial (PS) splenectomy in pediatric hereditary spherocytosis, a systematic review and meta-analysis was performed (PROSPERO registration CRD42015030056). There were 14 observational studies comparing pre- and post- operative hematologic parameters. Secondary outcomes include in-hospital infections, surgical complications, symptomatic recurrence and biliary disease. TS is more effective than PS to increase hemoglobin (3.6 g/dL vs 2.2 g/dL) and reduce reticulocytes (12.5% vs 6.5%) after one year; outcomes following PS are stable for at least 6 years. There were no cases of overwhelming post-splenectomy sepsis. A population-based patient registry is needed for long-term follow-up.

The Mysteries Of Your Blood, Alexandra J. Mauer

BU Well

Did you know that your blood type can be useful for determining disease susceptibility and even creating a diet that works best for your personal metabolism? The Mysteries of your Blood is an article that delves into the science behind the ABO blood types and explains why your body can handle certain foods, stress, and illness differently than someone with a different blood type. It proposes a new, perplexing way to determine how to avoid certain health problems while at the same time offering suggestions of what to eat or, rather, what to avoid eating.