Hematology Commons^™

The Role Of Platelet Microvesicles In Intercellular Communication., Leonard C. Edelstein

Cardeza Foundation for Hematologic Research

In recent years, there has been exponential growth in the interest in microvesicles, which is reflected by the number of publications. Initially referred to as "platelet dust" by Peter Wolf in 1967, platelet microvesicles (PMV) are now recognized as important mediators of intercellular communication. There are examples of PMV exerting physiological effects on almost all hematological and vascular cell types, including monocytes, macrophages, neutrophils, T-cells, endothelium cells, and smooth muscle cells (SMCs). PMV can exert these effects by multiple methods: extracellular signaling through receptors, transfer of surface molecules, and delivery of intracellular contents including miRNA. Recent work suggests a complex …

Autoimmune Hemolytic Anemia Associated With Babesiosis, Roshni Narurkar, Aleksandra Mamorska-Dyga, John Nelson, Delong Liu

NYMC Faculty Publications

BACKGROUND: Babesiosis is endemic in selected areas in North America. Babesia infection is commonly associated with anemia, thrombocytopenia, hyponatremia and elevated liver enzymes. Autoimmune hemolytic anemia (AIHA) is known to be caused by parasitic and viral infections but has not been well characterized. CASE PRESENTATION: We describe two cases diagnosed with babesiosis triggering severe AIHA. One case had history of splenectomy, and the other was an elderly patient. Older, immunocompromised and asplenic patients may be particularly at risk for post-babesiosis AIHA (PB-AIHA). CONCLUSIONS: The pathogenesis for conventional AIHA and PB-AIHA appears to be different, since splenectomy is a treatment for …

Diagnosis Of Lchad/Tfp Deficiency In An At Risk Newborn Using Umbilical Cord Blood Acylcarnitine Analysis, Donna Raval, Kristina Cusmano-Ozog, Omar Ayyub, Callie Jenevein, Laura Kofman, Brendan Lanpher, Natalie Hauser, Debra Regier

Pathology Faculty Publications

Trifunctional protein deficiency/Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD/TFP) deficiency is a disorder of fatty acid oxidation and ketogenesis. Severe neonatal lactic acidosis, cardiomyopathy, and hepatic dysfunction are caused by the accumulation of toxic long-chain acylcarnitines. The feasibility of umbilical cord blood use in screening for acylcarnitine analysis and free carnitine has been hypothesized but not reported in LCHAD/TFP neonates.

We present a 4 week old female who was at risk of inheriting LCHAD/TFP deficiency and was diagnosed at the time of delivery using umbilical cord blood. Umbilical cord blood was collected at delivery and sent for acylcarnitine analysis. Treatment was started …

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

Shereen M.F. Gheith, M.D., PhD.

No abstract provided.

Diagnosis And Treatment Of Cd20 Negative B Cell Lymphomas, Tasleem Katchi, Delong Liu

NYMC Faculty Publications

CD20 negative B cell non-Hodgkin lymphoma (NHL) is rare and accounts for approximately 1-2% of B cell lymphomas. CD20- negative NHL is frequently associated with extranodal involvement, atypical morphology, aggressive clinical behaviour, resistance to standard chemotherapy and poor prognosis. The most common types of these include plasmablastic lymphoma, primary effusion lymphoma, large B-cell lymphoma arising from HHV8-associated multicentric Castleman's disease, and ALK+ large B cell lymphoma. This review provides an overview of the diagnostic and treatment modalities for CD20 negative B cell NHL.

Aapt Diagnostic Criteria For Chronic Sickle Cell Disease Pain., Carlton Dampier, Tonya M Palermo, Deepika S Darbari, Kathryn Hassell, Wally Smith, William Zempsky

Pediatrics Faculty Publications

Pain in sickle cell disease (SCD) is associated with increased morbidity, mortality, and high health care costs. Although episodic acute pain is the hallmark of this disorder, there is an increasing awareness that chronic pain is part of the pain experience of many older adolescents and adults. A common set of criteria for classifying chronic pain associated with SCD would enhance SCD pain research efforts in epidemiology, pain mechanisms, and clinical trials of pain management interventions, and ultimately improve clinical assessment and management. As part of the collaborative effort between the Analgesic, Anesthetic, and Addiction Clinical Trial Translations Innovations Opportunities …

Dynamical System Modeling To Simulate Donor T Cell Response To Whole Exome Sequencing-Derived Recipient Peptides: Understanding Randomness In Alloreactivity Incidence Following Stem Cell Transplantation, Vishal Koparde, Badar Abdul Razzaq, Tara Suntum, Roy Sabo, Allison Scalora, Myrna Serrano, Max Jameson-Lee, Charles Hall, David Kobulnicky, Nihar Sheth, Juliana Feltz, Daniel Contaifer, Dayanjan Wijesinghe, Jason Reed, Catherine Roberts, Rehan Qayyum, Gregory Buck, Michael Neale, Amir Toor

Massey Comprehensive Cancer Center Data

Quantitative relationship between the magnitude of variation in minor histocompatibility antigens (mHA) and graft versus host disease (GVHD) pathophysiology in stem cell transplant (SCT) donor-recipient pairs (DRP) is not established. In order to elucidate this relationship, whole exome sequencing (WES) was performed on 27 HLA matched related (MRD), & 50 unrelated donors (URD), to identify nonsynonymous single nucleotide polymorphisms (SNPs). An average 2,463 SNPs were identified in MRD, and 4,287 in URD DRP (p

Post-Transfusion Purpura: A Case Report Of An Underdiagnosed Phenomenon, Hind Rafei, Raza Yunus, Samah Nassereddine

Medicine Faculty Publications

Post-transfusion purpura is a rare transfusion-related complication that often goes undiagnosed. It is due to alloimmunization against platelet antigens which leads to acute profound thrombocytopenia following the transfusion of any platelet-containing product (red blood cells or platelets). It is commonly seen in multiparous women. Here, we report a case of post-transfusion purpura in a 56-year-old multiparous woman who developed acute thrombocytopenia seven days following a packed red blood cell transfusion. We will discuss the clinical presentation, diagnosis, workup and treatment of this rare disease. It is important to recognize this entity separately and to include it in the differential diagnosis …

Plasmablastic Lymphoma: Case Report Of Prolonged Survival Of An Advanced Human Immunodeficiency Patient And Literature Review, Hind Rafei, Ehab El-Bahesh, Antoine Finianos, Min-Ling Liu, Geraldine Schechter

Medicine Faculty Publications

Clinical Practice Points. Plasmablastic lymphoma (PBL) is a rare and highly aggressive variant of diffuse large B cell lymphoma with median survival of advanced stage patients varying between 6 and 15 months in previous reports. We report here a human immunodeficiency virus-infected patient surviving over 12 years following treatment for advanced PBL with EPOCH chemotherapy and intrathecal therapy. This case highlights the potential for improved survival in PBL with intensive chemotherapy. Further, literature review suggests promising prospects utilizing novel targeted therapies to increase the rate of prolonged responses.

Your Guide To Immunotherapy, Lehigh Valley Health Network, Accc Association Of Community Cancer Centers

Lehigh Valley Topper Cancer Institute

No abstract provided.

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 …

A Journey Of Educational Transformation And Organizational Improvement Through Development Of An Institutional Patient Blood Management Program, Robert Raggi, Karen Klein, Marci Swearingen, Mark Domantay

Articles, Abstracts, and Reports

Providence Holy Cross Medical Center (PHCMC) has embarked on this journey to improve outcomes by reducing the number of unnecessary transfusions through the development of a PBM program. Transfusion-related health risks and increasing economic pressures have driven hospitals to recognize development of blood management programs as an important strategy to improve patient outcomes and reduce costs.

Babesiosis-Associated Immune Thrombocytopenia, Roshni Narurkar, Aleksandra Mamorska-Dyga, A Agarwal, John Nelson, Delong Liu

NYMC Faculty Publications

Thrombocytopenia is a common feature of babesiosis. The mechanism for thrombocytopenia in babesiosis remains elusive. We report a case of babesiosis with severe new onset immune thrombocytopenia (ITP). In addition to antibiotics treatment for babesiosis, ITP therapy was administered. ITP in the present case was most likely triggered by the babesia infection. The severity of ITP in this case was not proportional to the severity of parasitemia. The neoantigen triggering the autoimmune response in babesiosis requires further characterization.

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 …

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 …

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

Yehuda Z. Lebowicz, MD

Contrast enhanced CT showing multiple splenic infarcts

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

Yehuda Z. Lebowicz, MD

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 …