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The Pathophysiology of Acquired Aplastic Anemia
Idiopathic acquired aplastic anemia is a rare, life-threatening bone marrow failure syndrome characterized by cytopenias and hypocellular bone marrow. The pathophysiology is unknown; the most favored model is of a dysregulated immune system leading to autoreactive T-cell destruction of hematopoietic stem and progenitor cells in a genetically susceptible host. The authors review the literature and propose that the major driver of acquired aplastic anemia is a combination of hematopoietic stem and progenitor cells intrinsic defects and an inappropriately activated immune response in the setting of a viral infection. Alterati...
Source: Hematology/Oncology Clinics of North America - May 8, 2018 Category: Cancer & Oncology Authors: Michelle L. Schoettler, David G. Nathan Source Type: research

Complex Cancer Pain Assessment
Pain is widespread, multidimensional, and one of the most distressing symptoms patients with cancer face. Pain assessment is the foundation to optimal pain management. Despite evidence-based practice guidelines, inadequate pain assessment is a barrier. Patients should be routinely screened for pain at each encounter. If new, worsening, or persistent pain is present, a comprehensive pain assessment and reassessment should be regularly performed and documented to communicate the pain problem. Patient self-report of pain is the gold standard even in those who are nonverbal or cognitively impaired. Clinicians should follow the...
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Regina M. Fink, Jeannine M. Brant Source Type: research

Safe Opioid Use
This article provides a synopsis on the management of various opioid-related adverse effects and strategies to minimize aberrant opioid use in patients who have cancer. Many adverse effects can be effectively managed. Some patients on chronic opioid therapy may demonstrate aberrant behaviors suggestive of opioid misuse or diversion. Through intensive education, longitudinal monitoring, early identification, and timely management, clinicians can optimize the risk to benefit ratio to support safe opioid use. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Joseph Arthur, David Hui Source Type: research

Interventional Anesthetic Methods for Pain in Hematology/Oncology Patients
This article reviews anesthetic interventional approaches to the management of pain in hematology and oncology patients. It includes a discussion of single interventions including peripheral nerve blocks, plexus injections, and sympathetic nerve neurolysis, and continuous infusion therapy through implantable devices, such as intrathecal pumps, epidural port-a-caths, and tunneled catheters. The primary objective is to inform members of hematology and oncology care teams regarding the variety of interventional options for patients with cancer-related pain for whom medical pain management methods have not been effective. (Sou...
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Holly Careskey, Sanjeet Narang Source Type: research

Oncology Acupuncture for Chronic Pain in Cancer Survivors
Chronic pain syndromes associated with cancer treatment are common but difficult to manage. The American Society of Clinical Oncology recently published a practice guideline to address the unmet needs of cancer survivors, Management of Chronic Pain in Survivors of Adult Cancers, which stresses the importance of implementing integrative therapies including acupuncture. This review focuses on randomized clinical trials of acupuncture for chronic pain in cancer survivors, including its use in chemotherapy-induced peripheral neuropathy, aromatase inhibitor-associated arthralgia, and post neck dissection pain, and provides futu...
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Weidong Lu, David S. Rosenthal Source Type: research

Spiritual Considerations
Spiritual issues play a prominent role for patients with cancer. Studies have demonstrated a positive connection between a patient ’s spirituality and health outcomes, including quality of life, depression and anxiety, hopefulness, and the ability to cope with illness. Spiritual or existential distress is prominent in patients with cancer. Models are described that identify ways for clinicians to identify or diagnose spiritua l or existential distress, and to attend to that distress. It is critical that all clinicians assess for spiritual distress as part of a routine distress assessment, identify appropriate treatme...
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Christina M. Puchalski, Stephen D.W. King, Betty R. Ferrell Source Type: research

Mindfulness-Based Interventions for Hematology and Oncology Patients with Pain
Pain is a reality for approximately half of all of patients with cancer and can negatively affect patient cognitive and emotional states, resulting in “total pain.” Total pain may not respond to pharmacologic interventions and may pave the way for the onset of suffering, where suffering is defined as physical pain accompanied by negative cognitive interpretations. Mindfulness-based interventions provide an alternate interpretive framework for both pain and suffering and may lessen a patient’s experience of pain. Mindfulness-based interventions have the potential to alter a patient’s relationship to ...
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Denise Hess Source Type: research

Key Components of Pain Management for Children and Adults with Sickle Cell Disease
Sickle cell disease pain manifests as severe acute pain episodes and a debilitating chronic pain syndrome. Acute pain episodes are the most common reason for health care use; however, acute pain episodes are also frequently managed at home. Chronic pain syndrome develops in 30% to 40% of individuals with sickle cell disease, with an increasing incidence and severity with age. We review the critical aspects of pain management that are integral to the comprehensive approach to sickle cell disease pain and are rooted in the biopsychosocial model. The review focuses on opioid pharmacology and psychosocial comorbidities. (Sourc...
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Amanda M. Brandow, Michael R. DeBaun Source Type: research

Pain Control
HEMATOLOGY/ONCOLOGY CLINICS OF NORTH AMERICA (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Authors: Janet L. Abrahm Source Type: research

Copyright
ELSEVIER (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Source Type: research

Contributors
GEORGE P. CANELLOS, MD (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Source Type: research

Contents
Erratumxiii (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Source Type: research

Forthcoming Issues
Bone Marrow Failure (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Source Type: research

Erratum
In the article on “Emerging Therapies” appearing in the April 2018 issue of Hematology/Oncology Clinics of North America (Volume 32, Issue 2), the following errors were made on page 347 in Table 1: (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - May 3, 2018 Category: Cancer & Oncology Source Type: research

Significance of Clonal Mutations in Bone Marrow Failure and Inherited Myelodysplastic Syndrome/Acute Myeloid Leukemia Predisposition Syndromes
This article discusses recent advances in the understanding of context-dependent clonal hematopoiesis in MDS with focus on clonal evolution in inherited and acquired bone marrow failure syndromes. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 30, 2018 Category: Cancer & Oncology Authors: Eva J. Schaefer, R. Coleman Lindsley Source Type: research

Molecular Basis and Genetic Modifiers of Thalassemia
Thalassemia is a disorder of hemoglobin characterized by reduced or absent production of one of the globin chains in human red blood cells with relative excess of the other. Impaired synthesis of β-globin results in β-thalassemia, whereas defective synthesis of α-globin leads to α-thalassemia. Despite being a monogenic disorder, thalassemia exhibits remarkable clinical heterogeneity that is directly related to the intracellular imbalance between α- and β-like globin chains. Novel insig hts into the genetic modifiers have contributed to the understanding of the correlation between genotype a...
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: Sachith Mettananda, Douglas R. Higgs Source Type: research

Fertility and Pregnancy in Women with Transfusion-Dependent Thalassemia
As more women with transfusion-dependent thalassemia are seeking pregnancy, ensuring the best outcomes for both the mother and baby requires concerted, collaborative efforts between practitioners and the family. Proactive counseling, early fertility evaluation, recent developments in reproductive technology, and optimal management of iron overload, have resulted in more successful pregnancies and the birth of healthy newborns. With advances in technology for prenatal screening and increased awareness to perform screening for hemoglobinopathies, healthy pregnancy outcomes have become the expectation. Topics that require fur...
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: Katie T. Carlberg, Sylvia T. Singer, Elliott P. Vichinsky Source Type: research

Clinical Classification, Screening and Diagnosis for Thalassemia
At present, thalassemia diseases are classified into transfusion-dependent thalassemia and non –transfusion-dependent thalassemia. This classification is based on the clinical severity of patients determining whether they do require regular blood transfusions to survive (transfusion-dependent thalassemia) or not (non–transfusion-dependent thalassemia). In addition to the previous terminol ogy of “thalassemia major” or “thalassemia intermedia,” this classification has embraced all other forms of thalassemia syndromes such as α-thalassemia, hemoglobin E/β-thalassemia and combine...
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: Vip Viprakasit, Supachai Ekwattanakit Source Type: research

The Evolving Spectrum of the Epidemiology of Thalassemia
The thalassemias and other inherited disorders of hemoglobin are likely to remain a serious global health problem for the foreseeable future. Currently, they are most frequent in the tropical belt; an assessment of their true frequency and the likely cost of management for the governments of these countries will require a form of micromapping. Over recent years, there has been major progress toward better prevention and management of the thalassemias in richer countries; it is likely that, using the tools of molecular genetics, they will eventually be completely curable, although this is probably a long time in the future....
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: David J. Weatherall Source Type: research

Interaction of Transfusion and Iron Chelation in Thalassemias
The relationship between blood transfusion intensity, chelatable iron pools, and extrahepatic iron distribution is described in thalassemia. Risk factors for cardiosiderosis are discussed with particular reference to the balance of transfusional iron loading rate and transferrin-iron utilization rate as marked by plasma levels of soluble transferrin receptors. Low transfusion regimens increase residual erythropoiesis allowing for apotransferrin-dependent clearance of non –transferrin-bound iron species otherwise destined for myocardium. The impact of transfusion rates on chelation dosing required for iron balance is ...
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: John B. Porter, Maciej W. Garbowski Source Type: research

Hematopoietic Stem Cell Transplantation in Thalassemia
Although recent advances in gene therapy are expected to increase the chance of disease cure in thalassemia major, at present hematopoietic stem cell transplantation (HSCT) remains the only consolidated curative approach for this disorder. The widest experience has been obtained in the HLA-matched family donor (MFD) setting, with probabilities of overall and thalassemia-free survival exceeding 90% and 85%, respectively. As for most patients a suitable MFD is not available, alternative donors (HLA-matched unrelated donor, unrelated cord blood, HLA-haploidentical relative) have been increasingly explored, translating into th...
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: Luisa Strocchio, Franco Locatelli Source Type: research

MRI for Iron Overload in Thalassemia
MRI is a key tool in the current management of patients with thalassemia. Given its capability of assessing iron overload in different organs noninvasively and without contrast, it has significant advantages over other metrics, including serum ferritin. Liver iron concentration can be measured either with relaxometry methods T2*/T2 or signal intensity ratio techniques. Myocardial iron can be assessed in the same examination through T2* imaging. In this review, we focus on showing how MRI evaluates iron in both organs and the clinical applications as well as practical approaches to using this tool by clinicians taking care ...
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: Juliano Lara Fernandes Source Type: research

Erratum
An error was made in the article on “Identification and Targeting of Kinase Alterations in Histiocytic Neoplasms” appearing in the August 2017 issue of Hematology/Oncology Clinics of North America (Volume 31, Issue 4). Reference number 13 is incorrect. The correct reference is: Satoh T, Smith A, Sarde A, et al. B-RAF Mutant Allel es Associated with Langerhans Cell Histiocytosis, a Granulomatous Pediatric Disease. PLoS One 2012;7(4):e33891. This correction has been made in the online version. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Source Type: research

Thalassemia
HEMATOLOGY/ONCOLOGY CLINICS OF NORTH AMERICA (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Authors: Ali T. Taher Source Type: research

Copyright
ELSEVIER (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Source Type: research

Contributors
GEORGE P. CANELLOS, MD (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Source Type: research

Contents
Erratumxiii (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Source Type: research

Forthcoming Issues
Pain Control (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - April 1, 2018 Category: Cancer & Oncology Source Type: research

Cancer Pain Syndromes
This article reviews the phenomenology of common acute and chronic cancer pain syndromes. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 22, 2018 Category: Cancer & Oncology Authors: Russell K. Portenoy, Ebtesam Ahmed Source Type: research

Methadone
is a valuable opioid in the management of patients who have cancer with pain. Methadone is a mu-, kappa-, and delta-opioid agonist, and an N-methyl-D-aspartate receptor antagonist. These mechanisms of action make methadone an attractive option for complex pain syndromes. It is critically important that providers consider a patient ’s risk status before beginning methadone. Careful consideration must be given to dosing methadone in both opioid-naïve and opioid-tolerant patients, with vigilant monitoring for therapeutic effectiveness and potential toxicity until the patient achieves steady state. (Source: Hematol...
Source: Hematology/Oncology Clinics of North America - March 22, 2018 Category: Cancer & Oncology Authors: Mary Lynn McPherson, Ryan C. Costantino, Alexandra L. McPherson Source Type: research

Mechanisms of, and Adjuvants for, Bone Pain
This article discusses the effects of osteoclast overstimulation within the tumor microenvironment; the role of inflammatory factors at the tumor-nociceptor interface; the development of structural instability, causing mechanical nerve damage; and, ultimately, the neuroplastic changes in the setting of sustained pain. Several adjuvant therapies are available to attenuate metastatic bone pain. This article discusses the role of pharmacologic therapies, surgery, kyphoplasty, vertebroplasty, and radiofrequency ablation. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 22, 2018 Category: Cancer & Oncology Authors: Nicholas Figura, Joshua Smith, Hsiang-Hsuan Michael Yu Source Type: research

Cancer Pain Syndromes
This article reviews the phenomenology of common acute and chronic cancer pain syndromes. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 22, 2018 Category: Cancer & Oncology Authors: Russell K. Portenoy, Ebtesam Ahmed Source Type: research

Methadone
is a valuable opioid in the management of patients who have cancer with pain. Methadone is a mu-, kappa-, and delta-opioid agonist, and an N-methyl-D-aspartate receptor antagonist. These mechanisms of action make methadone an attractive option for complex pain syndromes. It is critically important that providers consider a patient ’s risk status before beginning methadone. Careful consideration must be given to dosing methadone in both opioid-naïve and opioid-tolerant patients, with vigilant monitoring for therapeutic effectiveness and potential toxicity until the patient achieves steady state. (Source: Hematol...
Source: Hematology/Oncology Clinics of North America - March 22, 2018 Category: Cancer & Oncology Authors: Mary Lynn McPherson, Ryan C. Costantino, Alexandra L. McPherson Source Type: research

Mechanisms of, and Adjuvants for, Bone Pain
This article discusses the effects of osteoclast overstimulation within the tumor microenvironment; the role of inflammatory factors at the tumor-nociceptor interface; the development of structural instability, causing mechanical nerve damage; and, ultimately, the neuroplastic changes in the setting of sustained pain. Several adjuvant therapies are available to attenuate metastatic bone pain. This article discusses the role of pharmacologic therapies, surgery, kyphoplasty, vertebroplasty, and radiofrequency ablation. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 22, 2018 Category: Cancer & Oncology Authors: Nicholas Figura, Joshua Smith, Hsiang-Hsuan Michael Yu Source Type: research

Radiation for Treatment of Painful Bone Metastases
Several variables may be considered when deciding on optimal modality of radiation therapy for each cancer patient with bone pain, including prognosis, tumor histology, location and extent of metastases, and association with cord compression. Hypofractionated external beam radiation therapy is as effective as a multiple fraction radiotherapy course in most cases, although retreatment rates are higher after a single dose of radiation. Stereotactic body radiation may be used in cases of oligometastatic disease, repeat irradiation, and radiation-resistant tumors. Radiopharmaceuticals may be used for pain from diffuse bone met...
Source: Hematology/Oncology Clinics of North America - March 16, 2018 Category: Cancer & Oncology Authors: Ron Shiloh, Monica Krishnan Source Type: research

Rehabilitation Medicine Approaches to Pain Management
Rehabilitation medicine offers strategies that reduce musculoskeletal pain, targeted approaches to alleviate movement-related pain, and interventions to optimize patients ’ function despite the persistence of pain. These approaches fall into four categories: modulating nociception, stabilizing and unloading painful structures, influencing pain perception, and alleviating soft tissue musculotendinous pain. Incorporating these interventions into individualized, compr ehensive pain management programs offers the potential to empower patients and limit pain associated with mobility and required daily activities. Rehabili...
Source: Hematology/Oncology Clinics of North America - March 16, 2018 Category: Cancer & Oncology Authors: Andrea L. Cheville, Sean Smith, Jeffrey R. Basford Source Type: research

Pain, a Complex Challenge
Despite the many diagnostic and therapeutic advances in the 26 years since the last Hematology/Oncology Clinics of North America issue on Pain, control of pain in our patients  remains a complex challenge. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 16, 2018 Category: Cancer & Oncology Authors: Janet L. Abrahm Tags: Preface Source Type: research

Radiation for Treatment of Painful Bone Metastases
Several variables may be considered when deciding on optimal modality of radiation therapy for each cancer patient with bone pain, including prognosis, tumor histology, location and extent of metastases, and association with cord compression. Hypofractionated external beam radiation therapy is as effective as a multiple fraction radiotherapy course in most cases, although retreatment rates are higher after a single dose of radiation. Stereotactic body radiation may be used in cases of oligometastatic disease, repeat irradiation, and radiation-resistant tumors. Radiopharmaceuticals may be used for pain from diffuse bone met...
Source: Hematology/Oncology Clinics of North America - March 16, 2018 Category: Cancer & Oncology Authors: Ron Shiloh, Monica Krishnan Source Type: research

Rehabilitation Medicine Approaches to Pain Management
Rehabilitation medicine offers strategies that reduce musculoskeletal pain, targeted approaches to alleviate movement-related pain, and interventions to optimize patients ’ function despite the persistence of pain. These approaches fall into four categories: modulating nociception, stabilizing and unloading painful structures, influencing pain perception, and alleviating soft tissue musculotendinous pain. Incorporating these interventions into individualized, compr ehensive pain management programs offers the potential to empower patients and limit pain associated with mobility and required daily activities. Rehabili...
Source: Hematology/Oncology Clinics of North America - March 16, 2018 Category: Cancer & Oncology Authors: Andrea L. Cheville, Sean Smith, Jeffrey R. Basford Source Type: research

Pain, a Complex Challenge
Despite the many diagnostic and therapeutic advances in the 26 years since the last Hematology/Oncology Clinics of North America issue on Pain, control of pain in our patients  remains a complex challenge. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 16, 2018 Category: Cancer & Oncology Authors: Janet L. Abrahm Tags: Preface Source Type: research

Psychological Treatment
Psychological approaches to pain management have been demonstrated to be effective for individuals newly diagnosed with cancer, in remission, and/or with progressive or terminal disease. Modalities that have been demonstrated to be most effective are cognitive behavioral approaches that include relaxation skills and/or hypnotherapy. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 12, 2018 Category: Cancer & Oncology Authors: Thomas B. Strouse, Brenda Bursch Source Type: research

Cancer-Related Neuropathic Pain
Neuropathic pain is the result of neuroplastic and neuroinflammatory changes from trauma or diseases that damage the somatosensory system. Cancer-related neuropathic pain is caused by treatment, cancer, or paraneoplastic reactions to cancer. Approximately 30% of patients with cancer have neuropathic pain, mostly mixed nociceptive and neuropathic pain. History, physical examination, quantitative sensory testing, skin punch biopsies, and functional MRIs help to divide pain into phenotypes that may facilitate analgesic choices. Guidelines for treating cancer-related neuropathic pain are not consistent and are highly dependent...
Source: Hematology/Oncology Clinics of North America - March 9, 2018 Category: Cancer & Oncology Authors: Mellar P. Davis Source Type: research

Pain Syndromes and Management in Adult Hematopoietic Stem Cell Transplantation
Pain is a significant physical symptom that can be observed across the spectrum of hematopoietic stem cell transplant (HSCT) care. Pain assessment should include evaluation of the physical and functional components of pain. Management varies based on the type of HSCT-specific pain syndrome. Future directions for management of pain syndromes include the early integration of palliative care. The purpose of this review is to summarize various pain syndromes and management approaches in adult HSCT patients. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 9, 2018 Category: Cancer & Oncology Authors: Joseph D. Ma, Areej R. El-Jawahri, Thomas W. LeBlanc, Eric J. Roeland Source Type: research

Cancer-Related Neuropathic Pain
Neuropathic pain is the result of neuroplastic and neuroinflammatory changes from trauma or diseases that damage the somatosensory system. Cancer-related neuropathic pain is caused by treatment, cancer, or paraneoplastic reactions to cancer. Approximately 30% of patients with cancer have neuropathic pain, mostly mixed nociceptive and neuropathic pain. History, physical examination, quantitative sensory testing, skin punch biopsies, and functional MRIs help to divide pain into phenotypes that may facilitate analgesic choices. Guidelines for treating cancer-related neuropathic pain are not consistent and are highly dependent...
Source: Hematology/Oncology Clinics of North America - March 9, 2018 Category: Cancer & Oncology Authors: Mellar P. Davis Source Type: research

Pain Syndromes and Management in Adult Hematopoietic Stem Cell Transplantation
Pain is a significant physical symptom that can be observed across the spectrum of hematopoietic stem cell transplant (HSCT) care. Pain assessment should include evaluation of the physical and functional components of pain. Management varies based on the type of HSCT-specific pain syndrome. Future directions for management of pain syndromes include the early integration of palliative care. The purpose of this review is to summarize various pain syndromes and management approaches in adult HSCT patients. (Source: Hematology/Oncology Clinics of North America)
Source: Hematology/Oncology Clinics of North America - March 9, 2018 Category: Cancer & Oncology Authors: Joseph D. Ma, Areej R. El-Jawahri, Thomas W. LeBlanc, Eric J. Roeland Source Type: research

Molecular Basis and Genetic Modifiers of Thalassemia
Thalassemia is a disorder of hemoglobin characterized by reduced or absent production of one of the globin chains in human red blood cells with relative excess of the other. Impaired synthesis of β-globin results in β-thalassemia, whereas defective synthesis of α-globin leads to α-thalassemia. Despite being a monogenic disorder, thalassemia exhibits remarkable clinical heterogeneity that is directly related to the intracellular imbalance between α- and β-like globin chains. Novel insig hts into the genetic modifiers have contributed to the understanding of the correlation between genotype a...
Source: Hematology/Oncology Clinics of North America - February 17, 2018 Category: Cancer & Oncology Authors: Sachith Mettananda, Douglas R. Higgs Source Type: research

Fertility and Pregnancy in Women with Transfusion-Dependent Thalassemia
As more women with transfusion-dependent thalassemia are seeking pregnancy, ensuring the best outcomes for both the mother and baby requires concerted, collaborative efforts between practitioners and the family. Proactive counseling, early fertility evaluation, recent developments in reproductive technology, and optimal management of iron overload, have resulted in more successful pregnancies and the birth of healthy newborns. With advances in technology for prenatal screening and increased awareness to perform screening for hemoglobinopathies, healthy pregnancy outcomes have become the expectation. Topics that require fur...
Source: Hematology/Oncology Clinics of North America - February 17, 2018 Category: Cancer & Oncology Authors: Katie T. Carlberg, Sylvia T. Singer, Elliott P. Vichinsky Source Type: research

Clinical Classification, Screening and Diagnosis for Thalassemia
At present, thalassemia diseases are classified into transfusion-dependent thalassemia and non –transfusion-dependent thalassemia. This classification is based on the clinical severity of patients determining whether they do require regular blood transfusions to survive (transfusion-dependent thalassemia) or not (non–transfusion-dependent thalassemia). In addition to the previous terminol ogy of “thalassemia major” or “thalassemia intermedia,” this classification has embraced all other forms of thalassemia syndromes such as α-thalassemia, hemoglobin E/β-thalassemia and combine...
Source: Hematology/Oncology Clinics of North America - February 17, 2018 Category: Cancer & Oncology Authors: Vip Viprakasit, Supachai Ekwattanakit Source Type: research

The Evolving Spectrum of the Epidemiology of Thalassemia
The thalassemias and other inherited disorders of hemoglobin are likely to remain a serious global health problem for the foreseeable future. Currently, they are most frequent in the tropical belt; an assessment of their true frequency and the likely cost of management for the governments of these countries will require a form of micromapping. Over recent years, there has been major progress toward better prevention and management of the thalassemias in richer countries; it is likely that, using the tools of molecular genetics, they will eventually be completely curable, although this is probably a long time in the future....
Source: Hematology/Oncology Clinics of North America - February 17, 2018 Category: Cancer & Oncology Authors: David J. Weatherall Source Type: research