Closing the gap: Novel therapies in treating acute lymphoblastic leukemia in adolescents and young adults
Acute lymphoblastic leukemia (ALL) is one of the most common cancer diagnoses identified in adolescents and young adults (AYAs). Although most children with ALL are cured of their disease, AYAs have experienced much worse outcomes over time, with event-free survival ranging from 30 to 45%. This survival disparity is likely due to differences in tumor biology, treatment-related toxicities, and nonmedical issues. This review summarizes these differences as well as focusing on the various trials that have demonstrated superior outcomes with pediatric protocols in AYAs with ALL. (Source: Blood Reviews)
Source: Blood Reviews - September 20, 2017 Category: Hematology Authors: Brian D. Friend, Gary J. Schiller Tags: Review Source Type: research

The global problem of early deaths in acute promyelocytic leukemia: A strategy to decrease induction mortality in the most curable leukemia
Acute promyelocytic leukemia (APL) is a hyper-acute illness and presents with profound cytopenias in most patients and disseminated intravascular coagulation (DIC). Excellent treatment options are now available with drugs such as all-trans retinoic acid (ATRA), arsenic trioxide (ATO), anthracyclines and cytarabine. The outcome in APL has improved tremendously in the last 50years due to better understanding of the disease, development of effective targeted agents and improvement in supportive care. (Source: Blood Reviews)
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: A.P. Jillella, V.K. Kota Tags: Review Source Type: research

Treatment of musculo-skeletal pain in haemophilia
Musculo-skeletal pain treatment is inadequate in many haemophilic patients. Analgesics are used only by 36% of adult patients. FVIII/FIX intravenous infusion is mainly used to lessen pain, followed in frequency by usage of NSAIDS (primarily COX-2 inhibitors). In about 30% of patients, pain continues after infusion of F VIII/IX. In acute haemarthroses pain treatment must continue until total disappearance (checked by ultrasonography) and include haematologic treatment, short-term rest of the involved joint, cryotherapy, joint aspiration and analgesic medication (paracetamol in mild pain, metamizole for more intense pain, an...
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: E. Carlos Rodriguez-Merchan Tags: Review Source Type: research

Immunoglobulin therapy in hematologic neoplasms and after hematopoietic cell transplantation
Immunoglobulins are used to prevent or reduce infection risk in primary immune deficiencies and in settings which exploit its anti-inflammatory and immune-modulatory effects. Rigorous proof of immunoglobulin efficacy in persons with lympho-proliferative neoplasms, plasma cell myeloma, and persons receiving hematopoietic cell transplants is lacking despite many clinical trials. Further, there are few consensus guidelines or algorithms for use in these conditions. Rapid development of new therapies targeting B-cell signaling and survival pathways and increased use of chimeric antigen receptor T-cell (CAR-T) therapy will like...
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: Masumi Ueda, Melvin Berger, Robert Peter Gale, Hillard M. Lazarus Tags: Review Source Type: research

Erythroleukemia-historical perspectives and recent advances in diagnosis and management
Acute erythroleukemia is a rare form of acute myeloid leukemia recognized by its distinct phenotypic attribute of erythroblastic proliferation. After a century of its descriptive history, many diagnostic, prognostic, and therapeutic implications relating to this unique leukemia subset remain uncertain. The rarity of the disease and the simultaneous involvement of its associated myeloid compartment have complicated in vitro studies of human erythroleukemia cell lines. Although murine and cell line erythroleukemia models have provided valuable insights into pathophysiology, translation of these concepts into treatment are no...
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: Prajwal Boddu, Christopher B. Benton, Wei Wang, Gautam Borthakur, Joseph D. Khoury, Naveen Pemmaraju Tags: Review Source Type: research

The global problem of early deaths in acute promyelocytic leukemia: A strategy to decrease induction mortality in the most curable leukemia
Acute promyelocytic leukemia (APL) is a hyper-acute illness and presents with profound cytopenias in most patients and disseminated intravascular coagulation (DIC). Excellent treatment options are now available with drugs such as all-trans retinoic acid (ATRA), arsenic trioxide (ATO), anthracyclines and cytarabine. The outcome in APL has improved tremendously in the last 50years due to better understanding of the disease, development of effective targeted agents and improvement in supportive care. (Source: Blood Reviews)
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: A.P. Jillella, V.K. Kota Tags: Review Source Type: research

Treatment of musculo-skeletal pain in haemophilia
Musculo-skeletal pain treatment is inadequate in many haemophilic patients. Analgesics are used only by 36% of adult patients. FVIII/FIX intravenous infusion is mainly used to lessen pain, followed in frequency by usage of NSAIDS (primarily COX-2 inhibitors). In about 30% of patients, pain continues after infusion. of F VIII/IX. In acute haemarthroses pain treatment must continue until total disappearance (checked by ultrasonography) and include haematologic treatment, short-term rest of the involved joint, cryotherapy, joint aspiration and analgesic medication (paracetamol in mild pain, metamizole for more intense pain, a...
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: E. Carlos Rodriguez-Merchan Tags: Review Source Type: research

Immunoglobulin therapy in hematologic neoplasms and after hematopoietic cell transplantation
Immunoglobulins are used to prevent or reduce infection risk in primary immune deficiencies and in settings which exploit its anti-inflammatory and immune-modulatory effects. Rigorous proof of immunoglobulin efficacy in persons with lympho-proliferative neoplasms, plasma cell myeloma, and persons receiving hematopoietic cell transplants is lacking despite many clinical trials. Further, there are few consensus guidelines or algorithms for use in these conditions. Rapid development of new therapies targeting B-cell signaling and survival pathways and increased use of chimeric antigen receptor T-cell (CAR-T) therapy will like...
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: Masumi Ueda, Melvin Berger, Robert Peter Gale, Hillard M. Lazarus Tags: Review Source Type: research

Erythroleukemia-historical perspectives and recent advances in diagnosis and management
Acute erythroleukemia is a rare form of acute myeloid leukemia recognized by its distinct phenotypic attribute of erythroblastic proliferation. After a century of its descriptive history, many diagnostic, prognostic, and therapeutic implications relating to this unique leukemia subset remain uncertain. The rarity of the disease and the simultaneous involvement of its associated myeloid compartment have complicated in vitro studies of human erythroleukemia cell lines. Although murine and cell line erythroleukemia models have provided valuable insights into pathophysiology, translation of these concepts into treatment are no...
Source: Blood Reviews - September 18, 2017 Category: Hematology Authors: Prajwal Boddu, Christopher B. Benton, Wei Wang, Gautam Borthakur, Joseph D. Khoury, Naveen Pemmaraju Tags: Review Source Type: research

Patient-reported outcomes in acute myeloid leukemia: Where are we now?
Outcomes for acute myeloid leukemia remain poor, and treatment decisions must consider not just quantity, but also quality of life (QOL). We conducted a systematic review of studies in patients with acute myeloid leukemia or high-risk myelodysplastic syndrome that incorporated patient-reported outcome (PRO) measures. PubMed and PsycINFO were searched for articles published from January 2000 through June 2016. Forty-one were relevant for our review with more published in recent years. There was considerable inter-study heterogeneity in which instruments were used, and many studies employed multiple (often overlapping) instr...
Source: Blood Reviews - September 1, 2017 Category: Hematology Authors: Sarah A. Buckley, Kedar Kirtane, Roland B. Walter, Stephanie J. Lee, Gary H. Lyman Tags: Review Source Type: research

Insights into defective serological memory after acute lymphoblastic leukaemia treatment: The role of the plasma cell survival niche, memory B-cells and gut microbiota in vaccine responses
Acute lymphoblastic leukaemia (ALL) is the most common type of cancer in children, accounting for approximately 25% of childhood cancer cases. As a result of effective treatments over the past decades, paediatric ALL mortality has been greatly reduced. Chemotherapy, however, has a range of harmful side effects including the loss of protective antibodies against vaccine-preventable diseases. Since ALL survivors have an increased risk of health problems including organ insufficiencies, acquired vaccine-preventable infections subsequent to clinical remission could become life threatening to these individuals. (Source: Blood Reviews)
Source: Blood Reviews - August 25, 2017 Category: Hematology Authors: Shanie Saghafian-Hedengren, Ismael S öderström, Eva Sverremark-Ekström, Anna Nilsson Tags: Review Source Type: research

Haemophilia B: Where are we now and what does the future hold?
Research has been lacking on the natural history, complications, and treatment of haemophilia B, which is less common than haemophilia A and was recognized as a distinct clinical entity in 1947. Although the two diseases share the same clinical manifestations, they differ in causative mutation, risk of inhibitor development, and patient quality of life. Frequently debated is whether haemophilia B is as clinically severe as haemophilia A, with much of the published data on overall and haemophilia-specific health outcomes suggesting that haemophilia B may have a less severe clinical phenotype. (Source: Blood Reviews)
Source: Blood Reviews - August 16, 2017 Category: Hematology Authors: Gerry Dolan, Gary Benson, Anne Duffy, Cedric Hermans, Victor Jim énez-Yuste, Thierry Lambert, Rolf Ljung, Massimo Morfini, Silva Zupančić Šalek Tags: Review Source Type: research

Placenta growth factor mediated gene regulation in sickle cell disease
Sickle cell anemia (SCA) is an autosomal recessive disorder caused by mutation in the β-globin gene. Pulmonary hypertension (PH), a complication of SCA, results in severe morbidity and mortality. PH is a multifactorial disease: systemic vasculopathy, pulmonary vasoconstriction, and endothelial dysfunction and remodeling. Placenta growth factor (PlGF), an angiogenic growth factor, el aborated from erythroid cells, has been shown to contribute to inflammation, pulmonary vasoconstriction and airway hyper-responsiveness (AH) in mouse models of sickle cell disease. (Source: Blood Reviews)
Source: Blood Reviews - August 16, 2017 Category: Hematology Authors: Vijay K. Kalra, Shuxiao Zhang, Punam Malik, Stanley M. Tahara Tags: Review Source Type: research

T cell acute lymphoblastic leukemia (T-ALL): New insights into the cellular origins and infiltration mechanisms common and unique among hematologic malignancies
T-cell acute lymphoblastic leukemia (T-ALL) accounts for 15% and 25% of total childhood and adult ALL cases, respectively. During T-ALL, patients are at risk of organ infiltration by leukemic T-cells. Infiltration is a major consequence of disease relapse and correlates with poor prognosis. Transendothelial migration of leukemic cells is required to exit the blood stream into target organs. While mechanisms of normal T-cell transmigration are well known, the mechanisms of leukemic T-cell extravasation remain elusive; but involvement of chemokines, integrins and Notch signaling play critical roles. (Source: Blood Reviews)
Source: Blood Reviews - August 15, 2017 Category: Hematology Authors: Vadillo Eduardo, Dorantes-Acosta Elisa, Pelayo Rosana, Schnoor Michael Tags: Review Source Type: research