Major Hematologic Diseases in the Developing World— New Aspects of Diagnosis and Management of Thalassemia, Malarial Anemia, and Acute Leukemia

Abstract

The three presentations in this session encompass clinical, pathophysiological and therapeutic aspects of hematologic diseases which impact most heavily on developing world countries. Dr. Victor Gordeuk discusses new insights regarding the multi-faceted pathogenesis of anemia in the complicated malaria occurring in Africa. He describes recent investigations indicating the possible contribution of immune dysregulation to this serious complication and the implications of these findings for disease management. Dr. Surapol Issaragrisil and colleagues describe epidemiologic and clinical characteristics of the thalassemic syndromes. In addition to being considered a major health problem in Southeast Asia, the migration throughout the world of people from this region has caused the disease to have global impact. A unique thalassemia variant, Hb Eβ-thalassemia, with distinctive clinical features, has particular relevance for this demographic issue. Special focus will be reported regarding recent prenatal molecular screening methods in Thailand which have proven useful for early disease detection and disease control strategies. Dr. Raul Ribeiro describes a clinical model for providing effective treatment for a complex malignancy (childhood acute lymphoblastic leukemia) in countries with limited resources. With the multidisciplinary approach in Central American of the joint venture between St. Jude Children's Research Hospital International Outreach Program and indigenous health care personnel, major therapeutic advances for this disease have been achieved. Given the major demographic population shifts occurring worldwide, these illnesses also have important clinical implications globally. These contributions demonstrate that lessons learned within countries of disease prevalence aid our understanding and management of a number of disorders prominently seen in developed countries. They will show how effective partnerships between hematologists in more and less developed nations may work together to produce important advances for treating major hematologic diseases in less developed regions. A major focus relates to the socio-economic and medical burden of these diseases in developing countries with limited resources. As such, these problems provide a challenge and an opportunity for collaborative interaction between hematologists and policy makers worldwide.

Induction of human fetal hemoglobin via the NRF2 antioxidant response signaling pathway

“…Despite advances in iron chelation therapy, median survival has been estimated to be 49 years for patients who use optimal chelation regimens, whereas a median survival of only 28 years was estimated for "typical" compliance to chelation schedules. 7 Greenberg et al, 8 in recent studies in Southeast Asia, estimate life expectancy in ␤-thalassemia to be Ͻ 10 years for patients without access to transfusion and chelators.…”

“…Despite advances in iron chelation therapy, median survival has been estimated to be 49 years for patients who use optimal chelation regimens, whereas a median survival of only 28 years was estimated for "typical" compliance to chelation schedules. 7 Greenberg et al, 8 in recent studies in Southeast Asia, estimate life expectancy in ␤-thalassemia to be Ͻ 10 years for patients without access to transfusion and chelators.Although hematopoietic stem cell transplantation 9 and gene therapy 10 have the potential to cure the ␤-hemoglobinopathies, neither is currently applicable to most patients with these diseases because of technical issues, cost, and a lack of the highly sophisticated medical care necessary to provide these therapies in the areas of the world in which most patients live. An alternative approach is pharmacologic induction of fetal Hb (HbF).…”

Cardiac Iron Determines Cardiac T2*, T2, and T1 in the Gerbil Model of Iron Cardiomyopathy

“…1,2 Chronic blood transfusion produces progressive tissue iron loading and toxicity, which results in severe endocrine, liver, and cardiac failure. Iron chelation with deferoxamine has dramatically improved length and quality of life for thalassemia patients; however, cardiac complications remain common.…”

Antimalaria assay of fruit extract of Morinda citrifolia and activity of mice (Mus musculus) macrophage after infecting it with Plasmodium berghei

“…Peningkatan aktivitas makrofag mencit setelah diberikan ekstrak buah M. citrifolia disebabkan adanya peningkatan sekret sitokin yang dihasilkan oleh sel-sel imunokompeten, antara lain Interleukin-1β Greenberg et al, (2001) sel T helper tipe 1 (Th1) pada saat infeksi malaria mensekresi sitokin antara lain IFN-γ, IL-2, dan TNF-α. Dengan adanya sitokinsitokin yang merupakan faktor pengaktif sel makrofag, maka aktivitas fagositosis makrofag dapat ditingkatkan (Gambar 2).…”