Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019045 (hemoglobinopathies)
 2,704 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a unique case of refractory acute myeloid leukemia (AML) precipitated by dengue virus induced marrow aplasia in a Chinese patient with hemoglobin (Hb) H disease. The quick temporal sequence of the three marrow abnormalities: hereditary hemoglobinopathy, reactive viral induced marrow change and malignant neoplastic process is highly unusual. Neither HbH disease nor viral induced marrow aplasia has known associations with AML. We propose that this unique case of AML may be caused by cytokine hyperstimulation in a stressed marrow.
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PMID:Acute myeloid leukemia precipitated by dengue virus infection in a patient with hemoglobin H disease. 1152 67

We studied anemia and bleeding risk among hematologic-oncologic pediatric patients with dengue infection. A total of 907 patients suspected of having dengue infection were included in the study. They were categorized into 2 groups: 1) patients with confirmed dengue infection (n=843) and 2) patients with other febrile illnesses (n = 64). Both groups included patients with underlying hematologic-oncologic diseases (55 vs 14) and without underlying disease (788 vs 50). Patients with underlying diseases were divided into 3 subgroups by risk: Subgroup A, anemia risk, including patients with thalassemia and hemoglobinopathies (n = 39) and G6PD deficiency (n=6); Subgroup B, patients with bleeding risk, including hemophilia (n = 7), von Willebrand disease (n = 1) and thrombocytopenia (n = 4); and Subgroup C, patients with anemia and bleeding risk, including oncologic diseases (n =12). Acute hemolysis in Subgroup A started during the febrile stage and required packed red cell transfusions. Bleeding risk in Subgroup B started during the early febrile stage with vasculopathy and continued to the late febrile stage with thrombocytopenia. These patients required factor concentrate and platelet concentrate transfusions. Anemia and bleeding risk in Subgroup C was greater among patients undergoing chemotherapy than those who had discontinued treatment. The greater the length of time since discontinuation of treatment, the lower risk. The case-fatality rate among dengue infected patients with underlying disease (2/55 = 3.64%) was significantly higher than those without underlying disease 0.63% (5/788).
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PMID:Dengue infection in hematologic-oncologic pediatric patients: aggravation of anemia and bleeding risk. 2308 83

Besides specific organisational requirements, the transfusional chain in French ultra-marine areas has specificities related to the epidemiology of infectious diseases and to population characteristics. We focus on some of these sociodemographic and medical peculiarities: the challenge of autosufficiency in relation to demographic trends; epidemiologic risks associated to emergent viruses such as dengue and Chikungunya, and the strategies that had been implemented to face last outbreaks; inappropriate selection criteria for eligibility to blood donation (biologic characteristics of Afro-Caribbeans not taken into account for the low hemoglobin deferral threshold; absence of guidelines for the screening of hemoglobinopathies AS/AC, present in 8% of the target population); specific indications for transfusion, such as platelet use in dengue fever or RBC transfusion in sickle cell disease. Due to the high polymorphism of erythrocyte antigens in Afro-Caribbeans, intra-ethnic transfusion facilitates compatibility for common antigens, but is responsible for the emergence of allo-antibodies difficult to identify in the absence of specific antisera or panels; molecular typing of erythrocyte antigens would allow detection of those patients at risk for immunization, expressing variant antigens or lacking high frequency antigens, as well as the characterization of RBC expressing immunogenic so called low frequency antigens. In an era of periodic emergence of new viruses in Europe (dengue, Chikungunya, West Nile virus...) and with the spreading of diseases with high transfusional requirements, such as sickle cell disease, ultra-marine services represent laboratories for the study of future trends and problems in transfusion medicine.
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PMID:[From donor to recipient: transfusion chain specificities in the French ultra-marine areas]. 2358 17

Infectious diseases and underlying medical conditions common to Africa may affect influenza frequency and severity. We conducted a systematic review of published studies on influenza and the following co-infections or co-morbidities that are prevalent in Africa: dengue, malaria, measles, meningococcus, Pneumocystis jirovecii pneumonia (PCP), hemoglobinopathies, and malnutrition. Articles were identified except for influenza and PCP. Very few studies were from Africa. Sickle cell disease, dengue, and measles co-infection were found to increase the severity of influenza disease, though this is based on few studies of dengue and measles and the measles study was of low quality. The frequency of influenza was increased among patients with sickle cell disease. Influenza infection increased the frequency of meningococcal disease. Studies on malaria and malnutrition found mixed results. Age-adjusted morbidity and mortality from influenza may be more common in Africa because infections and diseases common in the region lead to more severe outcomes and increase the influenza burden. However, gaps exist in our knowledge about these interactions.
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PMID:Potential Impact of Co-Infections and Co-Morbidities Prevalent in Africa on Influenza Severity and Frequency: A Systematic Review. 2606 16

Dengue fever and dengue hemorrhagic fever are re-emerging diseases that are endemic in the Tropics. The global prevalence of dengue cases has increased in South-East Asia, Africa, the Western Pacific, and the Americas. The increasingly widespread distribution and the rising incidence of dengue virus infections are related to increased distribution of Aedes aegypti, an increasingly urban population, and increasing air travel. Several Southeast Asian countries show that the age of the reported dengue cases has increased from 5-9 years, to older children and young adults. Dengue infection in adolescents and adults has also been recognized as a potential hazard to international travelers returning from endemic areas, especially SoutheastAsia. Dengue is one disease entity with different clinical presentations; often with unpredictable clinical evolutions and outcomes. Bleeding manifestations in adult patients, including petechiae and menorrhagia were also frequently found; however, massive hematemesis may occur in adult patients because of peptic ulcer disease and may not be associated with profound shock as previously reported in children. Although shock and plasma leakage seem to be more prevalent as age decreases, the frequency of internal hemorrhage rises as age increases. Increase in liver enzymes found in both children and adults indicated liver involvement during dengue infections. Pre-existing liver diseases in adults such as chronic hepatitis, alcoholic cirrhosis, and hemoglobinopathies may aggravate the liver impairment in dengue infection. Fulminant hepatitis is a rare but well described problem in adult patients with dengue infection. Currently, no specific therapeutic agent exists for dengue. The early recognition of dengue infection, bleeding tendency, and signs of circulatory collapse would reduce mortality rates in adult patients with dengue infection.
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PMID:DENGUE FEVER AND DENGUE HEMORRHAGIC FEVER IN ADULTS. 2650 34