Immune Thrombocytopenic Purpura ITP — A clinical syndrome where a decreased number of platelets causes bleeding, and easy bruising. ITP Science includes information about diagnosing ITP, treatment goals, as well as helpful information for patients recently diagnosed with the disorder. Essential Thrombocytosis — A disorder in which platelets are overproduced, which can lead to both blood clotting and bleeding.
Clotting Disorders — Problems affecting the ability to clot blood, leading to excessive bleeding or excessive clotting. The Coagulation Factors site features articles and news on coagulation disorders, and also includes a health directory and information on clinical trials for coagulation disorder patients.
Hemophilia — A bleeding disorder caused by a problem in one of the factors of blood clotting. The National Hemophilia Foundation, focusing on three major strategic initiatives: research support and promotion; health education and training; and advocacy and community service.
Von Willebrand Disease — A hereditary disease where there is a deficiency of the von Willebrand factor, which is a factor that affects platelet function. This often leads to excessive bleeding. Hemochromatosis — A disorder where patients absorb extra amounts of iron from their daily diet and over time. Normal hemoglobin cells can live up to days. Sickle cells risk being destroyed by the spleen because of their shape and stiffness.
The spleen helps filter the blood of infections. Sickle cells get "stuck" in this filter and die. Due to the decreased number of hemoglobin cells circulating in the body, a person with sickle cell is chronically anemic. The spleen also suffers damage from the sickle cells, which block the healthy oxygen-carrying cells. After repeated blockages, the spleen is very small and does not work properly.
Without a functioning spleen, these people are more at risk for infections. Infants and young children are at risk for life-threatening infections. Treatment includes prompt emergency care for fevers and infections, appropriate vaccinations, penicillin, and management of anemia. Tay-Sachs disease is a fatal disorder in children usually by age 5 that causes a progressive degeneration of the central nervous system. It is caused by the absence of an enzyme called hexosaminidase A or hex A.
Without hex A, a fatty substance builds up on the nerve cells in the body, particularly the brain. The process begins early in pregnancy when the baby is developing. It is not apparent until several months after the birth. Currently, clinical trials are investigating the safety and efficacy of gene therapy. Despite all of these advances, the life expectancy of somebody with sickle cell is 30 years shorter than the general U. The multi-disciplinary panel presentation at ASH gave participants an opportunity to hear about the challenges facing these patients and families.
The overview of new and emerging treatment options gave providers an understanding of treatment options. Patients can participate in two national registries in order to collect aggregate data that are used to identify trends that impact bleeding disorder patients. We conclude that it is about a post-circumcision bleeding due to major hemophilia A associated to sickle cell AS, this association was a systematic discovery. Haemophilia A is an inherited bleeding disorder, inherited in an X-linked recessive pattern, responsible of a deficiency in anti-haemophilic factor A factor VIII , with an incidence of 1 in Sickle cell disease is a genetic disorder of hemoglobin that is transmitted as an autosomal recessive disease.
It is one of the most common hereditary diseases in the world, affecting black populations especially in tropical Africa. Sickle cell trait is the heterozygous form of sickle cell disease, it does not display the severe symptoms of sickle-cell disease [ 3 , 4 ].
Combination the coexistence of Hb AS disease and haemophilia A must be exceedingly rare, while one is an X-linked disorder and the other a chromosomal disorder. Because of this rarity, we report the case of two brothers with concomitant Hb AS disease and major haemophilia A. No hemostasis assessment being carried out before the act. On admission, the brothers were conscious and had a good condition. There were no familial antecedents.
After stopping bleeding, blood test was requested for both brothers. For M. None of the children has received a blood transfusion. Haemophilia is a rare bleeding disorders, usually inherited, and as it is X-linked disease only occurring in males. The incidence of hemophilia A is times higher than of hemophilia B. The gene defect is a known mutation of a single nucleotide of the globin gene of the hemoglobin, which results in glutamate being substituted by valine at position 6 [ 3 , 4 ].
This mutation affects the syntheses of hemoglobin beta chain with formation of abnormal hemoglobin HbS , responsible for microcirculation obstruction, ischemia, tissue necrosis and systemic organ dysfunction in the homozygote form SCD [ 3 , 4 ]. Individuals with SCT affected have normal life expectancy. It is usually an asymptomatic carrier without severe symptoms of SCD [ 3 , 4 ]. More, The SCT confers survival advantage by providing resistance against severe malaria in the tropics.
The coexistence of SCD or SCT and haemophilia is an uncommon association ant it is rarely described in literature, even in predominantly black populations, in which the prevalence of both conditions is higher, probably due to the small number of cases. We have found few associations of sickle cell disease with hemophilia; Glenn and all has reported in the literature the concurrence of hemophilia A and homozygous sickle Hb SS cell disease in a year-old black male [ 5 ].
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