Institute Hematologia e Inmunologia

Cuba

Institute Hematologia e Inmunologia

Cuba

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Alfonso Valdes M.E.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2016

Behcet’s syndrome, also known as Behcet’s disease is a chronic autoinflammatory process of low frequency and unknown etiology. It is a vasculitis affecting arteries and veins of all sizes that causes an alteration of endothelial function and is expressed clinically by organ damage at various levels. It has a higher incidence in countries located in the ancient Silk Road (East Mediterranean, Middle East and East Asia). Its pathogenesis involves genetic, microbial and immunological factors.Symptoms vary from person to person and according to geographical regions, but the most common are oral and genital ulcers, eye inflammation (uveitis, iritis and retinitis), skin lesions and arthritis. In some cases they can present central nervous system, gastrointestinal tract and kidney. As a guide to the diagnosis criteria of International Study Group of Behcet’s disease are used. Treatment includes topical and systemic drugs. The most commonly used are steroids, tumor necrosis factor inhibitors and immunosuppressants. © 2016, Editorial Ciencias Medicas. All rights reserved.


Interferon a (IFN-a), is used together with other antivirals in the treatment of chronic hepatitis C. Autoimmune disorders have been described during its use, particularly thyroid diseases and type 1 diabetes mellitus (DM1), among others. We report the case of a patient with chronic hepatitis C, showing severe symptoms of coma, metabolic ketoacidosis, hyperglycemia and dehydration after 6 months of treatment with IFN-aand ribavirin. The beginning of a secondary DM 1 at the administration of IFN-a was diagnosed. There was no personal or family history of genetic factors predisposing to the development of this disease. The patient was treated in an Emergency Medical Unit (MEU) where medication to control its manifestations was received. Administration of IFN-a was suspended and the ambulatory treatment is maintained, applying insulin in fractional doses adjusted to blood glucose values.


Abraham C.M.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2015

Histocompatibility is the area of Immunology for the study or typing of antigens expressed in the surface of leukocytes, of high polymorfirsm, genetically determined by different allelic variants, implied in organs and tissues reject due to its high level of immunogenicity. The development of Immunology in Cuba since the 1970´s has allowed the gradual introduction of technologies that made it possible to type these antigens and the development of hematopoietic and kidney transplants in our country. The use of molecular biology in this area has made it possible the most precise identification of existing and new antigenic variants previously not known, which have contributed to enlarge new possibilities for hematopoietic transplant to non related donors and with blood cord cells, which increased the options for cure in different diseases. © 2015, Editorial Ciencias Medicas. All rights reserved.


Lavaut Sanchez K.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2014

Hemophilia is ahereditary bleeding disorderX-linkedthat arises due to mutationsin the genes of factor VIII (hemophilia A) and factor IX (hemophilia B), which causes a decrease or functional deficiency of these proteins in plasma. Their frequencies are 1 in 5 000 males and 1 in 30 000 live births, respectively. It affects males almost exclusively, and female carriers have a 50 % risk of transmitting the disease to their children. So, it is importantin families with history of hemophilia that carriers are identified through genetic counseling, which provides information about the disease, making pedigree, calculation of therisk of recurrence, molecular diagnosis and possibility of prenatal diagnosis inpregnant carriers. It is imperative that genetic counseling constitutes an educational and informative process, never as an imposition.


Coagulogram comprises a set of tests, which explore the participation of all components of hemostasia: vascular endothelium, platelet activity, plasma and fibrinolytic factors. Often, the technical staff finds complex to do these tests, so deepening knowledge, understanding, and interpreting the results of each of these tests should result in strengthening and training of health professionals. This paper describes the main conventional coagulation tests, the beginning and the reference values of each of them, and the possible diseases according to the alteration of the hemostatic system corresponding to the alteration of coagulation, with the aim of providing medical background information for the proper performance and proper interpretation of results.


Castillo-Gonzalez D.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2012

The sporadic hemophilia prevalence was estimated more than 40 years ago and it was shown that approximately a third of the cases are novo. Most of the mutations that occur in hemophilia are produced during the male spermatogenesis; in other cases, they occur in early stages of the embrión development or in the mother a germinal mutation. The X-cromosoma- inactivation process is at random. Extended studies have shown that male meiosis are more frequent than female ones, with a global proportion of 3,5/1, specially introns inversions 22 and 1. There were revised molecular and biochemical aspects of factors VIII and IX. We ruled out the importance of B domain in factor VIII, which contributes to multiple essential functions, as the quality control of synthesis, secretion, union with platelet phospholipids, inactivation and complete clearance of the molecule.


Viral reactivation predominantly occurs in the first 3 - 6 months after stem cell transplantation. The most clinically important are the herpes viruses; cytomegalovirus, Epstein-Barr virus and human herpes virus 6; the polyoma viruses BK and JC and adenovirus. Cellular adoptive immunotherapy is a treatment used to help immune system to fight against this kind of diseases. It is based on the extraction of specific T lymphocytes from an individual and its culture at the laboratory, to increase the number of T cells able to destroy viral targets, and these T cells are infused to the patient in order to help his immune system. In vitro experimental data suggest the high and perhaps unique potential of cord blood cells to generate biologically active products as therapeutic tools of this new type in order to facilitate graft, immunotherapy and regenerative medicine. A naive T cell phenotype derived from cord blood has been employed to reduce graft versus host disease after stem cell transplantation and as adoptive cellular immunotherapy with virus-specific cytotoxic T lymphocytes to prevent, in an effective way, this kind of viral infections. © 2016, Editorial Ciencias Medicas. All rights reserved.


Forrellat Barrios M.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2016

Human existence is indissolubly linked to iron, which is part of a wide variety of key enzymes such as catalase, aconitases, ribonucleotide reductase, peroxidases and cytochromes, exploiting the flexibility of its redox chemistry to run a large number of reactions essential for life. Human body has evolved to keep iron in different forms, including recycling after rupture of erythrocytes and the retention without excretion mechanism. Iron metabolism is balanced by two regulatory systems: one based on systemic hormone hepcidin protein export and ferroportin, and the other, which controls cell metabolism through the iron regulatory protein (IRP) binding to the mRNAs regulated iron regulatory elements (IRE). These systems work in a coordinated manner avoiding both deficiency and excess. © 2016, Editorial Ciencias Medicas. All rights reserved.


Ustariz Garcia C.R.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2016

Hereditary angioedema is a rare disease with autosomal dominant inheritance that is characterized by edema in skin and mucosa of various organs, mainly gastrointestinal tract and the respiratory system. Clinical manifestations may be mild or severe, depending on their location and intensity. The most severe forms are edema of the glottis, and the edema of gastrointestinal tract which can cause severe dehydration and abdominal pain that can be confused with an acute abdomen and unnecessary surgery. The edema is characterized by not being itchy, no temperature rise, non-marking when pressed and usually have a family history. No predilection for sex, or skin color. Its diagnosis is necessary to make a thorough examination and additional tests of the complement system. They described two classic forms called HAE type I and type II, type I is the most common. Recently it described the type III that occurs only in females, without quantitative or qualitative alteration of C1 inhibitor and is associated with the consumption of drugs or oral contraceptives containing estrogen. The treatment is based primarily on the use of attenuated androgens or antifibrinolytic and avoiding risk factors if they are known. In cases of acute conditions present you can use fresh frozen plasma and purified C1 inhibitor concentrate (Berinert-500) for intravenous use and rapid response; but the use of subcutaneous epinephrine may not be very effective. Steroids and antihistamines have no effectiveness when used in these patients. © 2016, Revista Cubana de Hematologia Inmunologia y Hemoterapia. All rights reserved.


Garcia S.M.,Institute Hematologia e Inmunologia
Revista Cubana de Hematologia, Inmunologia y Hemoterapia | Year: 2016

The development of molecular biology and the application of highly sensitive techniques have allowed better understanding genetic basis of hereditary diseases and use them to prevent, diagnose and treat. In Cuba hemoglobin S trait is the 3.08% in the general population and until 17% in African descent, which represents a public health problem and since the 90' prenatal diagnosis of the disease is made, fulfilling the ethical principles of autonomy, beneficence, non-maleficence and justice. The most important ethical conflict arises when is questioned who benefits from information obtained from prenatal diagnosis: the fetus, parents or society. Prenatal diagnosis of sickle cell disease in Cuba has an important scientific and social impact, and requires serious work from primary health care, providing scientific and technical support for the venture the couple of risk and their future son while respecting their bioethical rights. © 2016, Revista Cubana de Hematologia Inmunologia y Hemoterapia. All rights reserved.

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