Literature review and clinical observation of acquired idiopathic hemophilia with a new missense mutation in the factor VIII gene (His2026Arg)

The article provides review of possible mechanisms of inhibitor coagulopathies, in particular of acquired hemophilia A. This pathology is an extremely rare disease occurring in 1-2 cases in 1 million per year. In the present study we provide data for two clinical cases of hemophilia A in women. These cases had different development mechanisms, although both women have a newly discovered missense mutation His2026Arg in the VIII factor gene. The matter of main interest is the description of the disease development in the patient with an acquired idiopathic hemophilia A with a possible disease occurrence due to an asymmetric X-chromosome inactivation (lyonization). In this particular case lyonization led to the late manifestation of the hemophilia A carrier’s state and development of severe form of the inhibitor-associated acquired hemophilia A. We also discuss therapeutic approaches to these forms of the disease, considering there are no concise protocols for case management due to an extreme rarity of the pathology. Acquainting the clinical personnel working it the different areas of medicine with suchlike inhibitor coagulopathies has a major practical importance

Difficulties in diagnosing intestinal T-cell lymphoma. Case report

The article describes a rare diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), due to its veiled by a number of so-called masks of enteropathies. A detailed analysis of all clinical, morphological and immunohistochemical data made it possible to establish the correct diagnosis. The revealed pathology is extremely rare in practice, even among specialists in hematology. The article demonstrates the main stages of both a complex diagnosis and an attempt at therapy for this aggressive form of intestinal lymphoma

Cardiac involvement in lymphomas. Review of literature and case report of the clinical course of B-large-cell lymphoma

Clinical case of successful chemotherapy of patient with cardiac involvement in lymphoma, one of manifestations of which was the heart rhythm and conduction disorder, is presented as illustration. The data on the epidemiology of cardiac involvement in hematologic diseases, particularly in lymphomas and their clinical manifestations, modern diagnostic and treatment are presented. Clinical case of successful chemotherapy of patient with cardiac involvement in lymphoma, one of manifestations of which was the heart rhythm and conduction disorder, is presented as illustration.</p