Primary immunodeficiency is a group of pathological conditions, mainly of an innate nature, in which there is a violation of the work of certain parts of the immune system. Symptoms vary, depend on the type of disease, mainly there is an increased susceptibility to bacterial and viral agents. Pathology is diagnosed by means of laboratory…
Immune hemolytic anemia is a group of diseases caused by increased destruction of red blood cells due to the production of antibodies against unchanged red blood cells or haptens that appeared on the erythrocyte membrane. There are isoimmune, transimmune, heterogeneous and autoimmune hemolytic anemia. Clinical signs: pallor or jaundice of the skin, moderate enlargement of…
Hemophagocytic lymphohistiocytosis is a group of congenital and acquired diseases resulting from disorders of the regulation of the immune response and characterized by hyperproduction of histiocytes, as well as cytotoxic T-lymphocytes. Clinical symptoms are manifested by febrile fever, enlargement of the liver and spleen, peripheral lymph nodes, damage to the nervous system, bone marrow and…
Secondary immunodeficiency is a disease of the immune system that occurs in children and adults, not associated with genetic defects and characterized by the development of repeated, prolonged infectious and inflammatory pathological processes that are poorly amenable to etiotropic treatment. There are acquired, induced and spontaneous forms of secondary immunodeficiency. Symptoms are caused by a…
Bullous pemphigoid is a chronic autoimmune skin disease affecting mainly older people. Its symptoms are similar to those of pemphigus and are reduced to the formation of tense blisters on the skin of the arms, legs, and abdomen; the distribution of pathological foci is usually symmetrical. Diagnosis of bullous pemphigoid is performed by examining the…
Eosinophilic fasciitis is a diffuse connective tissue disease characterized by lesions of deep fascia, muscles, subcutaneous tissue, skin and occurring against the background of eosinophilia and hypergammaglobulinemia. For the clinical picture of eosinophilic fasciitis, the skin is typically compacted by the type of “orange peel”, edema of the distal extremities, polyarthralgia, flexion contractures of the…
Mikulicz disease is a rare chronic disease, manifested by a symmetrical increase in all salivary and lacrimal glands. Among the possible etiological factors, the role of viral infection, as well as autoimmune processes leading to dystrophic lesions of the salivary and lacrimal glands is considered. More often the disease (syndrome) Mikulicz occurs in patients with…
Heavy chain diseases (HCD) are rare tumor diseases of a lymphoproliferative nature, in which the synthesis of defective immunoglobulins (with missing fragments of light chains) occurs. There are several variants of the disease – HCD-α, HCD-γ, HCD-µ and HCD- δ. Clinical manifestations are caused by damage to the lymphatic system and hematopoietic organs (enlargement of…
