Children sea blue histiocytosis
First, the causes of the symptoms is considered an autosomal recessive genetic disease, this symptom of unknown etiology. Sea blue histiocytosis syndrome, also known as sea-blue histiocytes no regional or ethnic-specific, age-specific nor, from infants to the elderly may be the disease in 1947 first discovered moeschlin tissue cells blue or dark blue sea of coarse particles. first described in 1954 by the sawitsky, 1970 年 silverstein et formally proposed the name of the symptoms .Blankenship other reported suffering from this there is a 3 per capita disease, it prompted an autosomal recessive genetic disease can be divided into primary and secondary categories clinically, secondary are often secondary to idiopathic thrombocytopenic purpura, chronic myeloid leukemia, children with chronic granuloma, high lipoprotein leukemia, Niemann -. Pick disease, thalassemia, hyperlipoproteinemia, multiple myeloma characterized by hepatosplenomegaly, thrombocytopenic purpura with mild bone marrow smears large number of navy organization cell.
Second, the pathogenesis
Common hepatosplenomegaly, thrombocytopenia, hemolytic anemia and other symptoms may be due to abnormalities or mild enzyme system overloading even normal enzyme system, leading to the body caused by the accumulation of abnormal metabolism of mucopolysaccharides.
Any onset age, from infants to the elderly are likely to .40 onset age of onset is more common. This process is similar to the clinical symptoms of Gaucher disease type chronic, insidious onset, longer duration, have hepatosplenomegaly. Splenomegaly usually more than liver, superficial lymph nodes enlargement, due to thrombocytopenia, visible skin purpura, a few patients have jaundice, perhaps because of phospholipid or glycolipid accumulation in the liver causing progressive liver failure, cirrhosis of the liver can occur even. yet skin pigmentation, rashes, eye spot area with white ring case .1 / 3, pulmonary infiltrates, like tuberculosis or sarcoidosis.
Second, the diagnostic criteria
If the patient clinical symptoms above, confirmed the need to check the presence of sea-blue tissue cells. Bone marrow cells found in a large number of sea-blue is an important basis for the diagnosis of the symptoms in determining the sea-blue histiocytes syndrome, the need to be further find the reasons why the exclusion after the secondary, can be diagnosed with primary sea-blue histiocytes syndrome.
1. Primary sick: There is no effective therapy for medical instruments appear splenomegaly, hypersplenism feasible splenectomy surgery, can improve the patient's symptoms, but need to consider the impact on patients after splenectomy surgery. especially for children and adolescents 2. secondary sexually transmitted diseases: for the symptomatic treatment of disease when the primary infection, bleeding, liver damage can be symptomatic treatment.
First, the treatment
1. Primary sick: There is no effective therapy for medical instruments appear splenomegaly, hypersplenism feasible splenectomy surgery, can improve the patient's symptoms, but need to consider the impact on patients after splenectomy surgery. especially for children and adolescents.
2. Secondary sexually transmitted diseases: for the symptomatic treatment of disease when the primary infection, bleeding, liver damage can be symptomatic treatment.