Children sea blue histiocytosis

Children sea blue histiocytosis is how is it?

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.



Children sea blue histiocytosis Profile
Sea-blue histiocytosis (sea blue histiocyte syndrome) is a class of lipid catabolism enzyme abnormalities due to autosomal recessive genetic disease. Since sphingomyelin activity decreased in affected tissues and nerve sphingomyelin accumulation of glycolipids, bone marrow, liver, and spleen cells were staining was navy blue particles named, began in 1970. It was thought to be a variant of NPD.
Children sea blue histiocytosis Basics
Whether Medicare:Non-Medicare disease
Alias: Sea-blue histiocytes syndrome in children
Incidence of parts: whole body
Infectious:No relevant information
Multiple groups:No relevant information
Related symptoms: Hepatosplenomegaly, thrombocytopenia, skin pigmentation deepened hepatomegaly hepatic failure
Concurrent Disorders: Jaundice and cirrhosis thrombocytopenia
Pediatric Clinic sea-blue histiocytosis knowledge
Treatment department: Pediatric hematology
The cost of treatment: City Hospital about three (10000 - 50000 yuan)
The cure rate:0
treatment method: Surgical treatment of symptomatic treatment ..
Related tests: Bone marrow cell morphology EEG faculties liver ultrasonography
Commonly used drugs: Transfer Factor oral solution ... Transfer Factor oral solution ...
Sea-blue histiocytosis in children to the hospital must see
Best time to visit: No relevant information
Treatment duration: No relevant information
Frequency referral / treatment cycle: No relevant information
Treatment preparation: No relevant information


Children sea blue histiocytosis Profile
Sea-blue histiocytosis (sea blue histiocyte syndrome) is a class of lipid catabolism enzyme abnormalities due to autosomal recessive genetic disease. Since sphingomyelin activity decreased in affected tissues and nerve sphingomyelin accumulation of glycolipids, bone marrow, liver, and spleen cells were staining was navy blue particles named, began in 1970. It was thought to be a variant of NPD.
Children sea blue histiocytosis Basics
Whether Medicare:Non-Medicare disease
Alias: Sea-blue histiocytes syndrome in children
Incidence of parts: whole body
Infectious:No relevant information
Multiple groups:No relevant information
Related symptoms: Hepatosplenomegaly, thrombocytopenia, skin pigmentation deepened hepatomegaly hepatic failure
Concurrent Disorders: Jaundice and cirrhosis thrombocytopenia
Pediatric Clinic sea-blue histiocytosis knowledge
Treatment department: Pediatric hematology
The cost of treatment: City Hospital about three (10000 - 50000 yuan)
The cure rate:0
treatment method: Surgical treatment of symptomatic treatment ..
Related tests: Bone marrow cell morphology EEG faculties liver ultrasonography
Commonly used drugs: Transfer Factor oral solution ... Transfer Factor oral solution ...
Sea-blue histiocytosis in children to the hospital must see
Best time to visit: No relevant information
Treatment duration: No relevant information
Frequency referral / treatment cycle: No relevant information
Treatment preparation: No relevant information


Children sea blue histiocytosis What are the symptoms?

A symptom

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.



Sea-blue histiocytosis in children should be how to prevent?

First, prevention

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.



Sea-blue histiocytosis in children ate?


Children sea blue histiocytosis What is the 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.