Sea-Blue Histiocyte Syndrome (Sea Blue Histiocytosis)

A congenital disease caused by an inborn error involving APOLIPOPROTEINS E leading to abnormal LIPID METABOLISM and the accumulation of GLYCOSPHINGOLIPIDS, particularly SPHINGOMYELINS in the HISTIOCYTES. This disorder is characterized by SPLENOMEGALY and the sea-blue histiocytes in the spleen and bone marrow after May Grunwald staining.
Also Known As:
Sea Blue Histiocytosis; Histiocyte Syndrome, Sea Blue; Histiocyte Syndromes, Sea Blue; Sea-Blue Histiocyte Disease; Disease, Sea-Blue Histiocyte; Diseases, Sea-Blue Histiocyte; Histiocyte Disease, Sea-Blue; Histiocyte Diseases, Sea-Blue; Histiocyte Syndrome, Sea-Blue; Histiocyte Syndromes, Sea-Blue; Histiocytoses, Sea Blue; Histiocytoses, Sea-Blue; Histiocytosis, Sea Blue; Sea Blue Histiocyte Disease; Sea Blue Histiocyte Syndrome; Sea Blue Histiocytoses; Sea-Blue Histiocyte Diseases; Sea-Blue Histiocyte Syndromes; Sea-Blue Histiocytoses; Sea-Blue Histiocytosis; Syndrome, Sea-Blue Histiocyte; Syndromes, Sea-Blue Histiocyte; Histiocytosis, Sea-Blue
Networked: 17 relevant articles (0 outcomes, 2 trials/studies)

Disease Context: Research Results

Related Diseases

1. Splenomegaly
2. Niemann-Pick Diseases (Niemann Pick Disease)
3. Type C Niemann-Pick Disease (Niemann Pick Disease, Type C)
4. Dyslipidemias (Dyslipidemia)
5. Histiocytic Necrotizing Lymphadenitis (Kikuchi Disease)


1. Akar Özkan, Eylem: 1 article (04/2015)
2. Haberal, Mehmet: 1 article (04/2015)
3. Gerçeker, Funda: 1 article (04/2015)
4. Boyvat, Fatih: 1 article (04/2015)
5. Özdemir, B Handan: 1 article (04/2015)
6. Ayva, E ┼×ebnem: 1 article (04/2015)
7. Serre, Valérie: 1 article (01/2013)
8. Ouguerram, Khadija: 1 article (01/2013)
9. Leren, Trond Paul: 1 article (01/2013)
10. Boileau, Catherine: 1 article (01/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sea-Blue Histiocyte Syndrome:
1. Immunoglobulin M (IgM)IBA
2. Immunoglobulin G (IgG)IBA
3. CeroidIBA
4. Apolipoproteins E (ApoE)IBA
5. Sphingomyelin Phosphodiesterase (Sphingomyelinase)IBA
6. HDL CholesterolIBA
7. EnzymesIBA
9. Amyloid (Amyloid Fibrils)IBA
10. Sphingomyelin lipidosisIBA

Therapies and Procedures

1. Total Parenteral Nutrition
2. Splenectomy
3. Parenteral Nutrition
4. Home Parenteral Nutrition