Anemia Investigation Reveals a Primary Sea-Blue Histiocyte Syndrome

Abstract

Sea-blue Histiocyte Syndrome (SBHS) is a rare and poorly understood systemic histiocytosis that is sometimes associated with haematological and lipid storage diseases as well as other miscellaneous conditions, but in most cases its cause is unknown. Patients often have very disparate clinical features but share the same histological findings of sea-blue histiocytosis in the bone marrow, i.e. characteristic lipid-laden macrophages with deepblue or blue-green granules when stained with Romanovsky or May-Grunwald Giemsa stain. We describe a case of an 8 yearold girl admitted to the emergency department with a history of fever, adynamia, subicteric sclerotics, lymphadenopathies, anemia and leukopenia. During the investigation, histological examination of the bone marrow revealed a Sea blue histiocytosis. Patient workup revealed a spherocytosis, but it was inconclusive regarding major causes for these findings such as Niemann-Pick Disease, Gaucher Disease and other haematologic conditions, and was thus considered a Primary Sea-Blue Histiocyte Syndrome. A review of the literature was then carried out to outline current knowledge of this condition and to establish a rationale for the patient workup and management when such findings are encountered.

Apoe as a Risk Factor for Alzheimer's Disease and Lipid Metabolism Disorders: Molecular Analysis

“…The disease, which is also called lipemic splenomegaly, takes its name from the fact that when stained under a microscope, some histiocytes appear light blue [22]. Characteristic symptoms of the disease Health Problems of Civilization eISSN: 2354-0265, ISSN: 2353-6942 are increased levels of triglycerides and enlarged spleen [23]. Additionally, a low number of platelets -thrombopenia, as well as abnormal functioning of the liver and heart are observed [22].…”

“…This is the receptor-binding area of the APOE molecule [24]. The manifestation of the disease is influenced by gender, lipid concentration and the presence of other diseases [23].…”