Sea Blue Histiocytes


OLYMPUS DIGITAL CAMERA

Sea Blue Histiocytes are large lipid-containing histiocytes in the bone marrow which stain sea blue in color.

They may be found in a rare autosomal dominant condition characterized by neurological impairment and splenomegaly.

Sea-blue–colored histiocytes have been described in the setting of high rates of intramedullary cell death due to lipid storage diseases (e.g. Niemann-Pick disease), myelodysplastic syndromes (MDS), lymphomas, chronic myeloid leukemia, idiopathic thrombocytopenic purpura, autoimmune neutropenia, total parenteral nutrition and β-thalassemia major.

Sea-blue histiocytes are a common cytological feature in the bone marrow of patients with MDS. As other disorders frequently associated with marrow sea-blue histiocytes are relatively rare, MDS is probably the most common cause of this phenomenon in a northern European population.

The occasional sea-blue histiocyte may be a normal finding.

Sea Blue Histiocytosis (also known as inherited lipemic splenomegaly) is an extremely rare clinical syndrome associated with hypertriglyceridemia, splenomegaly, mild thrombocytopenia and bone marrow involvement. It is one of a group of related lipid disorders caused by certain changes in the APOE gene.  Many reports using the term “syndrome” are actually reporting just a microscopic finding.

Sea-Blue histiocytosis

Sea Blue Histiocytosis – BM Aspirate

There are currently no formal treatment guidelines. Management may involve the coordinated care of several different specialists including cardiologists, gastroenterologists, and hematologists. Patients with splenomegaly should be careful to avoid contact sports. Removal of the spleen (splenectomy) has been reported to make the condition worse.

References:

Candoni A, Grimaz S, Doretto P, Fanin R, Falcomer F, Bembi B (October 2001). “Sea-blue histiocytosis secondary to Niemann-Pick disease type B: a case report”. Ann. Hematol. 80 (10): 620–2. doi:10.1007/s002770100354.PMID 11732877

Bigorgne C, Le Tourneau A, Messing B, Rio B, Giraud V, Molina T, Audouin J, Diebold J. Br J Haematol. 1996 Nov; 95(2):258-62. Sea-blue histiocyte syndrome in bone marrow secondary to total parenteral nutrition including fat-emulsion sources: a clinicopathologic study of seven cases.

GARD https://rarediseases.info.nih.gov/diseases/8241/sea-blue-histiocytosis

Pongas, Georgios. Sea-blue–colored histiocytes associated with bone marrow granulomas. Blood. 2013; 122:475. http://www.bloodjournal.org/content/122/4/475?sso-checked=true. Accessed 12/9/2016.

Greyshock, Nicole, et al. APOE p.Leu167del-Related Lipid Disorders. GeneReviews. June 12, 2014; https://www.ncbi.nlm.nih.gov/books/NBK208534/

Summary
Sea Blue Histiocytes
Article Name
Sea Blue Histiocytes
Description
Sea Blue Histiocytes are large lipid-containing histiocytes in the bone marrow which stain sea blue in color.
Author
Publisher Name
askhematologist.com
Publisher Logo

Leave a Reply

Your email address will not be published. Required fields are marked *