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Genetic and acquired rare condition characterized by the presence of splenomegaly, mild thrombocytopenia, and progressive neurological disease (eg, ataxia, dementia and seizures). Morphological finding of sea-blue histiocytes in the bone marrow due to lipid storage diseases or hematological diseases.
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Sea-Blue Histiocytosis Disease; Niemann-Pick Disease Type F.
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The incidence is unknown; however, the presence of sea-blue histiocytes is a common abnormality of the bone marrow in myelodysplastic syndromes.
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Unknown. Sea-Blue Histiocytosis Syndrome is named for its characteristic macrophage. There are densely packed granules that stain blue with Wright-Giemsa stain. This syndrome is associated with abnormal storage of phosphosphingolipid and glycosphingolipid, which occurs mainly in the spleen and liver.
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Bone marrow examination reveals the characteristic cells. Also, a hepatosplenomegaly workup confirms the diagnosis for hepatosplenomegaly.
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Usually diagnosed before the age of 40 years. Hepatosplenomegaly, thrombocytopenia, macular abnormalities, pulmonary infiltrates on chest radiograph, patchy brownish-gray pigmentation of the upper body, occasional neurologic abnormalities (ataxia, dementia, seizures), predisposition for parasitic infections, and elevated bleeding times in albino patients with normal platelet counts.
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Precautions before anesthesia
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Check platelet count. Document neurologic abnormalities. Platelet availability.
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Anesthetic considerations
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Avoid central-neuraxial anesthesia in albino patient even with normal platelet counts (increased bleeding times) and patients with thrombocytopenia. Platelets should be transfused if platelet counts are less than 50,000/mm3, in presence of active oozing or bleeding and in albino patients.
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Pharmacological implications
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There are no specific pharmacological implications.
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Bermejo
N, Prieto
J, Arcos
MJ: Sea-blue histiocytosis in bone marrow of a patient with chronic thrombocytopenia.
Acta Haematol 133:277, 2015.
[PubMed: 25413372]
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Sawitsky
A: The sea-blue histiocyte syndrome, a review: Genetic and biochemical studies.
Semin Hematol 9(3):285, 1972.
[PubMed: 4114368]
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Wu
TT, Hoff
DS: Fish oil lipid emulsion-associated sea-blue histiocyte syndrome in a pediatric patient.
J Pediatr Pharmacol Ther 20:217, 2015.
[PubMed: 26170774]