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Sea-blue histiocytosis
Mónica Bronte Anaut1, Javier Arredondo Montero2
1Pathology Department, Hospital Universitario de Araba, Vitoria, Spain.
Insights
Sea-blue histiocytosis is a rare disorder causing organ enlargement and low platelets. Diagnosis involves identifying ceroid deposits in tissues, as seen in this autopsy case.
Area of Science:
- Cardiovascular Pathology
- Histopathology
- Rare Diseases
Background:
- Hypertrophic cardiomyopathy is a significant cardiac condition.
- Surgical interventions like septal myomectomy and valve replacement carry risks.
- Sea-blue histiocytosis is an exceptionally rare disorder.
Abstract:
A 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in granules in PAS and Giemsa stains should establish the diagnosis of suspicion.
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