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Amit R Rahalkar, Jian Wang, Sandra Sirrs, James Dimmick, Daniel Holmes, Nadine Urquhart, Robert A Hegele, Andre Mattman, An Unusual Case of Severe Hypertriglyceridemia and Splenomegaly, Clinical Chemistry, Volume 54, Issue 3, 1 March 2008, Pages 606–610, https://doi.org/10.1373/clinchem.2007.097139
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CASE
A 49-year-old man of Japanese and British ancestry was referred to a metabolic diseases clinic for evaluation 5 months after nontraumatic spleen rupture requiring splenectomy. Prior history included hypertension and mild frontal headaches, but no other neurological or cardiovascular symptoms. The patient did not smoke and used alcohol infrequently. His mother had coronary artery disease, and his father had mild hypertension. There was no family history of consanguinity, splenomegaly, diabetes, or developmental delay.
The ruptured spleen weighed 727 g, and splenomegaly was associated with marked sinus histiocytosis spreading apart the lymphoid component. The overwhelming majority of histiocytes were foamy (Fig. 1a ), and only a few had sea-blue appearance and reacted positively with periodic acid-Schiff (PAS), PAS and diastase, and May-Giemsa stains. A lipid storage disorder was suspected, but the histiocytes did not have the cytoplasmic linearity appearance of Gaucher cells and were otherwise nonspecific. Before splenic rupture, the patient’s lipoprotein profile was reported as being normal, with no past recorded triglyceride measurement exceeding 2 mmol/L.
