Phosphoglyceride crystal deposition disease

Abstract

An extremely rare phosphoglyceride deposition disease is reported. A healthy 62-year-old Japanese woman suffered from tumors that repeatedly appeared in injured soft tissues for more than 20 years. No immunologic disorders or abnormal laboratory data were found. Histology showed foreign body granulomas consisting of macrophages surrounding yellowish-white crystals. The crystals were weakly positive by von Kossa's method, were dissolved in 30% acetic acid with gas, and were easily dissolved in 0.1 N NaOH or potassium hydroxide, losing their crystal structure. Using a scanning electron microscopy X-ray microanalyzer, phosphorus and calcium peaks were detected. Phosphoglycerides were detected by microscopic infrared spectrophotometry and microsampling mass spectrometry. The gold hydroxamic acid method for detecting phosphoglyceride showed strong positive staining in the crystals. Based on the above analyses, the deposited crystals were regarded as phosphoglyceride, which bound calcium as a counter ion. The crystals tended to be deposited at sites of injury, where macrophages had accumulated. The patient had received many injections of a medicine made from alcohol extract from bovine liver. We suspect that this medicine was related to the cause of the deposition as the deposition repeatedly appeared at the site of the injections.

Phosphoglyceride (PG) crystal deposition disease: A novel acquired disease in which PG crystals are deposited in injured soft tissue and bone with phospholipid metabolism disturbance

“…The bands at 1741 and 1719 correspond to C=O (ester) stretching vibration, and bands at 1644 correspond to C=O (amido) stretching vibration. These absorption bands confirmed the presence of PG, as previously reported 2 …”

“…In experiments that tested the reactions of the PG crystals to acid and alkaline solutions, crystals dissolved in 30% acetic acid with production of gas that was confirmed to be oxygen by gas chromatography. This is a characteristic unique to PG and is not shared by other crystals found in the human body 2 . The crystals also dissolved easily in 0.1 N NaOH or KOH, and dissolved only slightly in 0.1 N HCl.…”

“…Foreign body granulomas form surrounding the crystals. To date, only three cases have been reported in English literature 1–3 . In all cases, patients were older than 50 years and the tumors gradually increased in size over more than 10 years.…”

Phosphoglyceride Crystal Deposition Disease in the Spine

“…As described in Table 1, only three cases of phosphoglyceride crystal deposition disease, including this one, have been reported. [1][2][3] In all three cases, the disease occurred in a site made susceptible to invasion by a previous injury. In our patient's case, the site was probably made susceptible as a result of the lumbar anesthetic given at the time of the appendectomy.…”

“…4,5 Because giant cell granulomas occurred at the sites of intramuscular injections of Nibenal in our patient, Nibenal is assumed to be responsible for the disease. 1 Macrophages at the injected sites might take up the liver extract (Nibenal) and slowly degrade them. Some macrophages may remain there, and others migrate into the whole body.…”