PMC7184092_01
PMC7184092_02
(A) Light microscopy image. No proliferative changes in the glomeruli were noted, but progression of tubulointerstitial injury was seen [left: Periodicacid-methenamine-silver (PAM) staining x100]. Glomerular podocytes were expanded, and fine vacuolated changes were noted (bottom left: inset x400). (B) Light microscopy image. A toluidine blue-stained Epon resin-embedded section shows the granular deposition of inclusion corpuscles in the podocytes but not in the tubular epithelium (right: toluidine blue staining x200). (C) (D) Electron microscopy image. Numerous osmiophilic, lamellated membrane structures, with a concentric pattern called myelin bodies or with elongated stripes called zebra bodies, were mainly found in the podocytes (C) and the cytoplasm of the vascular smooth cells (D) (C: podocytes x2,000, D: vascular tunica media x1,500, inset: x3,000).
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High rates of glomerulosclerotic lesions (approximately 75%) and interstitial fibrosis (approximately 60%) indicated disease progression (Fig. 1). On electron microscopy, numerous myeloid bodies and zebra bodies were found in the glomerular podocytes and vascular smooth muscles (Fig. 1).
Clinical course with eGFR and the count of Mulberry cells in two cases. (A) Case 1: Despite long-term ERT, the numbers of Mulberry bodies and cells remained almost constant. (B) Case 2: The numbers of Mulberry bodies and cells decreased promptly after initiating ERT, and the Mulberry cells disappeared altogether after five months. eGFR: estimated glomerular filtration rate, ERT: enzyme replacement therapy, UP/Cr: urinary protein per creatinine, gCr: gram creatinine
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Her proteinuria decreased, and her renal function was stable during the observation period (Fig. 2). Mulberry cells disappeared after initiating ERT (Fig. 2). Morphologically, numerous Mulberry bodies or fat globules in typical whirls and annular ring shapes were found inside and outside the Mulberry cells in Case 1.
PMC7184092_03
Clinical course with eGFR and the count of Mulberry cells in two cases. (A) Case 1: Despite long-term ERT, the numbers of Mulberry bodies and cells remained almost constant. (B) Case 2: The numbers of Mulberry bodies and cells decreased promptly after initiating ERT, and the Mulberry cells disappeared altogether after five months. eGFR: estimated glomerular filtration rate, ERT: enzyme replacement therapy, UP/Cr: urinary protein per creatinine, gCr: gram creatinine
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Her proteinuria decreased, and her renal function was stable during the observation period (Fig. 2). Mulberry cells disappeared after initiating ERT (Fig. 2). Morphologically, numerous Mulberry bodies or fat globules in typical whirls and annular ring shapes were found inside and outside the Mulberry cells in Case 1.
Morphology of Mulberry bodies and cells. (A) (D) Case 1 (during ERT): Numerous Mulberry cells with typical whirl-shaped and annular ring-shaped fat globules (Mulberry bodies) were found despite many years of treatment (A). Numerous free Mulberry bodies of similar morphology were also found (D). (B) (E) Case 2 (before ERT): Whirl- and ring-shaped fat globules (Mulberry bodies) were found in the Mulberry cells (B). Free Mulberry bodies also formed whirl-shaped structures, annular ring-shaped, and ring-shaped morphologies (E). (C) (F) Case 2 (6 months after initiating ERT): The proportion of whirl-shaped Mulberry bodies inside Mulberry cells decreased, and the proportion of ring-shaped Mulberry bodies increased (C). The majority of free Mulberry bodies were of ring-shaped morphology (F).
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However, six months after the initiation of therapy, the numbers of whirl-shaped Mulberry bodies in Case 2 decreased, and the numbers of ring-shaped Mulberry bodies increased (Fig. 3).