The Fine Structure of Blood Cells in Ceroid-Lipofuscinosis (Spielmeyer-Vogt's Disease)

Abstract

Peripheral blood and bone marrow in a case of juvenile type of Spielmeyer-Vogt's disease were examined with the light and electron microscope for the occurrence of curvilinear profile (CLP), rectilinear profile (RLP), finger-print profile (FPP) and other inclusions.

A large quantity of parallel tubular arrays (PTAs) and a few CLPs, RLPs and FPPs were present in the cytoplasm of the lymphocytes. In addition, many plasma cells in the bone marrow contained FPPs, RLPs and CLPs in the vacuoles of their cytoplasm. The other relevant inclusions were observed in the cytoplasm of the bone marrow cells. Moreover, amorphous or membranous inclusions with rectilinear or curvilinear profiles were seen in some vacuoles in the cytoplasm of many erythrocytes.

The incidence of these characteristic inclusions in the plasma cells or the erythrocytes were not described for the present. The correlation and the diagnostic significance of these characteristic inclusions were discussed.

Zusammenfassung

Periphere Blut- und Knochenmarkszellen bei einem Patienten mit juveniler Zeroid-Lipofuszinose enthielten kurvilineäre Körperchen (CLB), rektilineäre Körperchen (RLB), Fingerabdruck-körperchen (FPP) und andere Einschlüsse.

Im Zytoplasma der Lymphozyten fanden sich zahlreiche „parallel tubular arrays” und wenige CLB, RLB und FPP. Sie waren ebenfalls in den zytoplasmatischen Vakuolen vieler Plasmazellen zu finden.

Andere zytoplasmatische Einschlüsse lagen in Knochenmarkszellen. Zusätzlich enthielten einige Erythrozyten pleomorphe und membranöse Einschlüsse, von denen einzelne zytoplasmatische Vakuolen auch rektilineäre Profile aufwiesen.

Über derartige charakteristische Einsclüsse in Plasmazellen und Erythrozyten ist bisher nicht berichtet worden. Die Korrelation und die diagnostische Bedeutung dieser charakteristischen Einschlüsse werden diskutiert.

Keyword

Lymphocyte - plasma cell - erythrocyte - curvilinear profile - rectilinear profile - fingerprint - profiles juvenile ceroid-lipofuscinosis