Cerebral low-grade lymphoma and light chain deposition disease: exceedingly high IgG levels in the cerebrospinal fluid as a diagnostic clue

Periventricular white matter immunoglobulin lambda light chain deposition disease diagnosed by proteomic analysis

“…Because of the localized nature of this process and the previously described indolent nature, prognosis of cerebral LCDD is most likely favorable. Three prior cerebral LCDD cases showed no disease progression 12-24 months after diagnosis [6,7,9]. Our patient is stable at 4 months.…”

“…By clinical history, imaging, and pathology, this case closely resembles previously reported cerebral LCDD cases and, to some extent, primary cerebral amyloidomas, typically also of AL lambda type [1,2,4,6,7]. The similarity of both conditions suggests that they might represent the ends of the spectrum of the same disease [4].…”

“…Two previous cerebral LCDD cases occurred in the setting of primary CNS lymphoplasmacytic lymphoma [1,6]. In 1975, Jellinger et al [3] also described several cases of primary CNS lymphoma associated with aggregation of amyloid-like Congo red-negative material.…”

“…Currently, only five cases of cerebral LCDD have been reported, four surgical and one autopsy [1,6,7,9]. Four cases with localized lambda light chain deposition showed a characteristic deep white matter distribution.…”

“…Most LCDD cases are associated with a systemic plasma cell dyscrasia or lymphoma [1,6]. Two previous cerebral LCDD cases occurred in the setting of primary CNS lymphoplasmacytic lymphoma [1,6].…”