A 19-year-old male with generalized seizures, unconsciousness and a deviation of gaze.
Light chain deposition disease (LCDD) is a form of monoclonal immunoglobulin deposition diseases (MIDD) which in contrast to light-chain derived (AL) amyloidosis is characterized by non-congophilic, non-fibrillary monoclonal protein deposits. Systemic organ deposits are common with the kidney being a major target organ. A clonal lymphoplasmocytic proliferation, e.g. plasmacytoma, is present in the majority of cases. Here we report on a 19-year-old male who presented with generalized seizures and an enhancing white matter lesion on MRI scans. A stereotactic brain biopsy revealed a low-grade B cell lymphoma with plasmacellular differentiation as well as lambda light chain deposits without birefringence under polarized microscopy. No systemic lymphoma manifestations or systemic light chain deposits were found, nor was a monoclonal gammopathy detectable in serum and urine. After systemic chemotherapy with three courses high-dose methotrexate the size of the lesion and the condition of the patient have remained stable for 24 months now. This is the first description of cerebral LCDD developing without systemic disease in conjunction with the diagnosis of a cerebral low-grade B cell lymphoma. We present the clinical, laboratory and radiological findings and discuss the pathogenesis of this unusual LCDD manifestation.
Fischer
L
L
Department of Hematology, Oncology and Transfusion Medicine, Klinikum Benjamin Franklin, Freie Universität, Berlin, Germany.
Korfel
A
A
Stoltenburg-Didinger
G
G
Ransco
C
C
Thiel
E
E
D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D001927:Brain Diseases / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D001929:Brain Edema / Q000209:etiology / Q000473:pathology*; D001932:Brain Neoplasms / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D002220:Carbamazepine / Q000627:therapeutic use; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000009:adverse effects* / Q000378:metabolism*; D016393:Lymphoma, B-Cell / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D015835:Ocular Motility Disorders / Q000209:etiology; D010265:Paraproteinemias / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D012640:Seizures / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D016896:Treatment Outcome; D014474:Unconsciousness / Q000209:etiology / Q000473:pathology
D000927:Anticonvulsants; D000964:Antimetabolites, Antineoplastic; D007147:Immunoglobulin Light Chains; D002220:Carbamazepine; D008727:Methotrexate