Central nervous system
involvement in multiple myeloma: a case report.
Arturo Campos, Inmaculada Pérez , María
José Moreno, Mª Paz Queipo de llano, Mariano Narbona,
Gemma Ramirez
Servicio de Hematología y Hemoterapia, Hospital
Clínico Universitario Virgen de la Victoria, Málaga,
Spain
Correspondence: Arturo Campos Garrigues, Clínico
Universitario Virgen de la Victoria, Campus Universitario de
Teatinos s/n, 29010 Málaga, Spain. Phone: +34 952 64 93 96;
e-mail: acampos@aehh.org
Neurologic manifestations are not unusual in multiple myeloma.
Conversely meningeal and cerebral involvement have been rarely
reported (37 cases of meningeal involvement with demonstration of
plasma cells at cerebrospinal fluid in a review).1,2
The most important diagnostic test are lumbar puncture and
magnetic resonance studies of the brain and/or
spine.3,4 b2-microglobulin content in CSF is not
reliable marker for neuroleukemia, but can serve as a factor of
unfavorable prognosis of multiple myeloma.5 Patients
with meningeal myelomatosis can have a good response to treatment
initially, but their prognosis is poor with a mean time of
survival about two months.1,2,6 In a review, 8 of 9
patients had undergo inmunoblastic transformation at the time the
CNS disease was diagnosed, and they should probably undergo
routine spinal fluid examination to detect early CNS involvement6.
Differential diagnose are: infectious meningitis, intra-cranial
hemorrhage.1
We describe a patient who developed central nervous system
involvement by multiple myeloma.
A 64-years old man was diagnosed with multiple myeloma l light
chains stage III-B in May 1997. He was treated initially with VAD
(6 cycles), with a good response. One year after, because of
disease progression, he was treated with alternative cycles
VBCMP/VBAD plus Pamidronate. In the second year of the disease, he
developed with nausea, vomiting, headache, with no fever, no
neurologic symptoms and no disturbance of consciousness. Physical
examination did not shown disturbance of his neurologic status.
The complete blood count was: leukocytes 2.45x109/L (6%
plasma cells), hemoglobin 118 gr/L and platelets
108x109/L. Serum calcium concentration 2.34 mmol/L,
serum b2-micro-globulin 8.46 mg/L, serum creatinin 131 mmol/L, IgG
3.92 gr/L, IgA 260 mg/L, IgM 180 mg/L, urine l chain 18 mg/L,
urine k chain 10 mg/L. Cerebrospinal fluid: glucose 3.10 mmol/L,
proteins 1640 mg/L, b2-microglobulin 8.7 mg/L, 128
cells/mm3 (80% plasma cells). Electrophoresis in
cerebrospinal fluid: Gammaglobulin 29.2% (normal 3-12%), with M
component (Figure
1). Flow cytometry in
cerebrospinal fluid: CD38++, CD10-, CD19-. Microbiological culture
from cerebrospinal fluid was negative. Cranial CT and craniospinal
magnetic resonance did not shown tumoral mass nor leptomeningeal
enhancement. The patient was treated with standard intrathecal
therapy (methotrexate 12 mg, C-Ara 30 mg, Hydrocortisone 20 mg)
and endovenous cyclo-phosphamide 1 gr plus four days Dexametasone
40 mg. After three doses of intrathecal therapy (one dose every
ten days), the patient was asymptomatic and the cerebrospinal
fluid did not shown cells, the CSF protein level was 860 mg/L and
CSF gammaglobulin was 15.7% with persistence of M-component.
Endovenous treatment was administered monthly. Five months later
patient developed the same symptoms, and the CSF shown the similar
results. After new intrathecal treatment no response was achieved
and the patient died several days after.
