POSTER 26
A RARE CASE OF ISOLATED CENTRAL NERVOUS SYSTEM (CNS) RELAPSE OF MULTIPLE
MYELOMA (MM)
Natasa Eleni Loutsidi1, Natali El Gkotmi1, Konstantinos Kaouranis1, Evagelia Kyriaki Dimitraki1, Anna
Roumelioti1, Themistoklis Karmiris1 and Sosanna Delimpasi2
(1)Hematology-Lymphomas Dept and BMT Unit, Evangelismos, General Hospital of Athens, Greece,
Athens, Greece
(2)Hematology-Lymphomas Dept and BMT Unit, Evangelismos, General Hospital of Athens, Athens,
Greece
Objectives: CNS involvement in patients with MM is extremely rare and is associated with a poor
prognosis. Up to 5% of patients with MM develop extramedullary disease, which results from
hematogenous spread or via the bone cortex into contiguous tissue. CNS involvement is a rare form of
extramedullary disease. The diagnosis of CNS involvement is a challenge for the clinician, as it
manifests itself with neurological symptoms, which can be interpreted as typical manifestations of MM
or side effects of treatment. We present a case of a Caucasian male patient, 60 years old, diagnosed
with MM in 2017,who had a second isolated relapse in the CNS.
Methods: A 60-year-old man, with no personal history, was diagnosed on 10/2017 with MM, stage I
according to ISS. He was treated with the VCD regimen (Bortezomib, Cyclophosphamide,
Dexamethasone) and achieved complete remission. One year later, he relapsed with plasmacytomas
in the thoracic spine. He refused Autologous Hematopoietic Stem Cell Transplantation and received 17
KRD cycles (Carfizomib, Lenalidomide, Dexamethasone). The patient achieved complete remission of
plasmacytomas, underwent maintenance treatment with lenalidomide from 7/2020 and regular
follow-up at the Outpatient Department. In October 2020 he presented with numbness in the left
upper and lower extremity, gait instability and urinary cyst disorders. CAT scan of the brain was normal.
MRI of lumbar spine was performed, which showed thickening in cauda equina roots. In the differential
diagnosis, among others, there was leptomeningeal infiltration from MM. Laboratory testing was
normal. It is worth mentioning, that serum immunofixation was negative for monoclonal paraprotein.
Moreover, there was absence of marrow infiltration by plasma cells. Lumbar puncture was performed
and the CSF study by flow cytometry did not detect plasma cells, however they were found after
microscopy of CNS centrifuges (Figure1).
Results: The patient was treated with a combination of intrathecal injections of chemotherapy
(methotrexate, aracytin, dexamethasone), 2 times per week until non-detection of plasma cells in CNS
and systemic administration of Daratumumab every 15 days. He showed a dramatic improvement in
symptoms, with a complete remission of neurological semiology.
Conclusion: CNS infiltration is rare and poorly described in the literature. The optimal treatment
approach is not currently defined. The gold-standard method for detecting CNS-MM is CSF cytology
and flow cytometry. The treatment must be aggressive due to frequent treatment failure and includes
a combination of conventional chemotherapy, proteasome inhibitors, imids, autologous stem cell
transplantation and radiotherapy. Moreover, intrathecal combinational chemotherapy (methotrexate,
aracytine) is used in most of the cases. Even in the age of new therapies, the prognosis of CNS-MM
remains unfavorable, emphasizing the need to study the CNS penetration of existing drugs, but also
the development of new ones with sufficient penetration into the CNS.