Case
An 81-year-old woman with progressively deteriorating Parkinson’s
disease despite ongoing medical treatment for more than 5 years as well
as osteoporosis (on denosumab q6 months) presented to our institution’s
spine center for worsening back pain and frequent falls. She was
initially diagnosed with a mild degenerative disc disease of her
cervical, thoracic, and lumbar spine without myelopathy as well as
severe facet arthrosis in the lumbar spine from L2-3 through L5-S1 as
demonstrated on MRI images. Due to progressive pain and the possibility
of compression fractures from her recurrent falls, a PET-CT was
performed which reported several foci of marked hypermetabolism
including a dominant lesion in the right humeral head, SUV max 9.9 and
additional hypermetabolic lesions were seen in the bilateral scapulae,
clavicles, right hemi-sacrum, right iliac wing, left acetabulum anterior
column, left superior pubic ramus, and right femoral head
(Figure 1A ). The images were suggestive of MM-associated lytic
lesions and a subsequent serum protein electrophoresis and
immunofixation revealed small monoclonal IgA lambda immunoglobulins.
Complete blood cell count, quantitative serum free light chains,
β2-microglobulin, albumin, LDH and creatinine were all within normal
ranges. The patient did have mild hypercalcemia with a calcium level of
10.3 mg/dL. A subsequent bone biopsy from the left anterior acetabulum
was obtained, which revealed diffuse proliferation of small
B-lymphocytes with an interstitial and para-trabecular pattern
(Figure 2A ). Immunohistochemistry (IHC) studies showed that the
lymphocytes were positive for CD20 and PAX5 (Figure 2 B and C ),
which proved the B cell lineage of the lymphoma. The neoplastic
lymphocytes were negative for MUM1 and cyclin D1. Moreover, CD138
highlighted scattered plasma cells that were positive for IgA and lambda
light chain restricted (Figure 2D-F ). A MYD88 L265P alteration
was detected via amplification of DNA using allele-specific polymerase
chain reaction with an allele-specific primer. These findings were most
consistent with a lymphoplasmacytic lymphoma.
Given the diagnosis of lymphoplasmacytic lymphoma in a frail patient
with advanced Parkinson’s disease and an ECOG (Eastern Cooperative
Oncology Group) performance status of 2, it was decided to treat the
patient with chemoimmunotherapy consisting of IV rituximab
375mg/m2, IV cyclophosphamide
300mg/m2 and IV dexamethasone 20mg given every 21
days. The patient completed 6 cycles of treatment. Treatment was
complicated by the development of grade 2 neutropenia and grade 1
anemia. The patient also developed a grade 2 urinary tract infection
that needed treatment with oral antibiotics and grade 2 herpes labialis
which required acyclovir treatment for 10 days after cycle 4 causing
cycle 5 to be delayed by 1 week. Following completion of the 6 cycles of
treatment, the patient had complete resolution of lytic lesions on
PET-CT (Figure 1B) and no detectable IgA kappa monoclonal
protein on immunofixation. The patient went on to start maintenance
therapy with single agent rituximab every 3 months for up to 2 years.
The patient still had some back pain which is likely related to her
underlying degenerative joint disease and severe facet arthrosis in the
lumbar spine so she will follow-up with neuroradiology for possible
facet joint injections and/or potential kyphoplasty.