Case Presentation:
A 70-year-old male with an initial presentation of back pain and
difficulty walking was diagnosed with IgD lambda subtype of
International Staging System (ISS) stage II MM eight years prior to
current presentation with only bony lesions and without renal disease.
He was found to have a T3 tumor with lytic bone lesion which was managed
by a laminectomy and radiation therapy. He was able to achieve a very
good partial response with 6 cycles of lenalidomide, bortezomib, and
dexamethasone (RVD). The patient underwent high dose chemotherapy
followed by autologous stem cell transplant and was subsequently started
on maintenance therapy with 10 mg lenalidomide. He had multiple relapses
and underwent multiple lines of therapy. Most recently, the patient was
on once weekly salvage therapy with carfilzomib, pomalidomide, and
dexamethasone (KPd), however, he developed anemia grade 3 and
neutropenia grade 4. He was hospitalized due to acute kidney injury with
oliguria likely due to cast nephropathy in the setting of progressive
MM. His renal function improved significantly following treatment with
cyclophosphamide 1 gm/m2 for 2 days, pulse dexamethasone 40 mg for 5
days, and rasburicase 3 mg due to hyperuricemia and tumor lysis
syndrome. During hospital stay, the patient had significant abdominal
pain and distension. Paracentesis revealed ascitic fluid with elevated
white blood cell count indicating possible SBP. An abdominal ultrasound
with liver doppler ruled out any liver pathology and ascitic fluid
cultures were negative. The peritoneal fluid cytology demonstrated the
presence of numerous atypical CD138+ plasma cells with lambda light
chain restriction on kappa/lambda immunoglobulin light chain
immunostaining and this confirmed our suspicion of malignant plasmacytic
ascites (Figure 1A-D). The patient’s condition progressed rapidly over a
period of one month. Thereafter, he was further evaluated for possible
non-B cell maturation antigen (BCMA) directed therapies under clinical
trials. He was hospitalized multiple times for pleural effusions
requiring thoracentesis and pleural catheter placement to alleviate his
worsening dyspnea. The possibility of malignant effusion was considered
since his respiratory symptoms were new and of sudden onset.
Accordingly, a confirmatory pleural fluid cytology and
immunohistochemistry was performed which revealed atypical plasma cells
positive for CD138 with lambda immunoglobulin light chains restriction
(Figure 2A-D). Concurrently, his serum creatinine levels rose
dramatically from 0.86 mg/dL to 2.63 mg/dL and evolved to acute renal
insufficiency necessitating a cystoscopy to place ureteral stent.
Although the patient’s kidney function improved after one cycle of
cyclophosphamide, bortezomib, and dexamethasone (CyBorD), he developed
an uncontrolled atrial fibrillation. The patient was transitioned to
hospice care and ultimately died.