Case Presentation: Patient is a 62 year-old morbidly obese male (BMI 56), chronic obstructive pulmonary disease (COPD) on 4L of Nasal Cannula (NC) O2 at home, heart failure with preserved ejection fraction, who presented complaining of acute migratory left shoulder pain that radiates to his L ribs and now to the anterior chest. Pain is a constant 8/10 in severity, not sharp, stabbing, nor crushing but is worsened by changing positions. No recent trauma or falls. He also reports chronic lower back pain (35 years, 8/10, localized to the lumbosacral region) that has worsened over the past month. No complaints of saddle anesthesia, bladder or bowel incontinence, lower extremity weakness or loss of sensation. On physical exam, patient is obese, saturating in low 90’s on 8L O2 via NC, alert and orientedx3, with diffuse expiratory wheezes on lung exam, tenderness to palpation in the lumbosacral region, and left shoulder passive range of motion limited on abduction, and no focal neurologic deficits.
Significant labs include WBC 14.3 k/uL with 12% atypical lymphocytes, creatinine 1.82 (baseline 1.02; 78% increase), total protein 10.2, albumin 1.0, corrected calcium 11.2 mg/dL, phosphorous 4.9 mg/dL, uric acid 22.1 mg/dL. Blood smear demonstrated plasma cells with eccentric nuclei and rouleaux formation as in Figure 1a. CT spine demonstrate lytic lesions in the lumbosacral spine as in Figure 1b.
Patient experienced acute on chronic hypoxemic respiratory failure requiring intubation and transfer to the ICU. He was treated aggressively with fluid boluses, rasburicase, and allopurinol for tumor lysis syndrome (TLS). Eventually, patient required continuous veno-venous hemodialysis due to worsening kidney function secondary to TLS and/or myeloma kidney. Additional labs later returned during hospitalization were significant for elevated gamma protein (5.8), elevated IgG 6454, 6% circulating plasma cells, CD56, CD38, and CD138 positive, as well as elevated lambda:kappa ratio 1700. He was then treated for multiple myeloma (MM) with bortezomib and dexamethasone. Due to improving respiratory status patient was extubated; however, the patient unfortunately passed away several days later from suspected aspiration pneumonia.
Discussion: MM has a low proliferate rate and proportion of cells in the S phase is small. Therefore, TLS is rare in patients with MM. Only about 1% of MM patients receiving high dose chemotherapy have shown to develop TLS. Even fewer cases have been reported of spontaneous TLS (TLS prior to the initiation of chemotherapy), with only five reported cases. Spontaneous acute injury with marked hyperuricemia prior to initiation of chemotherapy has been described in other hematology malignancies such as acute leukemia and non-Hodgkin’s lymphoma. In a study by Tsokos et al, three patients with spontaneous TLS required hemodialysis due to renal failure from marked hyperuricemia.
This presentation is to draw attention to hospitalists of the possibility that patients with MM may present with spontaneous TLS without any prior chemotherapy treatment. Prompt recognition is extremely important for aggressive management in the hospital setting as spontaneous TLS is fulminant at its onset and associated with severe metabolic derangements that are potentially reversible
Conclusions: We present a case of MM with spontaneous TLS. This case highlights some important teaching points: hospitalists should suspect TLS in patients with MM with metabolic derangements and TLS can present prior to initiation of chemotherapy.
To cite this abstract:Shiao, JC; Plannegar, R; Varughese, M. A RARE CASE OF MULTIPLE MYELOMA PRESENTING WITH SPONTANEOUS TUMOR LYSIS SYNDROME. Abstract published at Hospital Medicine 2018; April 8-11; Orlando, Fla. Abstract 850. https://www.shmabstracts.com/abstract/a-rare-case-of-multiple-myeloma-presenting-with-spontaneous-tumor-lysis-syndrome/. Accessed October 23, 2019.