An explosive onset: Tumor lysis syndrome in newly diagnosed Mantle Cell Lymphoma. Free

An Explosive Onset: Tumor lysis syndrome in Newly diagnosed Mantle cell Lymphoma.

Introduction:

Tumor Lysis syndrome is an onco-metabolic emergency caused by massive tumor cell lysis resulting in electrolyte and metabolic derangement. TLS can be induced or occur spontaneously. Spontaneous tumor lysis syndrome (SPTLS) is a rare phenomenon that can manifest in rapidly proliferating hematological malignancies and solid tumors prior to initiating cytotoxic therapy. The exact incidence of SPTLS in mantle cell lymphoma is not well- defined as most available data come from individual case reports.

Case Presentation

Here we present a 90 year old male with newly diagnosed mantle cell lymphoma, who presented to our emergency room with generalized weakness. A week prior to his presentation, he was hospitalized at a different facility with abdominal pain, unintentional weight loss. He had elevated white count, elevated LFTs with hepatosplenomegaly and splenic infarcts. He underwent evaluation with bone marrow aspirate as well as liver biopsy and was diagnosed with blastoid variant mantle cell lymphoma.

At presentation he had profound leukocytosis( WBC 109.5), his other laboratory findings were consistent with hyperkalemia ( K 9.1), Hyperuricemia ( 16.2), Phosphate (4.3) Hypocalcemia ( 8.5) and Acute kidney injury ( Cr 3.1, BUN 90). His LFTs showed Hyperbilirubinemia (12.6) and transaminases ( alk phos 472, AST 59), EKG showed atrial fibrillation with peaked T waves. Patient was managed with aggressive intravenous hydration, rasburicase and was treated for hyperkalemia. He was admitted to the ICU and placed on CRRT. Unfortunately despite aggressive treatment, the patient went into multi organ failure soon and later died.

Discussion:

• TLS can be classified as laboratory or clinical TLS, with laboratory TLS defined as two or more abnormal levels values of uric acid, potassium, phosphorus or calcium at presentation or 25 % change from baseline. Clinical TLS is defined as laboratory TLS accompanied with seizures, cardiac arrhythmias, renal dysfunction or sudden death.

• SPTLS in MCL patients can lead to severe metabolic disturbances hyperuricemia, hyperkalemia, but unlike post chemotherapy tumor lysis syndrome, SPTLS is usually not associated with hyperphosphatemia. Complications from these can progress rapidly and may be fatal if not promptly recognized and treated.

• Blastoid variant Mantle cell Lymphoma is an aggressive subtype with poorer prognosis and has increased risk of SPTLS due to high tumor burden and rapid cell turnover.

• Given potential severity of SPTLS in MCL especially in aggressive subtypes, Clinician should maintain high index of suspicion, preventive strategies include risk stratification early identification, monitoring and intervention to mitigate the risk of metabolic complication

Conclusion

Clinical TLS has a high mortality rate , this case emphasises the need to recognise and treat this complication quickly as it can have fatal consequences. Additionally, it stresses the necessity to vigorously screen patients admitted with malignancy and high tumour burden for TLS, even when they do not receive cytotoxic treatment. TLS management includes adequate hydration, the use of uric acid-lowering therapies and minimisation of potassium intake.

References

Taha N, Bhaskar G. Spontaneous Tumour Lysis Syndrome in Mantle Cell Lymphoma: A Case Study. Cureus. 2022 Oct 3;14(10):e29851. doi: 10.7759/cureus.29851. PMID: 36212272; PMCID: PMC9531698.

Patel V, Case R. Spontaneous Tumor Lysis Syndrome in Blastoid-Variant Mantle Cell Lymphoma: Considerations for the General Internist. J Investig Med High Impact Case Rep. 2020 Jan-Dec;8:2324709620944709. doi: 10.1177/2324709620944709. PMID: 32720820; PMCID: PMC7388088.

Kjellstrand CM, Cambell DC, von Hartitzsch B, Buselmeier TJ. Hyperuricemic acute renal failure. Arch Intern Med 1974; 133:349-359.