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Medical City Arlington
small lymphochronic lymphocytic leukemiacytic lymphoma, b-cell chronic lymphocytic leukemia, neoplasms
Hemic and Lymphatic Diseases | Internal Medicine | Neoplasms
Although chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) differ in clinical presentation, they are both arise from the same pathologic process, namely the clonal expansion of mature B-cells. This malignancy is usually quite indolent and carries a favorable prognosis. Thanks to a number of highly-effective oral targeted therapies, such as the Bruton tyrosine kinase (BTK) inhibitor, Ibrutinib, patient outcomes have improved significantly. Unfortunately, these therapies are not always well tolerated and many patients are forced to discontinue the medication due to side effects. Many patients experience a disease flair and some even transformation to a high-grade malignancy after the discontinuation of one of these targeted maintenance therapies. Respiratory complications of CLL/SLL are not uncommon and are most often the result of infection. Airway obstruction is seen much less frequently and is usually caused by severe hilar or mediastinal lymphadenophy. CLL/SLL presenting as bilateral tonsillar enlargement is quite rare and the majority of cases described were incidental findings or presented as obstructive sleep apnea. Herein we present a case of rapidly progressive CLL/SLL that presented as upper airway obstruction due to bilateral tonsillar hypertrophy shortly after discontinuation of Ibrutinib maintenance therapy. Patient required fiberoptic nasotracheal intubation. His lengthy hospital course significant for severe leukocytosis (max WBC 439), pseudohyperkalemia, neoplastic fever, hypotension requiring vasopressors, and concern for tumor lysis syndrome. After tonsillar biopsy confirmed diagnosis and ruled out transformation, patient was started on CHOP chemotherapy. Although patient’s leukocytosis initially decreased after initiation of chemotherapy, it quickly rebounded, indicating unresponsiveness to treatment. Patient’s family ultimately elected for comfort care and patient expired soon after. This unique case has several important teaching points ranging from hematology/oncology, critical care, infectious disease, surgery, and laboratory science. This rare presentation of CLL/SLL after Ibrutinib discontinuation includes the management of a critical upper airway obstruction and neoplastic fever, reminder of the laboratory error caused by severe leukocytosis, and how to recognize and help prevent tumor lysis syndrome. Less than 10 cases of CLL/SLL presenting as bilateral tonsillar enlargement have been described in English literature, highlighting the rarity of this case. However, unlike the others, the case presented here was quite severe and progressed rapidly, unfortunately leading to a poor outcome. As targeted therapies continue to replace chemotherapy in treatment of CLL/SLL, this indolent malignancy may more frequently present in unusual and potentially devastating ways when these treatments are discontinued.
HCA Healthcare Graduate Medical Education
Mangin, William and Aslam, Kashif, "A Rare Case of Rapidly Progressive CLL/SLL Presenting as Bilateral Tonsillar Enlargement and Upper Airway Obstruction" (2023). North Texas Research Forum 2023. 14.