Tonsillar hypertrophy as a rare presentation of chronic lymphocytic leukemia/small lymphocytic lymphoma: A mini-review

Abstract Background Chronic Lymphocytic Leukemia (CLL) is a prevalent adult leukemia that primarily affects individuals over the age of 65. It is characterized by the gradual accumulation of dysfunctional B lymphocytes. While CLL usually manifests as lymphocytosis and generalized lymphadenopathy, tonsillar involvement is a rare and often overlooked complication. This mini review delves into all reported cases of CLL/Small Lymphocytic Leukemia (SLL) accompanied by tonsillar hypertrophy, underscoring the diagnostic challenges, histopathological findings, and clinical implications associated with this condition. Our analysis highlights the importance of increased awareness regarding CLL-related tonsillar enlargement, particularly in patients presenting with unexplained airway obstruction, dysphagia, or obstructive sleep apnea. Methods A comprehensive literature search was conducted in PubMed, Scholar Semantics, and Google Scholar using the keywords ‘CLL’, 'Tonsillar Enlargement,' and 'Case Report'. The search included all articles published throughout the years. Inclusion criteria included all peer-reviewed case reports detailing various presentations of CLL with tonsillar enlargement. Exclusion criteria included non-English articles, case reports with incomplete data and non-CLL /SLL malignancies. Data was extracted from the selected reports included patients' demographics, clinical presentation, diagnosis, FISH and cytogenetics, WBC count, LDH level, histopathology findings and treatment modalities. A standardized data extraction form was utilized to ensure consistency. Results This review included 13 case reports of tonsillar hypertrophy in CLL/SLL. The median age of presentation was 55 years with slight male predominance. Patients exhibited a diverse range of presentations, from being completely asymptomatic to severe bilateral tonsillar hypertrophy that manifested as obstructive symptoms such as dysphagia, apnea, and even respiratory distress and airway compression. Unfortunately, given the rarity of this presentation of CLL, some cases were mistaken as either infectious tonsilitis or benign hyperplasia where patients were treated symptomatically thus leading to delayed diagnosis. As a result, diagnosis was primarily established through biopsy and histopathological analysis after tonsillectomy, revealing CD5+, CD20+, CD23+ lymphoid infiltration. Management strategy of tonsillar CLL/SLL varies greatly depending on severity of symptoms. For example, in asymptomatic patients with normal counts diagnosed incidentally, a “watch and wait” approach can be adopted. However, patients with obstructive symptoms, tonsillectomy is crucial not only for diagnosis, but also for symptomatic relief. In patients with severe airway compromise and rapid progression, nasotracheal intubation or tracheostomy is life-saving. Further management with systemic therapy is often needed, especially in advanced cases. Fortunately, several cases can be noted to have achieved symptomatic resolution and clinical remission of the disease through the various treatment approaches and only one patient have expired Conclusions CLL with tonsillar involvement is a rare yet clinically significant condition that often manifests as sleep apnea, airway obstruction, or dysphagia. Recognizing atypical presentations is essential for prompt diagnosis and effective management. Therefore, we strongly recommend performing tissue biopsy through tonsillectomy, not only for symptomatic relief but also to obtain a definitive tissue diagnosis, particularly in cases without any other indicators suggesting CLL. Histopathology, flow cytometry, and FISH studies are crucial tools in distinguishing CLL from other causes of tonsillar enlargement. Further treatments should be tailored based on severity of symptoms and extent of disease.