CHRONIC LYMPHOCYTIC LEUKEMIA WITH PERICARDIAL INFILTRATION: A CASE REPORT

Abstract

Introduction: Chronic lymphocytic leukemia (CLL) is the most prevalent leukemia in adults. It commonly has an indolent course and can infiltrate any organ. Skin is the most commonly involved organ, while pericardial involvement is rare. We report a case of CLL with pericardial effusion as the initial clinical manifestation and the need for intervention. Case report: A 61-year-old woman with a previous history of follow-up at another facility, diagnosed with CLL in March 2022, Binet A/Rai 0, and kept in a watch-and-wait strategy. On March 2024, she was admitted to an external Emergency Room (ER) with complaints of cough and dyspnea on minimal exertion for 3 weeks. She was initially diagnosed with pneumonia (chest X-ray) and treated with antibiotics. Due to persistent symptoms, she sought another ER, where a chest CT revealed pericardial effusion, and she was transferred to the Heart Institute (INCOR). Upon admission, a transthoracic echocardiogram confirmed the diagnosis, followed by angiotomography. The patient underwent pericardiocentesis and pericardial biopsy, with removal of 350 mL of serous fluid. Microscopic evaluation of the pericardial fluid showed increased cellularity due to lymphocytes, and the biopsy revealed pericardial infiltration by CLL (CD20+/CD5+/CD23+/Ki67+(10% of cells) and CD3-/CyclinD1-). She was referred to the Cancer Institute (ICESP) and the immunophenotyping of peripheral blood confirmed the diagnosis of CLL. Laboratory tests showed mild normocytic/normochromic anemia (Hb = 11.3 g/dL) and moderate lymphocytosis (26,000/μL). Apart from the findings already described, no other changes were found on physical examination. Cytogenetic evaluation identified a complex karyotype with 3 aberrations: trisomy of chromosome 12, monosomy of 7 and t(18;22). Molecular analysis showed unmutated IgHV, TP53 mutation, FISH was negative for del17p and confirmed the BCL2 rearrangement (cr18). Discussion: Pericardial infiltration is a rare presentation in CLL. There are reports in the literature of associated cardiac tamponade, but most descriptions are autopsy findings. The potential severity of this manifestation necessitates early identification and immediate treatment. CLL exhibits biological heterogeneity, with various factors associated with clinical evolution and treatment response, including IGHV mutation status, TP53 gene mutation, del17p, and complex karyotype. The latter is defined by the presence of 3 or more structural or numerical abnormalities, with 5 or more abnormalities associated with worse outcomes in CLL(3). Unlike in acute myeloid leukemia, complex karyotype is not clearly defined in CLL. Currently, in the Brazilian public health system (SUS), only chemoimmunotherapy with FCR is available, which is not indicated due to the presence of TP53 mutation. Several therapeutic options are available with new agents showing superior results, especially in the context of TP53 mutation. In this scenario, options include finite therapies with BCL2 inhibitors combined with anti-CD20 or BTK inhibitors and continuous use of iBTK. As a clinical trial was available at our service and the patient's clinical condition was stable, we included her in the trial, and treatment with Venetoclax/Obinutuzumab was initiated. Conclusion: The present case demonstrates the importance of possible cardiac complications in patients with CLL presenting with respiratory symptoms and therapeutic approach considering factors of unfavorable prognosis.