Search

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) transforming into a more aggressive lymphoma (i.e., Richter syndrome) is well documented in the literature. In recent years, transdifferentiation of CLL/SLL to histiocytic/dendritic/Langerhans cell sarcomas has also been reported. We hereby describe a case of a 55-year-old female who was incidentally diagnosed with CLL after presenting to the hospital for symptoms of undiagnosed rheumatoid arthritis. At the time of presentation, CLL was stage 1, and the patient was placed on observation. Eight years after being diagnosed with CLL, and after several treatment modalities for her rheumatoid arthritis, the patient re-presented with progression of adenopathy, intermittent fevers, 5-pound weight loss, and worsening respiratory status requiring airway management. Computed tomography (CT) imaging revealed a soft tissue mass in the nasopharynx, lingual tonsillar hypertrophy with airway compromise, and bulky cervical, supraclavicular, and axillary lymphadenopathy. A biopsy of an enlarged cervical lymph node yielded a diagnosis of histiocytic/dendritic cell sarcoma favoring interdigitating dendritic cell sarcoma, likely representing transdifferentiation from CLL/SLL, of which there are no standard of care treatment guidelines. The patient was treated with ifosfamide, carboplatin, and etoposide (ICE) for three cycles, followed by rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH) in combination with zanubrutinib. She then underwent haploidentical hematopoietic stem cell transplantation. At the time of the making of this manuscript, the patient was 45 days post-transplant without any notable complications., Competing Interests: The authors declare no conflict of interest., (Copyright 2024, Cai et al.)

Prostatic PIRADS 4 and 5 lesions on multiparametric MRI typically represent adenocarcinoma with small lymphocytic lymphoma being a rare pathological finding. We report a case of small lymphocytic lymphoma masquerading as PIRADS 4 and 5 lesions with associated lymphadenopathy in a 69-year-old male on active surveillance for low-risk prostate cancer that was subsequently confirmed on targeted and systematic prostate biopsy. Following treatment of lymphoma with ibrutinib, there was complete resolution of the PIRADS lesions on follow-up mpMRI.

Small lymphocytic lymphoma (SLL) is a relatively rare B-cell non-Hodgkin lymphoma that is considered to be the tissue equivalent of the much more common entity chronic lymphocytic leukemia (CLL). Most patients with SLL present with indolent generalized lymphadenopathy, which has frequently been present for several years. We hereby describe an unusual case of SLL in a patient who presented with a huge swelling at the right shoulder region and a single right enlarged axillary lymph node. The other uncommon findings were the presence of peripheral blood hypereosinophilia and CD5 immunonegativity.

Chronic lymphocytic leukemia is one of the most common lymphoid malignancies. Often treatment modalities are tailored to individual patients based on age, presence of comorbidities and cytogenetics. The advent of ibrutinib has significantly changed the management of the disease in all patient groups and has had the largest impact on clinical practice to date. Over the last 15 years, a series of trials have established that chemoimmunotherapy improves both progression-free survival and overall survival compared to chemotherapy alone. Despite its proven role, efficacy of ibrutinib has not been well established in young, fit patients and in comparison with standard care and as combination therapy with other agents such as venetoclax. New data have strengthened the role of ibrutinib in the front-line setting and establish its place in therapy. In addition, combination therapies are geared to achieve negative minimal residual disease and allow patients to potentially be off of therapy. The management of this leukemia has extensively changed over the past years, and this review article will aim to highlight key trials that have changed practice and led to guideline updates. It is not unlikely that treatment modalities will continue to improve in light of new data. [ABSTRACT FROM AUTHOR]

Humoral hypercalcemia due to parathyroid hormone-related peptide (PTHrP) elevation is a well-known complication of various malignancies, but the situation is rare concerning hematological malignancies except for adult T-cell leukemia/lymphoma. We report a case of Richter’s syndrome with humoral hypercalcemia, and demonstrate by reverse transcription polymerase chain reaction (RT-PCR) that peripheral blood PTHrP levels were 2,500-fold higher compared to healthy controls. PTHrP production by tumor cells in chronic lymphocytic leukemia (CLL) and Richter’s syndrome has been previously demonstrated by nonquantitative methods such as immunohistochemistry and northern blot analysis, but this is the first report using the RT-PCR method. The presented case did not have hypercalcemia when initially diagnosed as small lymphocytic lymphoma (SLL), and as reported earlier, the development of hypercalcemia may be an indication of the transformation to Richter’s syndrome in patients with CLL/SLL.

We describe a case of progressive multifocal leukoencephalopathy (PML) caused by infection with the human polyomavirus JC virus in a patient with B-cell small lymphocytic leukemia who was treated with rituximab. The first symptoms of PML appeared immediately following the last of five cycles of rituximab, cyclophosphamide and pentostatin. Magnetic resonance imaging revealed changes consistent with PML, although JC virus DNA was not detected by polymerase chain reaction assay of the cerebrospinal fluid. A stereotactic biopsy of the brain showed histological changes consistent with PML, while electron microscopy revealed JC virus particles attached to the nuclei of astrocytes. The patient was treated supportively but died 53 days after the initial onset of symptoms.

Humoral hypercalcemia due to parathyroid hormone-related peptide (PTHrP) elevation is a well-known complication of various malignancies, but the situation is rare concerning hematological malignancies except for adult T-cell leukemia/lymphoma. We report a case of Richter's syndrome with humoral hypercalcemia, and demonstrate by reverse transcription polymerase chain reaction (RT-PCR) that peripheral blood PTHrP levels were 2,500-fold higher compared to healthy controls. PTHrP production by tumor cells in chronic lymphocytic leukemia (CLL) and Richter's syndrome has been previously demonstrated by nonquantitative methods such as immunohistochemistry and northern blot analysis, but this is the first report using the RT-PCR method. The presented case did not have hypercalcemia when initially diagnosed as small lymphocytic lymphoma (SLL), and as reported earlier, the development of hypercalcemia may be an indication of the transformation to Richter's syndrome in patients with CLL/SLL. [ABSTRACT FROM AUTHOR]

Richter's transformation from chronic lymphocytic leukemia (CLL) to aggressive lymphoma is a relatively rare event with well-characterised clinical and radiological features, and can typically be distinguished from infectious complications of CLL. Opportunistic infections in CLL happen predominantly in the context of immunosuppressive therapy and/or relapsed/refractory disease. Herpes simplex viral (HSV) lymphadenitis is a rare phenomenon in treatment-naive CLL patients with only six cases reported in the English-language literature. Its diagnosis is challenging; its management and the outcome of the subsequent treatment for CLL are not well documented. We report three cases of rapidly progressive lymphadenopathy occurring in the context of previously untreated CLL, clinically and radiologically mimicking Richter's transformation, but histologically confirmed as necrotic HSV lymphadenitis. We describe the presentation, diagnosis and management of all three cases, including for the first time the positron emission tomography-computed tomography (PET-CT) appearance of this condition, as well as how we later on delivered CLL-directed immunochemotherapy safely and successfully without recrudescence of HSV-related disease. Our cases underscore the importance of obtaining biopsy in all cases of rapidly progressive or disconcordant lymphadenopathy in CLL patients, or in those with highly 18 FDG-avid adenopathy on PET-CT., Competing Interests: None to declare., (Copyright 2019, Hodgson et al.)