Abstract Richter transformation denotes the transformation of a ‘low-grade’ lymphoma into a ‘high-grade’, or more aggressive malignancy. T rex lymphoma represents an exceptionally rare subtype of Richter’s transformation resulting in the development of a mature T-cell lymphoma. These transformations are uncommon, and portend a poor outcome especially when seen in combination with complications such as tumor lysis syndrome. A sexagenarian male presented for evaluation of fever, malaise and fatigue. His medical history was significant for DLBCL refractory to numerous attempts at treatment and eventual transformation to angioimmunoblastic T-cell lymphoma with chronic pancytopenia and neutropenia on tbo-filgrastim. Initial evaluation found fever in the setting of pancytopenia with an absolute neutrophil count of 150 cells/microliter, consistent with febrile neutropenia. Broad spectrum antimicrobials (cefepime, vancomycin and fluconazole) including his home prophylactic atovaquone and acyclovir were started, however evaluation found no additional causes for fever and neutropenia aside from recent chemotherapy. Plans for outpatient management were made, but would not come to fruition as the patient deteriorated due to respiratory failure. He was transported to the MICU for emergent intubation after which CT chest demonstrated bilateral infiltrates without effusion (Figure 1) while echocardiogram demonstrated preserved cardiac function. Bronchoscopy with bronchoalveolar lavage was performed with washings positive for involvement T-cell lymphoma. Additional lab work found elevated serum uric acid, phosphorus and potassium, consistent with tumor lysis syndrome for which rasburicase was started. The following days involved up titration of vasopressors and mechanical ventilator settings as the patient required increasing support in the setting of ARDS, and monitoring of progressive multi-organ dysfunction. Unfortunately, aggressive intervention proved unsuccessful. Richter syndrome is a complex pathway culminating in the replication of a malignant clone of germinal or post-germinal B cell origin. The process most often involves a low-grade B cell malignancy such as chronic lymphocytic leukemia (CLL) transforming into a more aggressive malignancy. The syndrome is rare, with incidence of CLL transforming to diffuse large B cell lymphoma (DLBCL) in approximately 2-9 percent of cases 1 over a period of 5 years. Even less common is the generation of a mature T-cell malignancy, a phenomenon known as T rex lymphoma due to its aggressive nature. Although poorly studied (owing to its relative infrequence), Richter transformation is associated with a poor prognosis with an average survival of 5-8 months 2, and even these estimates are generous in unfortunate cases involving T rex lymphoma or the development of complications of treatment. This abstract is funded by: None
Mckinney et al. (Fri,) studied this question.