Case report of high-grade B-cell lymphoma with MYC and BCL2 rearrangements presenting as compartment syndrome of the leg.

Abstract

Background: Diffuse large B-cell lymphoma (DLBCL)/high-grade B-cell lymphoma with MYC and BCL2 rearrangements ("double-hit" lymphoma) is an uncommon subtype of mature B-cell lymphoma characterized by the concurrent rearrangements of MYC and BCL2 oncogenes. Rarely, aggressive high-grade lymphomas manifest as compartment syndrome, necessitating urgent surgical intervention. Here, we describe a case of high-grade B-cell lymphoma with an unusual presentation of compartment syndrome.

Case description: A 68-year-old woman presented to the emergency room with increased swelling and pain in her right leg, was subsequently diagnosed with right thigh compartment syndrome, and underwent urgent fasciotomy followed by repeat debridement in the medical wound closure. Additionally, the patient was found to have inguinal and external iliac lymphadenopathy along with deep vein thrombosis (DVT). Her past medical history included human immunodeficiency virus (HIV) infection, which was well-controlled with bictegravir, emtricitabine, and tenofovir alafenamide. Excisional biopsy of the right thigh muscle and fluorescence in situ hybridization (FISH) analysis confirmed the diagnosis of high-grade B-cell lymphoma with MYC and BCL2 gene rearrangements arising in the setting of immunodeficiency/dysregulation (lymphoma associated with HIV infection). Despite aggressive medical management in the intensive care unit, the patient succumbed to the disease and ultimately died from sepsis and hemorrhagic shock.

Conclusions: High-grade B-cell lymphoma with MYC and BCL2 rearrangements represents an aggressive lymphoma with a poor prognosis. The unusual manifestation of this lymphoma involving skeletal muscle and presenting as compartment syndrome is rare. Unfortunately, the patient passed away shortly after undergoing debridement surgery. This case highlights the importance for clinicians to be vigilant and attentive to atypical presentations, as delays in diagnosis and treatment can have significant consequences. Early recognition and prompt intervention are crucial in saving the patient's life.