Aradhana Verma1, Akanksha Sharma2, Ryan Robetorye3, Alyx Porter2, Talal Hilal4. 1. California Northstate University of Medicine, Elk Grove. 2. Department of Neurology, Mayo Clinic, Phoenix, AZ. 3. Department of Pathology, Mayo Clinic, Phoenix, AZ. 4. Division of Hematology and Medical Oncology, Mayo Clinic, Phoenix, AZ.
Abstract
INTRODUCTION: We present a case of an Asian variant of intravascular large B-cell lymphoma associated with systemic and central nervous system hemophagocytic lymphohistiocytosis (HLH) with multiple neurologic manifestations. CASE PRESENTATION: A 68-year-old, previously healthy, Korean woman presented with a 4-week history of fever and weight loss. She had pancytopenia with neutropenia, a ferritin level of 5030 μg/L, and elevated liver enzyme levels. Bone marrow, liver biopsy specimens, and cerebrospinal fluid demonstrated hemophagocytosis. The patient was treated with the HLH-2004 protocol, but her disease relapsed 3 months later. A repeated liver biopsy specimen confirmed the initially missed diagnosis of intravascular large B-cell lymphoma, a known driver of HLH in patients of Asian origin. She was then treated with lymphoma-directed therapy and had a prompt resolution of fever and neurologic symptoms as well as normalization of her blood cell counts and ferritin level. DISCUSSION: This case serves as a reminder that patients with an Asian variant of intravascular large B-cell lymphoma frequently present with HLH and neurologic manifestations, including seizures, strokes, and cognitive deficits. A high index of suspicion is needed for prompt diagnosis and initiation of lymphoma-directed therapy.
INTRODUCTION: We present a case of an Asian variant of intravascular large B-cell lymphoma associated with systemic and central nervous system hemophagocytic lymphohistiocytosis (HLH) with multiple neurologic manifestations. CASE PRESENTATION: A 68-year-old, previously healthy, Korean woman presented with a 4-week history of fever and weight loss. She had pancytopenia with neutropenia, a ferritin level of 5030 μg/L, and elevated liver enzyme levels. Bone marrow, liver biopsy specimens, and cerebrospinal fluid demonstrated hemophagocytosis. The patient was treated with the HLH-2004 protocol, but her disease relapsed 3 months later. A repeated liver biopsy specimen confirmed the initially missed diagnosis of intravascular large B-cell lymphoma, a known driver of HLH in patients of Asian origin. She was then treated with lymphoma-directed therapy and had a prompt resolution of fever and neurologic symptoms as well as normalization of her blood cell counts and ferritin level. DISCUSSION: This case serves as a reminder that patients with an Asian variant of intravascular large B-cell lymphoma frequently present with HLH and neurologic manifestations, including seizures, strokes, and cognitive deficits. A high index of suspicion is needed for prompt diagnosis and initiation of lymphoma-directed therapy.
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