[Recurrent fever, persistent cytopenia]

Area of Science:

• Hematology
• Oncology
• Rare Diseases

Background:

• Intravascular large B-cell lymphoma (IVLBCL) is an uncommon subtype of large B-cell lymphoma.
• Patients often present with non-specific symptoms, complicating early diagnosis.

Purpose of the Study:

• To report a challenging case of IVLBCL initially misdiagnosed.
• To underscore the importance of a multidisciplinary approach in diagnosing and managing IVLBCL.
• To provide insights for clinical practice regarding IVLBCL diagnosis and treatment.

Main Methods:

• Case report of a 64-year-old female with a history of Waldenstrom's macroglobulinemia.
• Utilized positron emission tomography/computed tomography (PET/CT) and adrenal biopsy for diagnosis.
• Treatment involved R-CHOP chemotherapy followed by identification of central nervous system (CNS) involvement.
• Diagnosis was revised to IVLBCL after adrenal biopsy review.

Main Results:

• The patient initially presented with recurrent fever and pancytopenia.
• Adrenal gland enlargement was detected on PET/CT, leading to an initial diagnosis of diffuse large B-cell lymphoma, not otherwise specified.
• CNS infiltration was identified after initial chemotherapy.
• The final diagnosis was confirmed as IVLBCL, but the disease progressed despite aggressive treatment, leading to the patient's death.

Conclusions:

• IVLBCL poses significant diagnostic challenges due to its rarity and non-specific presentation.
• Multidisciplinary collaboration involving imaging, pathology, and clinical expertise is crucial for accurate IVLBCL diagnosis.
• The aggressive nature of IVLBCL necessitates prompt and appropriate management strategies, though outcomes remain poor in many cases.