Introduction 
Intravascular large B-cell lymphoma is a rare subtype of 
diffuse large B-cell lymphoma with an estimated frequency 
of less than one percent of all lymphomas. It has a variety 
of presentations and is known as “the great imitator” as it 
may mimic other conditions. It has a fulminant course with 
high mortality if left untreated.  
Case Presentation 
Discussion 
We report a 65-year old male who presented with exertional 
dyspnea, dry cough, and hoarseness of a few days. Work-up 
did not reveal any specific condition or lesion in the 
cardiopulmonary systems. He developed worsening 
pancytopenia within one week, underwent a bone marrow 
biopsy, and was found to have intravascular diffuse large B 
cell lymphoma with hypertetraploidy (karyotype XXYY). 
His symptoms improved after one cycle of chemotherapy. 
Bone marrow biopsy after six cycles of chemotherapy 
showed no evidence of lymphoma and a normal karyotype. 
Intravascular large B-cell lymphoma is a type of extranodal 
large B-cell lymphoma where growth is restricted to the 
Lumina of vessels. It is a rapidly fatal malignancy when 
diagnosis and treatment is delayed. There are two forms: 
the Western form presents with skin, neurologic findings 
and lymphadenopathy, and the Asian form presents with  fever, anemia, thrombocytopenia, hepatosplenomegaly, 
bone marrow invasion, respiratory disturbance and 
disseminated intravascular coagulopathy. Our patient 
presents as a hybrid of both variants despite being of 
Western origin.