This is a 68-yr-old female of Asian-Indian origin with seronegative rheumatoid arthritis on prednisone and leflunomide with associated newly diagnosed autoimmune hemolytic anemia (AIHA) who was admitted to the general medicine service for shortness of breath. She was found to be anemic with Hgb of 8.1 g/dL. Hematology was consulted for further management for suspected AIHA which led to a bone marrow biopsy.

She had a low haptoglobin (<20 mg/dL) and elevated D-dimer (11.38 ug/mL) with low platelets (48,000/uL), abnormal elevated PT of 32.7s and INR of 3.4 with low fibrinogen (168 mg/dL). Her serum ferritin was elevated at 9833 ng/mL as was serum triglcyerides at 346 mg/dL. Also EBV quantitative PCR showed elevated EBV DNA at 25,000 copies/mL

Bone marrow biopsy shown below is a typical example of intravascular  large B-cell lymphoma associated with extensive hemophagocytic lymphohistiocytosis (HLH).

The patient received a modified R-CHOP regimen with cyclophosphamide, rituxumab, and high dose steroids (no vincristine or doxorubicin due to hepatic and renal failure). Patient's condition rapidly deteriorated and she passed away shortly thereafter.

Learning points:

1. This type of lymphoma is often widely disseminated in extranodal sites, but lymph nodes are usually spared and with significant delay in reaching an accurate diagnosis.
2. Two major patterns of organ involvement by intravascular large B-cell lymphoma have been reported.
   1. 'Western form' predominantly involving CNS or skin
   2. 'Asian form' which often involves multiple organs and presents with hepatosplenomegaly, anemia, HLH, and multiorgan failure.

The clinical presentation of this case are more consistent with the Asian form of intravascular large B-cell lymphoma.