This is a 68-yr-old female of Asian-Indian origin with a newly diagnosed autoimmune hemolytic anemia (AIHA) who was admitted for shortness of breath. Her past history included a seronegative rheumatoid arthritis being treated by prednisone and leflunomide.  Complete blood count showed Hgb of 8.1 g/dL and low platelets (48,000/uL).

Bone marrow biopsy was performed to further diagnose the cause of an underlying hemolytic anemia.

She had a low haptoglobin (<20 mg/dL) and elevated D-dimer (11.38 ug/mL), 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 images shown below are 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 succumbed to the disease shortly.

Learning points:

1. Intravascular DLBCL is a rare sybtype of non Hodgkin lymphoma, with tumour cells growing in blood vessel lumina and usually with rare circulating neoplastic cells in peripheral blood. Extranodal sites such as skin, CNS are mainly affected.
2. Lymph nodes are usually spared causing a significant delay in reaching an accurate diagnosis.
3. Many cases present with non specific signs and symptoms such as fever, CNS and skin involvement. Cutaneous variants have a relatively better prognosis. 
4. Two major patterns of organ involvement by intravascular large B-cell lymphoma have been reported.
   • 'Western form'- seen in Western countries with a cutaneous variant predominantly involving CNS or skin
   • 'Asian form' which often involves multiple organs and presents with hepatosplenomegaly, anemia, HLH, and multiorgan failure
   • The often poor prognosis has substanially improved by the use of chemotherapy specifically R-CHOP regimen.