Case Presentation:

A 64‐year‐old Japanese man was admitted because of the low‐grade fever and progressive shortness of breath on exertion for two months. He also developed visual disturbance one month prior to admission and ocular steroid therapy has been instituted based on the diagnosis of uveitis. About a year ago, he had right lower leg necrotizing fasciitis which was treated by surgical debridement and antibiotics. He had no known drug allergy. On physical exam, he was alert and oriented but appeared chronically‐ill and anemic. The vital signs were normal. No palpable lymphadenopathy was present. The abdomen was soft and non‐tender and there was mild splenomegaly. He had the operative scar over the right lower leg. Bilateral vision test revealed low visual acuity. Laboratory data showed anemia (Hb 9.3 g/dl), low platelets (154,000/μl), low serum albumin (2.6 g/dl),and elevated lactate dehydrogenase (LDH, 1,567 IU/L). For workups on his anemia, upper gastrointestinal endoscopy was performed and identified ulcerating tumor in the gastric body and the pathological examination revealed that it was gastric adenocarcinoma. Although this cancer lesion could explain his anemia, it could not explain an extremely high value of LDH with isozyme II predominance (40%). Thus we proposed another possibility such as lymphoma, which is a common cause for elevated LDH. Since there was no lymphadenopathy on physical exam as well as in the whole body computed tomographic scan, we suspected intravascular lymphoma (IVL) and conducted random skin and bone marrow biopsies. There was infiltration of large abnormal lymphocytes around small vessels of the subcutaneous fat and there was numerous blood cell phagocytosis in bone marrow. Diagnosis of intravascular large B‐cell lymphoma with hemophagocytic syndrome was given. With high IL‐6 level of anterior chamber fluids and vitreous opacity, uveitis due to the central nervous system infiltration of lymphoma was considered. CHOP chemotherapy with rituximab was initiated and the progression of visual disturbance was halted and LDH was also quickly normalized. After the progression of lymphoma was controlled, he underwent surgical treatment of gastric adenocarcinoma successfully.


Although non‐specific symptoms are common in IVL, increased serum LDH and low albumin are its important clinical features. In addition, IVL patients in Asian and Western countries differ regarding clinical findings: hepatosplenomegaly, B symptoms, hemophagocytic syndrome, and abnormalities of bone marrow biopsies are more common among Asians, while the involvement of CNS and skin is more prevalent in western patients with IVL. Thus bone marrow and random skin biopsies are considered important for Asian patients suspected of IVL, which has become treatable illness.


Although coexisting case of gastric cancer and IVL is very rare, investigation for a cause for unexplained high LDH should be thoroughly continued. Diagnosis of ILV can be performed by bone marrow and random skin biopsies and it is important because of the potential curability.