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Intravascular large B‐cell lymphoma presenting with extensive pulmonary embolism

A 64-year-old female presented with shortness of breath, hypoxia, high fevers, night sweats and prominent cutaneous telangiectasia (top). Computed tomography pulmonary angiography demonstrated right-sided pulmonary thromboembolism and mild splenomegaly. Lactate dehydrogenase (LDH) was elevated to 891 iu/l (normal range 120–250 iu/l). Despite intravenous antibiotics and anticoagulation with low-molecular-weight heparin, the hypoxia did not improve. F-fluorodeoxyglucose (FDG)-positron emission tomography showed moderate FDG uptake in the spleen but no FDG-avid lymph nodes. Skin biopsy revealed a single focal intravascular infiltrate of large lymphoid cells (bottom) in the deep dermis suggestive of intravascular large B-cell lymphoma (IVLBCL). The malignant cells were positive for CD20, BCL2, BCL6, MYC and multiple myeloma oncogene 1 (MUM-1) and had a high Ki-67 Proliferation Index. The patient underwent a splenectomy, confirming the diagnosis of IVLBCL. There was no evidence of lymphomatous involvement on bone marrow biopsy. The patient commenced on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) with rapid resolution of hypoxia, fevers and telangiectasia and normalisation of LDH. IVLBCL is a rare, aggressive form of lymphoma. Skin is a common extranodal site of disease. In this case, the patient presented with pulmonary embolism and failed to respond to standard anticoagulation.