Abstract
Background/Aims
Intravascular large b-cell lymphoma (IVLBCL) is a rare and aggressive form of extranodal diffuse lymphoma which due to its heterogeneous manifestations is often diagnosed at post mortem. The disease is typically contained within the small vessels thus sparing the surrounding tissue. Indeed, the presentation of the disease will depend on the organ involved with classical involvement of the central nervous system and skin.
Methods
We herein report two compelling cases of patients presenting with features of myositis, only to find on muscle biopsy evidence of IVBCL. Biopsy of the affected area and indeed random skin biopsies (RSB) may be a fundamental tool in establishing this elusive diagnosis.
Results
Features of the two cases are discussed, along with their various presentations, investigations, treatment and overall trajectory.
Conclusion
IVBCL is a rare disease that can present in various ways and often a delay in reaching a diagnosis leads to patient deterioration and death. A multidisciplinary approach involving the microbiologists, rheumatologists and haematologists led to a timely identification of the disease and subsequent management in these two cases. As the disease is characteristically contained within the blood vessels, the diagnosis is normally not confirmed from peripheral blood tests or even bone marrow biopsy. Biopsy of the affected area is what revealed the diagnosis in our two cases. Random skin biopsies may be very useful in establishing the diagnosis when suspecting IVBCL. Treatment may be very effective in controlling the disease as demonstrated by these two cases.
Disclosure
M.A. Mirza: None.
A 68-year-old man, with a history of non-Hodgkin’s lymphoma in remission, was admitted for homonymous hemianopsia, headaches and subacute progressive cognitive decline. Imaging revealed brain infarcts and angiography suggested vasculitis. A brain biopsy, however, revealed an intravascular large B-cell lymphoma (IVLBL). Central nervous system (CNS) vasculitis and IVLBL of the brain are extremely rare diseases that can have an almost identical clinical presentation. Angiographic findings are very similar but usually are reported as compatible with vasculitis. Brain biopsy or a random skin biopsy are crucial in diagnosing IVLBL as the accuracy of angiographic findings for CNS vasculitis is low.
Intravascular Large B Cell Lymphoma Presenting as Fever of Unknown Origin and Diagnosed by Random Skin Biopsies: A Case Report and Literature Review