Intravascular large B-cell lymphoma: The Great Imitator

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma with an estimated incidence of less than one per million. Unlike other hematopoietic malignancies, lymphadenopathy and hepatosplenomegaly are uncommon, and patients typically present with nonspecific symptoms. IVLBCL presents a diagnostic challenge and patients are usually diagnosed late in the disease course, if at all, and the prognosis is poor. The differential diagnosis is broad, and physicians often pursue multiple diagnostic possibilities during patient workup. We present a case of IVLBCL discovered at autopsy in an 80-year-old male who presented with history and symptoms pointing to the tick-borne illness ehrlichiosis.

Intravascular large B-cell lymphoma

“…The systemic symptoms are mainly fever [5,31,32] and spleen enlargement [12,33], while lymphadenopathy is not reported.…”

“…The disease is usually diagnosed in the advanced stage [3,5], diagnosis in more than half of the cases is made posthumously [10]. Skin biopsy is an easy available diagnostic method, it is also recommend-ed for patients with suspected IVLBCL without skin lesions [31,33,34]. Sensitivity and specificity of random skin biopsy is estimated at 77.8% and 98.7% [35], not all authors agree with these values [36,37], but most of authors claim that skin biopsy could accelerate diagnosis.…”

“…1). Microcytic anaemia (Hb 7.6 g/dl), throm- [12,33], natomiast powiększenie obwodowych węzłów chłonnych nie jest opisywane.…”

“…Rozpoznanie ustala się zwykle w stadium zaawansowanym [3,5], w ponad połowie przypadków pośmiertnie [10]. Łatwo dostępnym narzędziem diagnostycznym jest biopsja skóry, przez niektórych autorów zalecana w przypadku nieobecności zmian skórnych u osoby z podejrzeniem IVBCL [31,33,34]. Czułość i swoistość biopsji z przypadkowego miejsca są szacowane odpowiednio na 77,8% i 98,7% [35], nie wszyscy autorzy są zgodni co do tych wartości [36,37], ale w ocenie większości wykonanie biopsji skóry może przyspieszyć rozpoznanie.…”

Rehabilitation Course and Management of Intravascular Lymphoma Presenting as Recurrent Strokes: A Case Report

“…However, the presentation of IVL can vary considerably and frequently mimics central nervous system disorders such as Guillain-Barré syndrome, dementia, central nervous system vasculitis, and stroke, making diagnosis difficult 2–4,7. As a result, IVL is often diagnosed either postmortem or late in the disease course when prognosis is very poor 2,4…”