Intravascular B-Cell Lymphoma: An Elusive Diagnosis

Abstract

Intravascular B-cell lymphoma (IVBCL) is a rare and aggressive subtype of extranodal diffuse large B-cell lymphomas. First described by Pflegrand and Tappeiner 1 in 1959 as "angioendotheliomatosis proliferans systemisata," it is characterized by preferential growth of neoplastic lymphoma cells within the lumina of small blood vessels, particularly capillaries. Despite the 50 years that have passed since the first description of this syndrome, our understanding about its pathophysiology is still limited, and the syndrome continues to be a diagnostic and therapeutic challenge for practicing hematologists and oncologists. In this article, we describe an unusual case of IVBCL in a patient who presented with neurologic symptoms, hemolytic anemia, and synchronous carcinoma. Case ReportA previously healthy 47-year-old African American male presented with slurred speech, left-sided facial droop, and upperextremity jerky movements. A neurologic examination showed left, upper motor neuron facial nerve palsy. The laboratory workup was significant for mild leucocytosis with a WBC count of 15 ϫ 10 9 /L with a normal hemoglobin and platelet count. Magnetic resonance imaging (MRI) of the brain showed multiple small bright lesions mainly in the right cerebral hemisphere associated with vasogenic edema. An embolic or vascular phenomenon was favored in the differential diagnosis. Spinal fluid analysis did not reveal neoplastic cells. Computed tomography (CT) of the chest, abdomen and pelvis showed bilateral adrenal lesions, and positron emission tomography/CT showed hypermetabolic disease only in bilateral adrenal glands. A core-needle biopsy of the left adrenal lesion revealed a poorly differentiated epithelial neoplasm. Cytokeratin was the only stain that was positive of the multiple stains tested on the specimen. Because of continued confusion regarding the etiology of brain lesions (metastatic disease v ischemia), a brain biopsy was recommended, which the patient declined. While in the hospital, the patient had an acute decline in hemoglobin to 6.5 g/dL. The anemia workup revealed Coombs-positive hemolytic anemia. An esophagogastroduodenoscopy and colonoscopy were negative for malignant lesions. Prednisone 1 mg/kg was started, and a rapid improvement in hemoglobin was seen in the next several days. The patient left the hospital against medical advice and was seen in a clinic with a lag period of 2 months. The patient was completely asymptomatic at that point, and steroids were tapered off. Restaging CT scans of the chest, abdomen, and pelvis showed interval improvement in the size of adrenal lesions. MRI of the brain revealed old ischemic changes but no evidence of a discrete metastatic lesion.The patient remained asymptomatic for the next few months until he started having vague abdominal discomfort. Repeat-staging studies showed an interval increase in the size of adrenal lesions. While D I A G N O S I S I N O N C O L O G Y V O L U M E 3 0 ⅐ N U M B E R 1 5 ⅐ M A Y 2 0 2 0 1 2 e144

The labyrinth of pyrexia of unknown origin: A case of intravascular B cell lymphoma

“…Median age of presentation is 70 years with particularly male predominance 3. Two variants have been described based on geographical prevalence: a) Classical variant characterized by neurological and dermatological manifestations, generally seen in the western population and b) Asian variant characterized by hematological manifestations like hemophagocytic syndrome, hepatosplenomegaly, fever and thrombocytopenia, most commonly described in Asian population, particularly Japan 1. The Asian variant of IVLBCL, namely with isolated bone marrow involvement in Caucasians is an extremely rare occurrence and has been reported in very few instances.…”

“…A defect in the adhesion molecules CD 29, CD54 (intercellular adhesion molecule-1) and CD 11a is probably the root of intravascular localization of the tumor cells. The neoplastic cells commonly express B cell antigens CD 19, CD 20 and CD 79a,1 however there is no characteristic cytogenetic aberration 1. Involvement can be ubiquitous with varied symptomatology.…”

“…First described by Pflegrand and Tappeiner1 in 1959 as “angioendotheliomatosis proliferans systemisata”, it is characterized by preferential growth of neoplastic lymphoma cells within the lumina of small blood vessels, particularly capillaries 1…”

Linfoma intravascular, un desafio diagnóstico: Caso clínico

“…Las recomendaciones se realizan extrapolando de la experiencia con subtipos más frecuentes de linfoma difuso de células grandes B. Todos los casos de LI se consideran diseminados por lo tanto se tratan como enfermedad avanzada. Se recomienda quimioterapia asociada del tipo del R-CHOP en casos de inmunofenotipos compatibles con células B [19][20][21][22][23][24] . Si bien el trasplante autólo-go de medula ósea es una opción en algunos casos (en especial jóvenes) luego de la primera remisión, en general se indica como terapia de salvataje 23,24 .…”

“…Se recomienda quimioterapia asociada del tipo del R-CHOP en casos de inmunofenotipos compatibles con células B [19][20][21][22][23][24] . Si bien el trasplante autólo-go de medula ósea es una opción en algunos casos (en especial jóvenes) luego de la primera remisión, en general se indica como terapia de salvataje 23,24 . La terapia con R-CHOP no atraviesa la barrera hematoencefálica por lo que se recomienda terapia intratecal ya sea como profilaxis o tratamiento 19 .…”