Pituitary Gland and Neurological Involvement in a Case of Hemophagocytic Syndrome Revealing an Intravascular Large B-Cell Lymphoma

Abstract

Intravascular large B-cell lymphoma is a rare entity characterized by the proliferation of neoplastic lymphocytes in the lumen of small blood vessels and high mortality. Diagnosis of intravascular lymphoma is often delayed or established postmortem. Here, we report the case of a 48-year-old woman presenting hemophagocytic syndrome, with pituitary gland and neurological involvement. Diagnosis of intravascular large B-cell lymphoma was made on perisplenic vessels, while liver and bone marrow biopsy was noncontributive. This case demonstrates the importance of thorough histopathologic investigations in the setting of high suspicion.

Figure 1

PET-CT hypermetabolic splenomegaly, perihepatic hypermetabolic lymph nodes, and cervical hypermetabolism.

Figure 2

Hemophagocytosis at bone marrow aspiration.

Figure 3

Brain MRI showing enlargement of the pituitary gland and pituitary stalk without evidence of adenoma.

Figure 4

Brain MRI showing left prerolandic lesion and medial medullary hypersignal. (a) Prerolandic lesion with homogeneous corticosubcortical ring enhancement. (b) Medial medullary hypersignal in T2 and flair.

Figure 5

Histopathology of a splenectomy piece. (a) Extramedullary hematopoiesis (H&E, 10x). (b) Extramedullary hematopoiesis (H&E, 40x) with megakaryocytes (blue arrow), erythroblasts (orange arrow), and neutrophils (green arrow). (c) CD20 immunostaining showing no spleen infiltration (scale on image).

Figure 6

Histopathology of perisplenic tissues. (a) Tumoral intravascular infiltration (arrows) (H&E, 10x). (b) Large hyperchromatic cell inside small vessels around splenic lymph nodes (H&E, 40x). (c) Strong positive CD20 B-cell surface expression. (d) Positive expression of MUM1. (e) Positive expression of PAX5.