Central nervous system involvement in CD4+/CD56+ hematodermic neoplasm: a report of two cases

Abstract

CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK-cell lymphoma, is an uncommon, aggressive non-Hodgkin's lymphoma with cutaneous, lymph node, and bone marrow involvement at presentation. The disease is characterized by early leukemic phase; however, central nervous system involvement is rarely reported. Herein we describe two cases of CD4+/CD56+ hematodermic neoplasm with meningeal manifestation. Microscopic analysis and flow cytometry of cerebrospinal fluid proved to be diagnostic; however, imaging studies were not informative. These observations call attention to the possibility of central nervous system involvement, which could be more common than expected previously. Authors recommend routine cerebrospinal fluid analysis and prophylactic intrathecal chemotherapy in patients with this highly aggressive disease.

Blastic Plasmacytoid Dendritic Cell Neoplasm with Extensive Cutaneous and Central Nervous System Involvement

“…Bone marrow, peripheral blood, lymph nodes and soft tissues are commonly involved, but CNS involvement is more unusual and reported in only a few cases. 3 , 4 The term blastic was initially adopted because the tumor is composed of medium to large-sized cells with fine chromatin and scanty cytoplasm resembling lymphoblasts, or in some cases, myeloblasts, 5 and may on occasion exhibit sub-membranous cytoplasmic vacuolations surrounding the nucleus. The leukemic cells are usually agranular, and angiodestructive or necrotic lesions are rare.…”