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Background Chronic lymphocytic leukemia (CLL) is a clonal disorder of B lymphocytes, characterized by proliferation of small mature lymphocytes involving the blood, bone marrow, and lymphoid tissues. CLL can rarely involve the central nervous system (CNS), either by involving brain parenchyma or cerebrospinal fluid (CSF). We present a series of three cases with clinically significant involvement of the CNS with CLL. Results During the past 2 years, our medical center managed three patients with CLL who presented with symptomatic CNS involvement, as determined by flow cytometry. The immunophenotypic profile was that of a typical CLL with light chain restricted small B cells positive for CD20 (dim) and coexpressing CD5 and CD23. In addition, two patients had brain involvement by SLL that was confirmed by brain biopsy. Notably, the brain lesions had a mildly elevated Ki-67 proliferative index (10%-30%). Bone marrow was involved in two patients, showing nodular, interstitial, and diffuse patterns. Bone marrow involvement ranged from 60% to 80% and showed very low Ki-67 proliferative index. None of the patients had features suggestive of a CLL transformation. FISH was performed on either bone marrow or CSF and demonstrated that patient 1 had Del11q(ATM) and Dell13q, patient 2 had trisomy 12, and patient 3 had del17(TP53) and IGH rearrangement. All of the patients showed persistent CSF involvement, ranging from 4 to 12 weeks, requiring aggressive treatment with intrathecal chemotherapy. At the end of treatment, all of the patients were clear of CNS involvement as judged by flow cytometry of CSF. Conclusion We report three patients who had persistent involvement of CSF. Only one patient had del17(TP53), a cytogenetic feature that is associated with high-risk CLL. It would be interesting to study clonal evolution of CLL to understand the mechanisms that underlie involvement of the CNS.

Objectives: To report a patient with primary effusion lymphoma who was negative for human herpesvirus-8 (HHV-8), human immunodeficiency virus, Epstein-Barr virus, hepatitis C virus, and hepatitis B virus, as well as review 54 reported cases of HHV-8–unrelated primary effusion lymphoma (PEL)–like lymphoma in the literature to clarify the nature of this entity. Methods: The patients’ characteristics, clinical presentation, pathogenesis, morphologic-immunophenotypic features, clinical management, and prognosis were studied. Results: HHV-8–negative PEL-like lymphomas often occur in immunocompetent and elderly patients, are sometimes associated with chronic inflammation–related fluid overload, are mostly large B-cell or large B-cell with plasmacytic differentiation type, and are associated with a better prognosis. Conclusions: In various aspects, HHV-8–unrelated PEL-like lymphoma is a different entity from HHV-8–related PEL. Immunophenotype, morphology, and c-myc/8q24 status should be included for differential diagnosis. A test for c-myc or 8q24 abnormalities should be recommended for subdividing HHV-8–unrelated PEL-like lymphoma, which may have benefits in patient management.

Plasma cell neoplasms (PCNs) refer to a group of true plasma cell disorders that include but are not limited to monoclonal gammopathy of undetermined significance (MGUS), plasmacytoma, and plasma cell myeloma (PCM). Epstein-Barr virus (EBV) expression has been reported in several hematopoietic and nonhematopoietic disorders. However, EBV expression …

Plasma cell neoplasms (PCNs) refer to a group of true plasma cell disorders that include multiple myeloma, plasmacytoma, primary amyloidosis, and light and heavy-chain deposition diseases. Epstein-Barr virus (EBV) expression has been reported in several hematopoietic and nonhematopoietic disorders like Hodgkin lymphoma, Burkitt's lymphoma and nasopharyngeal carcinoma. Till recently, EBV expression in PCNs has been limited to cases with …

Solitary plasmacytoma of the bone (SPB) is a rare plasma cell neoplasm that usually presents as a lytic lesion mainly localized within the axial skeleton with no accompanying paraproteinemia or bone marrow involvement. The occurrence of SPB in young individuals is exceedingly rare. Trauma can cause enhanced release of cytokines resulting in increased proliferation of plasma cells. …