Diagnosis and Management of Leukemic and Lymphomatous Meningitis

Hemant Murthy, MD; Claudio Anasetti, MD; Ernesto Ayala, MD

Disclosures

Cancer Control. 2017;24(1):33-41. 

In This Article

Abstract and Introduction

Abstract

Background. Leukemic and lymphomatous meningitis is a major presentation of primary or secondary central nervous system (CNS) involvement by aggressive lymphomas or acute leukemia.

Methods. The medical literature and ongoing clinical trials were reviewed on the clinical presentation, diagnosis, prognosis, prevention, and treatment of leukemic and lymphomatous meningitis.

Results. Treatment for secondary leukemic and lymphomatous meningitis remains unsatisfactory, and efforts should be made to prevent and treat subclinical disease. Intrathecal and systemic chemotherapy remain the main therapeutic approaches for this disease. Outcomes have improved in patients with primary CNS lymphoma and meningeal involvement.

Conclusions. Appropriate selection of patients at high risk for leukemic and lymphomatous meningitis is important so that preventive strategies can decrease the incidence of this complication of leukemia and lymphoma. Use of chemotherapy agents that cross the blood–brain barrier and the adoption of high-dose chemotherapy with autologous hematopoietic stem cell transplantation have increased the proportion of patients whose primary disease is cured.

Introduction

Leukemic and lymphomatous meningitis may occur in patients with primary central nervous system (CNS) lymphoma or as a secondary manifestation of systemic lymphoma or leukemia. In rare cases, leptomeningeal involvement may be the initial presentation of these diseases. Although its clinical presentation is variable, leukemic and lymphomatous meningitis from hematological malignancies tends to have a higher frequency of cranial nerve involvement than solid tumors.[1]

Diagnosis is based on findings after cerebrospinal fluid (CSF) examination and radiological evaluation of the neuraxis. Therapeutic options include radiotherapy, intrathecal (IT) and systemic chemotherapy, and, in select cases, high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HSCT).

In patients with secondary leukemic and lymphomatous meningitis, treatment remains suboptimal and frequently is palliative. The most appropriate strategy is aggressive prevention. By contrast, patient outcomes have improved with the use of modern intensive approaches for leukemic and lymphomatous meningitis associated with primary CNS lymphoma (PCNSL).

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