Initial presentation of CNS-restricted acute lymphoblastic B cell leukaemia as peripheral polyneuropathy.
We report a case of a 58-year-old woman who presented with a 1-month course of progressive lower and upper extremity weakness in addition to binocular diplopia. Diagnostic lumbar puncture revealed atypical lymphoid cells with 28% blasts. Immunophenotype was consistent with B cell acute lymphoblastic leukaemia (B-ALL). Further work up showed no systemic involvement but extensive thoracolumbar-sacral leptomeningeal disease. The patient was treated with several courses of intrathecal and systemic chemotherapy followed by craniospinal irradiation for consolidation. There was initial steady improvement in neurological symptoms and leptomeningeal disease, the latter being ascertained through radiological studies and cerebrospinal fluid examination. After 10 months of response, the patient relapsed with central nervous system (CNS) and systemic disease. B-ALL is a rare precursor lymphoid neoplasm that generally presents with systemic disease. While CNS involvement is not uncommon, isolated involvement of this compartment without systemic disease is exceedingly rare.
Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Guilherme
G
Piovezani Ramos
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Jose C
JC
Villasboas Bisneto
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Dong
D
Chen
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Animesh
A
Pardanani
D016543:Central Nervous System Neoplasms / Q000150:complications*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D011115:Polyneuropathies / Q000209:etiology*; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications*