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Frequency and severity of central nervous system lesions in hemophagocytic
lymphohistiocytosis
E Haddad, ML Sulis, N Jabado, S Blanche, A Fischer and M Tardieu
Unite d'Immuno-Hematologie et Unite INSERM 429, Hopital Necker-Enfants
Malades, Paris, France.
We have retrospectively assessed the neurological manifestations in 34
patients with hemophagocytic lymphohistiocytosis (HLH) in a single center.
Clinical, radiological, and cerebrospinal fluid (CSF) cytology data were
analyzed according to treatment modalities. Twenty-five patients (73%) had
evidence of central nervous system (CNS) disease at time of diagnosis,
stressing the frequency of CNS involvement early in the time course of HLH.
Four additional patients who did not have initial CNS disease, who did not
die early from HLH complications, and who were not transplanted, also
developed a specific CNS disease. Therefore, all surviving and
nontransplanted patients had CNS involvement. Initially, CNS manifestations
consisted of isolated lymphocytic meningitis in 20 patients and meningitis
with clinical and radiological neurological symptoms in nine patients. For
these nine patients, neurological symptoms consisted of seizures, coma,
brain stem symptoms, or ataxia. The outcome of patients treated by systemic
and intrathecal chemotherapy and/or immunosuppression exclusively (n = 16)
was poor, as all died following occurrence of multiple relapses or CNS
disease progression in most cases. Bone marrow transplantation (BMT) from
either an HLA identical sibling (n = 6) or haplo identical parent (n = 3)
was performed in nine patients, once first remission of CNS and systemic
disease was achieved. Seven are long-term survivors including three who
received an HLA partially identical marrow. All seven are off treatment
with normal neurological function and cognitive development. In four other
patients, BMT performed following CNS relapses was unsuccessful. Given the
frequency and the poor outcome of CNS disease in HLH, BMT appears,
therefore, to be the only available treatment procedure that is capable of
preventing HLH CNS disease progression and that can result in cure when
performed early enough after remission induction.
Volume 89,
Issue 3,
pp. 794-800,
02/01/1997
Copyright © 1997 by The American Society of Hematology
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