RESUMO
BACKGROUND: Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome (CS) in the presence of leukemic central nervous system infiltration is very rare. CASE: A 3.8-year-old girl who had been treated for B-cell acute lymphoblastic leukemia (ALL) for 1.5 years was admitted to our hospital with excessive weight gain and depression for the last 2 months. Prior to her admission, she was on maintenance with the ALL-BFM95 study protocol for 10 months that does not contain corticosteroids. On physical examination, central obesity and moon face appearance were determined. Laboratory tests revealed high morning ACTH, cortisol level, and 24-h urinary free cortisol level. Morning cortisol level was 33.94 nmol/L after a 2-day (4 × 0.5 mg) dexamethasone suppression test. A lumbar puncture revealed leukemic cells in the cerebrospinal fluid. No pituitary adenoma was detected on magnetic resonance imaging. We diagnosed the patient with ACTH-dependent CS related to leukemic infiltration of the central nervous system. CONCLUSION: Central nervous system infiltration should be considered in leukemic patients who have developed CS. We believe increased leukemia inhibitory factor levels may be a factor for CS in our patient with ALL.
Assuntos
Transformação Celular Neoplásica/metabolismo , Neoplasias do Sistema Nervoso Central/secundário , Síndrome de Cushing/etiologia , Fator Inibidor de Leucemia/metabolismo , Infiltração Leucêmica/fisiopatologia , Modelos Biológicos , Crise Blástica/metabolismo , Crise Blástica/patologia , Crise Blástica/fisiopatologia , Crise Blástica/psicologia , Neoplasias do Sistema Nervoso Central/patologia , Neoplasias do Sistema Nervoso Central/fisiopatologia , Neoplasias do Sistema Nervoso Central/psicologia , Pré-Escolar , Síndrome de Cushing/metabolismo , Depressão/etiologia , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Leucemia de Células B/tratamento farmacológico , Leucemia de Células B/metabolismo , Leucemia de Células B/patologia , Infiltração Leucêmica/metabolismo , Infiltração Leucêmica/patologia , Infiltração Leucêmica/psicologia , Quimioterapia de Manutenção , Obesidade Abdominal/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Recidiva , Aumento de PesoRESUMO
Leukemic infiltration of the central nervous system (CNS) resulting in neurological manifestations is a rare complication of chronic lymphocytic leukemia (CLL). Furthermore, symptomatic CNS involvement as the initial presentation of previously undiagnosed CLL is extremely rare. In the present report, the authors describe a case of an 89-year-old female previously diagnosed with Alzheimer's disease who suddenly developed rapidly worsening mental changes. Cytological and immunocytological examinations of the lymphoid cells present on the cerebrospinal fluid (CSF) revealed CNS involvement by a clonal B-cell lymphoproliferative disorder, most consistent with de novo B-CLL expressing kappa light chain restriction. Subsequently, flow cytometric analysis done on the peripheral blood lymphocytes confirmed the diagnosis of B-CLL in this patient. Thus, this study shows the potential usefulness of immunocytological evaluation in detecting monoclonal lymphoid populations on CSF samples in adult patients presenting with altered mental status and CSF pleocytosis of lymphocytes.
