A Case of Chronic Lymphocytic Leukemia Complicated by Hemophagocytic Lymphohistiocytosis: Identifying the Aberrant Immune Response.
J Gen Intern Med
; 37(6): 1542-1546, 2022 05.
Article
en En
| MEDLINE
| ID: mdl-35178648
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that may complicate hematologic malignancies. HLH and malignancies have common clinical features, and HLH diagnostic criteria (HLH-2004/Hscore) were not validated in this specific population. We describe a case of a 72-year-old patient with a history of chronic lymphocytic leukemia stable for over 10 years who presented with fever and cytopenia. After excluding infectious etiologies and the progression of her disease, HLH was diagnosed. The patient was treated with etoposide, dexamethasone, intravenous immunoglobulin, and rituximab. Despite initial clinical improvement, the patient deteriorated and developed pulmonary aspergillosis and CNS involvement that reflected uncontrolled HLH. The patient died 45 days after her presentation. An unusual feature of this case was that HLH was not triggered by infection, disease transformation, or treatment. This case emphasizes the challenges of differentiating the development of overwhelming HLH from other complications associated with hematologic malignancy.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Leucemia Linfocítica Crónica de Células B
/
Linfohistiocitosis Hemofagocítica
Tipo de estudio:
Diagnostic_studies
/
Prognostic_studies
Límite:
Aged
/
Female
/
Humans
Idioma:
En
Revista:
J Gen Intern Med
Asunto de la revista:
MEDICINA INTERNA
Año:
2022
Tipo del documento:
Article
País de afiliación:
Israel