Central Nervous System Hemophagocytic Lymphohistiocytosis (CNS-HLH) from Leptomeningeal Anaplastic Large Cell Lymphoma

Lehrer, Helaina; Scigliano, Eileen; Chan, Amy

doi:10.1007/s00062-021-00998-3

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Cited by 2 publications

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“…Few studies have reported CNS involvement in HLH secondary to lymphoma. We searched the literature for studies on CNS involvement in HLH secondary to lymphoma that provided detailed MRI data ( 15 , 18 , 20 , 23 ) ( Table 1 ). The mean age of the 5 patients (including the present patient) was 50±21 years, and 3 were female (60%).…”

Section: Discussionmentioning

confidence: 99%

Central nervous system involvement in adult-onset hemophagocytic lymphohistiocytosis secondary to lymphoma: a case presentation and literature analysis

He¹,

Zhong²,

Li³

et al. 2023

Quant Imaging Med Surg

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Section: Discussionmentioning

confidence: 99%

Central nervous system involvement in adult-onset hemophagocytic lymphohistiocytosis secondary to lymphoma: a case presentation and literature analysis

He¹,

Zhong²,

Li³

et al. 2023

Quant Imaging Med Surg

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Autoimmune encephalitis followed by hemophagocytic lymph histiocytosis: a case report

Huang,

Tan,

Lin

et al. 2024

Front. Immunol.

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ObjectiveThis study aims to report three cases of autoimmune encephalitis followed by hemophagocytic lymphohistiocytosis.MethodsData of relevant patients treated between 2019 and 2022 were retrospectively collected from the Department of Neurology at the Second Affiliated Hospital of Guangzhou Medical University.ResultsThe age at onset of the three patients was 37, 63, and 36 years, respectively. All three patients were female and presented with cognitive dysfunction and seizures. Behavioral and psychological symptoms were also observed in two cases. All patients were positive for autoantibodies in both the cerebrospinal fluid and serum, while two showed multiple abnormal brain signals on magnetic resonance imaging. All patients exhibited hypocytosis and elevated soluble CD25 and serum ferritin levels. The final diagnoses in two cases were lymphomas, while the remaining case without tumors suffered from a severe infection. All patients received immunotherapy, and the two with lymphoma received anti-tumor treatment. The patient with infection died, and two patients with tumors improved after chemotherapy.ConclusionAutoimmune encephalitis followed by hemophagocytic lymphohistiocytosis is a rare and severe condition. Prompt attention should be paid to the decline in blood cell counts, particularly in patients who show a slight improvement after immunotherapy or have a risk of lymphoma. Screening for potential tumors and infections and early treatment may help these patients.

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Central Nervous System Hemophagocytic Lymphohistiocytosis (CNS-HLH) from Leptomeningeal Anaplastic Large Cell Lymphoma

Cited by 2 publications

References 13 publications

Central nervous system involvement in adult-onset hemophagocytic lymphohistiocytosis secondary to lymphoma: a case presentation and literature analysis

Central nervous system involvement in adult-onset hemophagocytic lymphohistiocytosis secondary to lymphoma: a case presentation and literature analysis

Autoimmune encephalitis followed by hemophagocytic lymph histiocytosis: a case report

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