Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution

Rodrigues, Jéssica Benigno; Nasr, Bárbara Pinto; Cypriano, Mônica

doi:10.1016/j.htct.2021.05.003

Cited by 3 publications

(1 citation statement)

References 14 publications

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“…Given the high risk of EBV infection (6.02 × 10 4 IU/mL) before transplant, the slow speed of immune reconstitution, high recurrent EBV infection rate, and increased incidence of post-transplant lympho-proliferation-related disease (PTLD), post-transplant cyclophosphamide (PT-Cy) was given on +3d and +4d. On the other hand, the patient experienced reactive disease before a salvage transplant which was high probability of relapse ( 8 ). To improve patient survival, transplant strategies need to be optimized.…”

Section: Case Informationmentioning

confidence: 99%

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Bingjie,

Linlin,

Futian

et al. 2024

Front. Immunol.

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ObjectivesThis study aims to discuss the clinical manifestations and treatment of Familial hemophagocytic lymphohistiocytosis (FHL) caused by a mutation in the UNC13D gene.MethodsA 6-year-old female child presented with unexplained febricity, splenomegaly, pancytopenia, hemophagocytic lymphohistiocytosis in bone marrow, decreased NK cell activity, soluble CD25 levels > 44000ng/ml. Genetic sequencing revealed a mutation in the UNC13D gene. Additionally, the patient experienced intermittent fever with seizures characterized by involuntary twitching of the left upper limb. Head magnetic resonance imaging (MRI) showed white matter lesions.ResultsAccording to the HLH-2004 diagnostic criteria revised by the International Society of Histiocytosis the patient was diagnosed with FHL. Despite receiving HLH-2004 treatment, the disease relapsed. However, after a salvage allogeneic Hematopoietic Stem Cell Transplant (HSCT), febricity, abnormal blood cells, and neurological symptoms significantly improved.ConclusionsPrompt performance of allogeneic HSCT is crucial upon diagnosis of FHL, especially when neurological involvement is present.

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Section: Case Informationmentioning

confidence: 99%

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Bingjie,

Linlin,

Futian

et al. 2024

Front. Immunol.

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Cutaneous Perivascular Hemophagocytosis: A Report of 2 Cases With Emphasis on Clinical and Histological Features

Cazzato,

Rongioletti

2024

The American Journal of Dermatopathology

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Cutaneous perivascular hemophagocytosis (CH) is a histological manifestation that manifests as systemic hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, when accompanied by fever, hepatosplenomegaly, liver dysfunction, and cytopenia, and may rarely manifest independently of hemophagocytic lymphohistiocytosis. CH typically presents as purpuric or brownish macules and patches on the extremities, abdomen, and trunks. Histopathologically, the hallmark of CH includes extravasated erythrocytes and karyorrhectic debris phagocytized by histiocytes, associated with dermal capillary ectasia, perivascular infiltration of neutrophils, nuclear dust, and histiocytes without atypia. In this study, we report 2 cases of CH encountered in routine diagnostic practice and elucidate their significant clinical and histologic features. Our first patient had leukocytoclastic vasculitis with CH in the setting of Yersinia enterocolitis, and the second case represents CH in association with non-Hodgkin lymphoma. This study highlights the importance of considering CH as a potential indicator of underlying systemic pathology, including infectious and hematological disorders, in clinical practice.

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Cure of hemophagocytic lymphohistiocytosis triggered by Epstein–Barr virus infection after early diagnosis and appropriate treatment with etoposide, dexamethasone, and immunoglobulin

Faleiros de Azevedo,

Sversut Brionte,

Freitas Junior

2024

IJFCM

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Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening condition characterized by uncontrolled activation of T lymphocytes and inappropriate activation of mature macrophages secreting excessive cytokines leading to marked hypercytokinemia. It can also be secondary to neoplasms, rheumatologic diseases, or infections. This was a case report, described in Central Brazil, of a 17-year-old female patient diagnosed with HLH after infection by Epstein–Barr virus (EBV), which eventually improved after early diagnosis and treatment with etoposide, dexamethasone, and immunoglobulin. The early diagnosis and treatment of this condition are of utmost importance owing to its high mortality rate. Therefore, this is a case report of a young patient who developed HLH, after EBV infection, requiring transfer to a reference hospital for infectious diseases, with optimization of diagnosis and early initiation of specific treatment after discussion between a medical team made up of several specialists.

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Hemophagocytic lymphohistiocytosis: presentation and outcome of twenty-one patients at a single institution

Cited by 3 publications

References 14 publications

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Allogeneic hematopoietic stem cell transplant for familial hemophagocytic lymphohistiocytosis: a case report and literature review

Cutaneous Perivascular Hemophagocytosis: A Report of 2 Cases With Emphasis on Clinical and Histological Features

Cure of hemophagocytic lymphohistiocytosis triggered by Epstein–Barr virus infection after early diagnosis and appropriate treatment with etoposide, dexamethasone, and immunoglobulin

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