Hemophagocytic lymphohistiocytosis (HLH) is a rare autosomal recessive disorder of infancy and childhood that is invariably fatal if not treated. We report on the first patient to receive post-natal HSCT for HLH after receiving in utero chemotherapy for disease stabilization.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2592085PMC
http://dx.doi.org/10.1002/pbc.21778DOI Listing

Publication Analysis

Top Keywords

hemophagocytic lymphohistiocytosis
8
pre- post-natal
4
post-natal treatment
4
treatment hemophagocytic
4
lymphohistiocytosis hemophagocytic
4
lymphohistiocytosis hlh
4
hlh rare
4
rare autosomal
4
autosomal recessive
4
recessive disorder
4

Similar Publications

Hemophagocytic lymphohistiocytosis post chimeric antigen receptor T cell therapies.

Expert Rev Clin Immunol

December 2024

Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Introduction: Besides cytokine release syndromes (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS), immune effector cell-associated HLH-like syndrome (IEC-HS) is increasingly recognized across CAR-T recipients. This emergent and fatal syndrome is difficult to separate from other disorders during the early phase, and urgently requires more integrated diagnostic and therapeutic frameworks.

Areas Covered: Existing literature has pointed out the potential role of unbridled proliferation of cytotoxic T lymphocytes, lymphopenia of natural killing cells, and hypercytokinemia in triggering the IEC-HS.

View Article and Find Full Text PDF

Secondary hemophagocytic lymphohistiocytosis (HLH) syndrome, a fatal disorder characterized by NK/T-cell deficiency, cytokine storm, and organ damage, is rare in chronic lymphocytic leukemia (CLL). Ibrutinib, the first generation of irreversible Bruton's tyrosine kinase inhibitor, has been the first-line therapy for CLL. As an off-target effect, it can also block IL-2 inducible T-cell kinase (ITK), which is essential in maintaining normal NK and T-cell functions.

View Article and Find Full Text PDF

Hemophagocytic lymphohistiocytosis is associated with disseminated tuberculosis in patients with advanced HIV infection.

J Infect

December 2024

Guangzhou Medical Research Institute of Infectious Diseases, Infectious Disease Center, Guangzhou Eighth People's Hospital, Guangzhou Medical University, Guangzhou, Guangdong 510440, China. Electronic address:

View Article and Find Full Text PDF

Low-dose emapalumab combined with chemotherapy for adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

Transpl Immunol

December 2024

National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Department of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China. Electronic address:

Hemophagocytic lymphohistiocytosis (HLH) is a severe disorder with poor clinical outcomes. Use of emapalumab, an IFN-γ inhibitor, enables primary HLH control in over 85 % of affected children. However, data on emapalumab use for Epstein-Barr virus-associated HLH (EBV-HLH) are limited.

View Article and Find Full Text PDF

DOCK8 at the crossroads of immunodeficiency and hyperinflammation.

J Allergy Clin Immunol

December 2024

Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Mass. Electronic address:

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!