AH Filipovich - Immunology and Allergy Clinics of North America, 2008 - Elsevier Hemophagocytic disorders result when critical regulatory pathways responsible
for the natural termination of immune/inflammatory responses are disrupted or
overwhelmed. Hemophagocytic disorders reflect pathologic defects that alter ... Cited by 8 - Related articles - All 38 versions
AC Horne, KG Ramme, E Rudd, C Zheng, Y Wali … - British Journal of Haematology, 2008 - ingentaconnect.com Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive
lethal condition characterized by fever, cytopenia, hepatosplenomegaly and
hemophagocytosis. The hallmark of FHL is defect apoptosis triggering and ... Cited by 6 - Related articles - All 3 versions
- ►hematologylibrary.org U Moreno… - Blood, 2009 - bloodjournal.hematologylibrary.org Perforin-mediated lymphocyte cytotoxicity is critical for pathogen elimination
and immune homeostasis. Perforin disruption of target cell membranes is
hypothesized to require binding of a calcium-dependent, lipid-inserting, C2 ... Related articles - All 2 versions
MJ Vermeulen, V de Haas, MF Mulder, C … - European Journal of Medical Genetics, 2009 - Elsevier Familial hemophagocytic lymphohistiocytosis (FHLH) is a genetic heterogeneous
autosomal recessive disorder. We report two siblings with FHLH caused by a PRF1
mutation. The first child died in utero with hydrops fetalis and the second ... Related articles - All 2 versions
- ►haematologica-thj.org [PDF] A Santoro, S Cannella, A Trizzino, G Bruno, … - Haematologica, 1996 - haematologica.org Mutations of UNC13D have been described in patients affected by familial
hemophagocytic lymphohistiocytosis (FHL3). The Munc13-4 protein contributes to
the priming of the secretory granules. Mutation in this gene results in ... Related articles - All 4 versions
AC Horne - diss.kib.ki.se Page 1. DEPARTMENT OF WOMAN AND CHILD HEALTH Karolinska Institutet, Stockholm,
Sweden CLINICAL AND EPIDEMIOLOGICAL STUDIES OF HAEMOPHAGOCYTIC ... Related articles
- ►haematologica.com A Santoro, S Cannella, A Trizzino, G Bruno, C … - Haematologica, 2008 - haematologica.com Mutations of UNC13D have been described in patients affected by familial
hemophagocytic lymphohistiocytosis (FHL3). The Munc13-4 protein contributes to
the priming of the secretory granules. Mutation in this gene results in ... Related articles - All 12 versions
F Al-Jasmi, M Abdelhaleem, T Stockley, KS … - Journal of Pediatric Hematology/Oncology, 2008 - journals.lww.com Familial hemophagocytic lymphohistiocytosis is a rare disorder characterized by
lethal primary immunodeficiency associated with hypercytokinemia and a
concomitant defect in natural killer cell cytotoxicity. We report a fatal ... Related articles - All 2 versions
AK Sarcon, MJ Desierto, W Zhou, V Visconte, … - Experimental Hematology, 2009 - Elsevier Prf −/− mice showed normal lymphoid cell composition, but Prf −/− LN
cells had reduced ability to induce C.B10 BM cell apoptosis in vitro. Infusion
of 5 to 10 × 10 6 Prf −/− LN cells produced obvious BM failure in ... Related articles - All 9 versions
FO Smith - cincinnatichildrens.org First Row: F. Smith, T. Kalfa, R. Gruppo; Second Row: S. Wells, M. Fouladi, T.
Cripe; Third Row: K. Burns, S. Joshi, P. Mehta, L. Wagner; Fourth Row: K.
Kalinyak; Fifth Row: R. Nagarajan, B. Weiss; Sixth Row: C. Joiner, R. ... Cited by 1 - Related articles - View as HTML - All 3 versions
