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1 ar to that of macrophage activation syndrome/hemophagocytic lymphohistiocytosis.
2 rimary immunodeficiencies including familial hemophagocytic lymphohistiocytosis.
3 ells, resulting in the development of lethal hemophagocytic lymphohistiocytosis.
4 e 18 years of age or younger and had primary hemophagocytic lymphohistiocytosis.
5 ppropriately classified as X-linked familial hemophagocytic lymphohistiocytosis.
6 equired to induce disease in mouse models of hemophagocytic lymphohistiocytosis.
7 g them are Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis.
8 y results in several human diseases, such as hemophagocytic lymphohistiocytosis.
9 des, T-cell lymphoproliferative disease, and hemophagocytic lymphohistiocytosis.
10 mutations in Munc13-4 as a cause of familial hemophagocytic lymphohistiocytosis.
11 with familial and virus-associated reactive hemophagocytic lymphohistiocytosis.
12 are associated with some cases of CAEBV with hemophagocytic lymphohistiocytosis.
13 ked lymphoproliferative disease and familial hemophagocytic lymphohistiocytosis.
14 he multisystem organ failure associated with hemophagocytic lymphohistiocytosis.
15 perforin have been reported in patients with hemophagocytic lymphohistiocytosis.
16 AdV type 41, one of whom developed secondary hemophagocytic lymphohistiocytosis.
17 ion related to severely elevated ferritin is hemophagocytic lymphohistiocytosis.
18 ria serves as a valuable diagnostic tool for hemophagocytic lymphohistiocytosis.
19 etiology was associated with younger age and hemophagocytic lymphohistiocytosis.
20 y and immune regulatory disorders, including hemophagocytic lymphohistiocytosis.
21 apy, disease progression, and development of hemophagocytic lymphohistiocytosis.
22 ulation, dysgammaglobulinemia, lymphoma, and hemophagocytic lymphohistiocytosis.
23 etic cell transplantation in severe familial hemophagocytic lymphohistiocytosis.
24 lammation associated with signs of fulminant hemophagocytic lymphohistiocytosis.
25 1 gene, confirming the diagnosis of familial hemophagocytic lymphohistiocytosis.
26 s targeted therapy for patients with primary hemophagocytic lymphohistiocytosis.
27 east five of the eight HLH-2004 criteria for hemophagocytic lymphohistiocytosis.
28 o those implicated in the setting of primary hemophagocytic lymphohistiocytosis.
29 escribed by examining patients with familial hemophagocytic lymphohistiocytosis.
30 erest in discussing a specific treatment for hemophagocytic lymphohistiocytosis.
31 esults occurred only for infection-triggered hemophagocytic lymphohistiocytosis.
34 c lymphohistiocytosis diagnosis was based on Hemophagocytic Lymphohistiocytosis-2004 criteria and the
35 tify Munc13-4, a protein mutated in familial hemophagocytic lymphohistiocytosis 3, as a WPB-tethering
36 mutations account for 20% to 40% of familial hemophagocytic lymphohistiocytosis, a fatal disease of e
37 ng mutations in these genes develop familial hemophagocytic lymphohistiocytosis, a primary immunodefi
38 ed odds ratio, 1.86; 95% CI, 1.15-2.99), and hemophagocytic lymphohistiocytosis (adjusted odds ratio,
39 ed odds ratio, 3.54; 95% CI, 1.80-6.95), and hemophagocytic lymphohistiocytosis (adjusted odds ratio,
40 ty arose in 4 patients (2/4, grade 3-4), and hemophagocytic lymphohistiocytosis affected 2 patients.
41 chosocial outcomes in childhood survivors of hemophagocytic lymphohistiocytosis after hematopoietic s
42 24fs60*) previously associated with familial hemophagocytic lymphohistiocytosis and a novel homozygou
43 nts with a clinically suspected diagnosis of hemophagocytic lymphohistiocytosis and found 28 patients
45 0.99-0.99) and 0.99 (95% CI, 0.99-0.99) for hemophagocytic lymphohistiocytosis and HScore, respectiv
46 ment is indicated for patients with familial hemophagocytic lymphohistiocytosis and is often valuable
47 Although it has been proposed that in both hemophagocytic lymphohistiocytosis and macrophage activa
48 as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activa
49 ic benefits in preclinical disease models of hemophagocytic lymphohistiocytosis and multiple sclerosi
50 -eating macrophages, is seen in the disorder hemophagocytic lymphohistiocytosis and other inflammator
51 f cytopenia and auto-inflammation, including hemophagocytic lymphohistiocytosis and rash in the most
53 yperinflammatory syndromes, however, such as hemophagocytic lymphohistiocytosis and the newly classif
54 iated hydroa vacciniforme-like lymphoma with hemophagocytic lymphohistiocytosis, and 2 had EBV-positi
55 including some peripheral T-cell lymphomas, hemophagocytic lymphohistiocytosis, and chronic active E
56 rtality during SARS-CoV-2 infection, sepsis, hemophagocytic lymphohistiocytosis, and cytokine shock.
57 rtality during SARS-CoV-2 infection, sepsis, hemophagocytic lymphohistiocytosis, and cytokine shock.
58 and malignant lymphoproliferative disorders, hemophagocytic lymphohistiocytosis, and smooth muscle an
59 were to identify the frequency of secondary hemophagocytic lymphohistiocytosis, and the main prognos
60 atory phenomena such as hyperinflammation or hemophagocytic lymphohistiocytosis are a frequent yet pa
62 ogic abnormalities in patients with familial hemophagocytic lymphohistiocytosis are decreased natural
63 92.5% sensitivity and 91.9% specificity for hemophagocytic lymphohistiocytosis (area under the curve
64 e for treating some cases of type 2 familial hemophagocytic lymphohistiocytosis, based on the pharmac
65 atients admitted with suspected or diagnosed hemophagocytic lymphohistiocytosis, between January 1, 2
66 isease and central nervous system-restricted hemophagocytic lymphohistiocytosis, can be non-invasivel
67 haracterized by immunodeficiency and a fatal hemophagocytic lymphohistiocytosis caused by impaired fu
69 rve was then of 0.98 (95% CI, 0.96-0.99) for hemophagocytic lymphohistiocytosis criteria and 0.99 (95
70 nding factors, our study suggests HScore and hemophagocytic lymphohistiocytosis criteria to be highly
71 ic syndrome- patients (p < 0.001); number of hemophagocytic lymphohistiocytosis criteria was 4 (4-5)
73 D, STX11, or STXBP2 associated with familial hemophagocytic lymphohistiocytosis, CTL and NK cell degr
74 tion, hyperinflammatory syndromes, including hemophagocytic lymphohistiocytosis, developed after SARS
87 f PBMCs obtained from patients with familial hemophagocytic lymphohistiocytosis (FHL) to screen for b
88 Cytokine storm syndromes, such as familial hemophagocytic lymphohistiocytosis (FHL), are lethal dis
89 of patients, referred to as having familial hemophagocytic lymphohistiocytosis (FHL), have various u
92 -4, in which mutations cause type 3 familial hemophagocytic lymphohistiocytosis (FHL3), a fatal disea
93 (NK) cytotoxicity, and give rise to familial hemophagocytic lymphohistiocytosis (FHL4 or FHL5, respec
95 ive disorders collectively known as familial hemophagocytic lymphohistiocytosis (FHLH), all associate
96 a, in addition to meningoencephalitis and/or hemophagocytic lymphohistiocytosis following live-attenu
97 We analyze ferritin levels to differentiate hemophagocytic lymphohistiocytosis from other causes of
98 ive cytopenias, hyperferritinemia, and other hemophagocytic lymphohistiocytosis hallmarks were appare
99 about half of patients with severe forms of hemophagocytic lymphohistiocytosis have been developed.
100 cells and developed all clinical symptoms of hemophagocytic lymphohistiocytosis (HLH) after infection
101 ive T-cell activation and the fatal disorder hemophagocytic lymphohistiocytosis (HLH) after infection
102 ed to be associated with a high incidence of hemophagocytic lymphohistiocytosis (HLH) and a lack of l
106 he hyperinflammatory state characteristic of hemophagocytic lymphohistiocytosis (HLH) and macrophage
107 forms of the severe immunoregulatory disease hemophagocytic lymphohistiocytosis (HLH) arise from bial
108 poietic stem cell transplantation (HSCT) for hemophagocytic lymphohistiocytosis (HLH) at the cost of
109 e 2019 (COVID-19) has parallels to secondary hemophagocytic lymphohistiocytosis (HLH) both clinically
111 e small ssRNAs and develop spontaneous fatal hemophagocytic lymphohistiocytosis (HLH) characterized b
114 eneic hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis (HLH) disorders is as
115 tients with primary and infection-associated hemophagocytic lymphohistiocytosis (HLH) from patients w
155 imary, genetic forms from secondary forms of hemophagocytic lymphohistiocytosis (HLH) is crucial for
156 receptor (CAR) T-cell toxicities resembling hemophagocytic lymphohistiocytosis (HLH) occur in a subs
158 ate-onset inflammatory toxicities resembling hemophagocytic lymphohistiocytosis (HLH) or macrophage a
159 c herpes simplex virus (HSV)-1 infection and hemophagocytic lymphohistiocytosis (HLH) postpartum, and
160 iable clinical consequences, including fatal hemophagocytic lymphohistiocytosis (HLH) triggered predo
161 ns can improve the outcomes of patients with hemophagocytic lymphohistiocytosis (HLH) undergoing allo
163 of pediatric and adult patients with primary hemophagocytic lymphohistiocytosis (HLH) with refractory
165 ad set of triggering signals can precipitate hemophagocytic lymphohistiocytosis (HLH), a life-threate
166 evere COVID-19, with parallels proposed with hemophagocytic lymphohistiocytosis (HLH), a life-threati
168 erferritinemia and cytopenias are present in hemophagocytic lymphohistiocytosis (HLH), and somatic mu
169 thologically, MAS bears strong similarity to hemophagocytic lymphohistiocytosis (HLH), and some autho
170 2) inhibitor ruxolitinib in murine models of hemophagocytic lymphohistiocytosis (HLH), and the HLH-si
171 Cytokine storm syndromes (CSSs), including hemophagocytic lymphohistiocytosis (HLH), are increasing
172 t spontaneously develop clinical features of hemophagocytic lymphohistiocytosis (HLH), but do so when
173 ne have been described in some patients with hemophagocytic lymphohistiocytosis (HLH), but the role o
174 led to the life-threatening immune disorder hemophagocytic lymphohistiocytosis (HLH), characterized
175 that can present with recurrent episodes of hemophagocytic lymphohistiocytosis (HLH), though the exa
177 oss of normal immune regulation and underlie hemophagocytic lymphohistiocytosis (HLH), which requires
178 Here, we characterize 2 novel monoallelic hemophagocytic lymphohistiocytosis (HLH)-associated muta
179 mbined clinical and functional data from our hemophagocytic lymphohistiocytosis (HLH)-network registr
191 of genetic factors to the severity of adult hemophagocytic lymphohistiocytosis (HLHa) remains unclea
192 is often valuable in patients with acquired hemophagocytic lymphohistiocytosis (i.e., secondary hemo
193 n in low baseline severity of illness versus hemophagocytic lymphohistiocytosis in higher baseline se
195 etermine optimal treatment for patients with hemophagocytic lymphohistiocytosis in ICUs, including th
198 investigation of the underlying triggers of hemophagocytic lymphohistiocytosis, including infections
199 irms the occurrence of reversible AKI due to hemophagocytic lymphohistiocytosis-induced activated mac
200 r (ICI) therapy, including severe cases like hemophagocytic lymphohistiocytosis (irHLH), and to disti
211 c hyperinflammation, fulfilling criteria for hemophagocytic lymphohistiocytosis, is an emerging pheno
212 virally triggered inflammatory episodes with hemophagocytic lymphohistiocytosis-like disease, early-o
214 onse such as immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome may occ
215 One grade 2 immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome was obs
216 re and often fatal infectious mononucleosis, hemophagocytic lymphohistiocytosis, lymphoproliferative
217 ncluding fulminant infectious mononucleosis, hemophagocytic lymphohistiocytosis, lymphoproliferative
218 mental status and neurologic dysfunction, or hemophagocytic lymphohistiocytosis-macrophage-activation
219 ng examples from several diseases, including hemophagocytic lymphohistiocytosis/macrophage activation
221 iggers include malignant neoplasm-associated hemophagocytic lymphohistiocytosis (MN-HLH), cytokine re
222 e (n = 9), central nervous system-restricted hemophagocytic lymphohistiocytosis (n = 3), anti-GABA(A)
224 , renal, hepatic, and neurologic toxicities, hemophagocytic lymphohistiocytosis, opportunistic infect
225 by inborn errors of immunity (i.e., familial hemophagocytic lymphohistiocytosis) or develop as a comp
226 genetic disorders such as leukodystrophies, hemophagocytic lymphohistiocytosis, or genetic vasculopa
227 t cytopenia, autoinflammation, and recurrent hemophagocytic lymphohistiocytosis, p.C188Y and p.*192Ce
228 IFN-gamma is largely considered a driver of hemophagocytic lymphohistiocytosis pathology, IFN-gamma
229 igating treatment tailored to critically ill hemophagocytic lymphohistiocytosis patients are highly w
230 Maximum ferritin levels were highest in hemophagocytic lymphohistiocytosis patients compared wit
234 sttransplant plasma cell hepatitis, familial hemophagocytic lymphohistiocytosis, pediatric nonalcohol
240 neurotoxicity, cytokine release syndrome and hemophagocytic lymphohistiocytosis, represented only a m
241 set pancytopenia and severe autoinflammation/hemophagocytic lymphohistiocytosis requiring emapalumab
242 y, including macrophage activation syndrome (hemophagocytic lymphohistiocytosis secondary to autoimmu
249 ed lymphomas, lymphoproliferative disorders, hemophagocytic lymphohistiocytosis, solid tumors, and ot
250 n between macrophage activation syndrome and hemophagocytic lymphohistiocytosis still remain to be de
251 sociated with macrophage activation syndrome/hemophagocytic lymphohistiocytosis, such as interleukin
252 ny clinical and laboratory manifestations of hemophagocytic lymphohistiocytosis syndrome (HLS), also
253 anatory mechanisms, possibly integrated into hemophagocytic lymphohistiocytosis syndrome, of infectio
255 ead to a spectrum of diseases, from familial hemophagocytic lymphohistiocytosis to an increased risk
258 Munc13-4, the mutation of which in familial hemophagocytic lymphohistiocytosis type 3 results in a p
262 n-knockout mouse strains and from a Familial Hemophagocytic Lymphohistiocytosis type 4 (FHL4) patient
263 ients with STX11 deficiency develop familial hemophagocytic lymphohistiocytosis type 4 (FHL4), a life
265 Recent analysis of patients with familial hemophagocytic lymphohistiocytosis type 5 has identified
266 atal immune dysregulation disorder, familial hemophagocytic lymphohistiocytosis (type 2 FHL, FHL2).
267 Similarly, treating children with familial hemophagocytic lymphohistiocytosis using reduced intensi
268 he perforin gene have been found in familial hemophagocytic lymphohistiocytosis, which shares some fe
270 gocytic lymphohistiocytosis (i.e., secondary hemophagocytic lymphohistiocytosis) without previous the