What’s in a name? The heterogeneous clinical spectrum and prognostic factors in a cohort of adults with hemophagocytic lymphohistiocytosis

Abstract Chemotherapy and immunotherapy based treatments improved survival of patients with hemophagocytic lymphohistiocytosis(HLH), but the outcome is still unsatisfactory. We analyzed the putative prognostic factors in a nationwide cohort of patients with HLH. Retrospective data recruitment for the patients diagnosed as HLH during the past 10-year period from 1996 to 2005 was carried out by the Histiocytosis Working Party of the Korean Society of Hematology. The HLH diagnostic criteria of the Histiocyte Society were strictly applied to confirm the eligibility of patients for this study. We analyzed the outcome of pediatric patients with HLH according to the age at diagnosis, sex, central nervous system(CNS) involvement, disease condition(familial or secondary), treatment modalities and disease state after 2 months of initial treatment. One hundred twenty nine patients from 19 centers fulfilled the diagnostic criteria(n=112) and/or had affected siblings together with some of the criteria(n=17). The male to female ratio was 0.95:1. The probability of 3 year overall survival(OS) in HLH patients was 41% with a median follow-up of 51 months. The 3 year OS in patients under 12 months of age at presentation(n=23) was 21.7%, and 44.3% in those over 12 months of age(n=106)(p=0.001). The 3 year OS in patients with CNS involvement(n=16) was 29.1%, and 44.4% in patients without CNS involvement(n=112)(p=0.01). The 3 year OS in patients with active state after 2 months of initial treatment(n=63) was 14.1% compared to 77.2% in those with inactive state(n=61)(p=0.0001). The 3 year OS in patients who received hematopoietic stem cell transplantation(HSCT)(n=17) was 82.3%, and 35.2% in patients treated with chemoimmunotherapy only(n=112)(p=0.03). Among the HSCT patients, complete remission was obtained in 14 patients except 3 other patients who died of infection and graft failure at early post-transplant period. The reasons for HSCT were active disease after chemoimmunotherapy(n=8), relapsed disease(n=5), and familial HLH(n=4). Other prognostic factors were not significantly correlated with outcome in our survey. The age and CNS involvement at diagnosis, disease state after 2 months of initial treatment were important prognostic factors which affected the outcome of HLH significantly in this cohort. This survey also demonstrated excellent outcome of familial or relapsed, persistent secondary HLH after HSCT compared to chemoimmunotherapy only.

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Infective endocarditis in the intensive care unit: clinical spectrum and prognostic factors in 228 consecutive patients

Intensive Care Medicine ◽

10.1007/s00134-004-2436-9 ◽

2004 ◽

Vol 30 (11) ◽

pp. 2046-2052 ◽

Cited By ~ 88

Author(s):

Bruno Mourvillier ◽

Jean-Louis Trouillet ◽

Jean-François Timsit ◽

Jérome Baudot ◽

Jean Chastre ◽

...

Keyword(s):

Intensive Care Unit ◽

Intensive Care ◽

Prognostic Factors ◽

Infective Endocarditis ◽

Clinical Spectrum

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Vascular graft infections in the intensive care unit: Clinical spectrum and prognostic factors

Journal of Infection ◽

10.1016/j.jinf.2010.12.006 ◽

2011 ◽

Vol 62 (3) ◽

pp. 204-211 ◽

Cited By ~ 11

Author(s):

Magdalena Szczot ◽

Agnès Meybeck ◽

Laurence Legout ◽

Armelle Pasquet ◽

Nicolas Van Grunderbeeck ◽

...

Keyword(s):

Intensive Care Unit ◽

Intensive Care ◽

Prognostic Factors ◽

Vascular Graft ◽

Clinical Spectrum

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Comparison between clinical features and prognosis of malignancy- and non-malignancy–associated pediatric hemophagocytic lymphohistiocytosis

BMC Pediatrics ◽

10.1186/s12887-019-1702-5 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 1

Author(s):

Hua Pan ◽

Yongmin Huo ◽

Lirong Sun

Keyword(s):

Overall Survival ◽

Prognostic Factors ◽

Survival Time ◽

Clinical Features ◽

Hemophagocytic Lymphohistiocytosis ◽

Clinical Characteristics ◽

Univariate Analysis ◽

Control Group ◽

Survival Times ◽

Overall Survival Time

Abstract Background The differences between the clinical characteristics and survival time in malignancy- and non-malignancy–associated secondary hemophagocytic lymphohistiocytosis (HLH) are unclear. Here, we describe the clinical characteristics, prognostic factors, and survival outcomes of malignancy-associated HLH compared to that of non-malignancy–associated HLH. Methods We retrospectively analyzed 91 pediatric patients with HLH (age < 14 years) at the Affiliated Hospital of Qingdao University Pediatric Department between January 2005 and October 2016. The patients were divided into the malignancy-associated group (n = 22) and non-malignancy–associated group (n = 69, also considered the control group). The clinical features were compared using the Mann–Whitney U and χ2 tests. The overall survival time was compared using log rank and Mann–Whitney U tests. Results Hemoglobin (HGB; p = 0.004), alanine aminotransferase (ALT; p = 0.002), and aspartate aminotransferase (AST; p = 0.001) levels in the malignancy-associated group differed from that in the non-malignancy–associated group. The mean survival times were 26.9 ± 3.82 months (malignancy-associated HLH) and 35.03 ± 2.19 months (non-malignancy–associated HLH). The overall survival time between the two groups was not statistically significantly different (p = 0.055). Univariate analysis showed that disseminated intravascular coagulation (DIC) score > 5 (p = 0.001), albumin < 25 g/L (p = 0.000), HGB < 60 g/L (p = 0.001), and platelet count (PLT) < 30 × 109/L (p = 0.042) correlated with prognosis. Multivariate Cox analysis showed that albumin < 25 g/L (p = 0.017), HGB < 60 g/L (p = 0.027), and bone marrow hemophagocytosis (p = 0.034) correlated with worse prognosis. Conclusions Patients with non-malignancy–associated HLH do not have better survival, although their prognosis is relatively better in clinical practice. A higher DIC score at diagnosis and lower albumin, HGB, and PLT levels are negative prognostic factors in malignancy-associated HLH.

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Clinical Features and Prognostic Factors of Acute Kidney Injury Caused by Adult Secondary Hemophagocytic Lymphohistiocytosis

SSRN Electronic Journal ◽

10.2139/ssrn.3824672 ◽

2021 ◽

Author(s):

Siwen Wang ◽

Jiaojiao Zhou ◽

Jia Yang ◽

Ling Ji ◽

Xin Wang ◽

...

Keyword(s):

Acute Kidney Injury ◽

Prognostic Factors ◽

Clinical Features ◽

Hemophagocytic Lymphohistiocytosis ◽

Kidney Injury ◽

Secondary Hemophagocytic Lymphohistiocytosis

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Experience with Hemophagocytic Lymphohistiocytosis at a Single Institution.

Blood ◽

10.1182/blood.v114.22.4517.4517 ◽

2009 ◽

Vol 114 (22) ◽

pp. 4517-4517

Author(s):

Meerim Park ◽

Kyung Nam Koh ◽

Keun Wook Bae ◽

Mee Jeong Lee ◽

Ho Joon Im ◽

...

Keyword(s):

Treatment Outcome ◽

Hemophagocytic Lymphohistiocytosis ◽

Conflicts Of Interest ◽

Medical Center ◽

Collaborative Study ◽

Delayed Diagnosis ◽

Clinical Spectrum ◽

Unrelated Donor ◽

Korean Children ◽

Familial Form

Abstract Abstract 4517 Background Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS), whether primary or secondary, is a serious and potentially life- threatening histiocytic disorder and generally fatal without immune modulating chemotherapy. This study aimed to define the clinical spectrum and treatment outcome in Korean children. Patients and Method Patients who were diagnosed with HLH/MAS at Asan Medical Center between October 1997 and April 2009 were identified and charts were reviewed. Results Twenty-six patients (16 male, 10 female) were evaluated during 13 - year period. Median age at diagnosis was 29.5 months (range: 0 - 180 months). Twelve patients had HLH secondary to underlying disorders, including viral infection, autoimmune disease. Epstein-Barr virus (EBV) - associated HLH was present in 9 patients, 1 of whom died, while 4 of 17 non-EBV associated patients died. Initial treatment with intravenous immunoglobulin (IVIG) was given to 9 patients and they had no response to IVIG. Eventually, all the patients except two underwent full protocol therapy (HLH 1994/2004) and the 2 patients died of rapid disease progression with delayed diagnosis. Seven patients revealed disease reactivation, median 3 months from HLH protocol therapy, and further treatment using the same regimen induced second remission of short duration. Seven patients (26.9%) had evidence of central nervous system disease at the time of diagnosis and neurological symptoms consisted of seizure, irritability, vomiting. Five of them are long-term survivors including one who received allogeneic stem cell transplantation (SCT) and all 5 patients show normal neurological function after treatment. The familial form of HLH was identified in 3 patients and all received allogeneic SCT from unrelated donor in second remission. They are still alive without relapse. At a median follow-up of 17.5 months, the estimated one-year overall survival rate was 82.8 ± 7.9 %. Conclusion Treatment outcomes using HLH 1994/2004 protocols in Korean children have become comparable to those reported in an international collaborative study. And allogeneic SCT represents a curative treatment for familial form of HLH. Further studies are required to more precisely define clinical spectrum and treatment outcome. Disclosures: No relevant conflicts of interest to declare.

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Prognostic Factors and Long-Term Outcome in 52 Turkish Children With Hemophagocytic Lymphohistiocytosis*

Pediatric Critical Care Medicine ◽

10.1097/pcc.0000000000000449 ◽

2015 ◽

Vol 16 (6) ◽

pp. e165-e173 ◽

Cited By ~ 13

Author(s):

Zühre Kaya ◽

Ali Bay ◽

Meryem Albayrak ◽

Ulker Kocak ◽

Idil Yenicesu ◽

...

Keyword(s):

Prognostic Factors ◽

Hemophagocytic Lymphohistiocytosis ◽

Term Outcome ◽

Turkish Children ◽

Long Term Outcome

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Spectrum of acute kidney injury in intensive care unit: a single centre experience

Indian Journal of Medical Sciences ◽

10.18203/issn.0019-5359.indianjmedsci20170498 ◽

2017 ◽

Vol 69 (1) ◽

pp. 44

Author(s):

Suresh Kumar Sinha ◽

Mukteshwar Rajak ◽

Prabhakar . ◽

Rajneesh . ◽

Vivek Tripathi

Keyword(s):

Intensive Care Unit ◽

Acute Kidney Injury ◽

Intensive Care ◽

Prognostic Factors ◽

Kidney Injury ◽

Final Outcome ◽

Clinical Spectrum ◽

Icu Stay ◽

Organ System Failure ◽

Apache Iv

Background: Acute kidney injury (AKI) in the intensive care unit (ICU) is associated with high mortality. A thorough understanding of the clinical spectrum of the disease is needed in order to device methods to improve the final outcome due to this problem. Aims and Objectives: The aim of present study was to analyze the clinical spectrum, causes, risk and prognostic factors and final outcome of AKI in the setting of ICU. Materials and Methods: This prospective study involved patients admitted to ICU during the period between June 09 to June 10. Patients who developed AKI during the ICU stay were included in the study. The clinical and laboratory data were collected at admission and then on daily basis. Data recorded includes patients demographic profile, underlying clinical illness responsible for ICU admission, dialysis requirement, need for ventilation, total duration of ICU stay, acute physiology and chronic health evaluation (APACHE)-IV score and final outcome and these data were analyzed for predicting survival using univariate and multivariate analysis. Results: 574 patients were admitted to ICU from June 09 to June 10 and (n = 124; 21.6%) patients developed AKI after admission to ICU. Mean age 44.87 ± 15.14 years and (n = 71; 57.1%) were males and (n = 53; 42.9%) were females. Out of 124 patients (50.80%; n = 63) had medical, (33.87% n = 42) had surgical and (15.32%; n = 19) had obstetric cause of admission in ICU. Of the 574 patients (12.02%; n = 69) had associated co morbidities, hypertension is the most common associated morbidities (4.7%; n = 27), others were diabetes mellitus (3.6%; n = 21), coronary artery disease (3.0%; n = 17), cerebrovascular disease (0.3%; n = 2), chronic obstructive pulmonary disease (0.3%; n = 2;). The etiology of AKI was multi-factorial, sepsis were the most common cause observed in (69.64%; n = 39), hypotension (67.84%; n = 38), volume depletion (19.64%; n = 11), nephrotoxic drugs (64.28%; n = 36) patients. Multi organ system failure (MOSF) was noted in (29.03%; n = 36) patients. MOSF and sepsis were found to be significant adverse prognostic factors when multiple logistic regression analysis was done. Conclusion: AKI was seen in 21.6% of cases in our ICU and associated with poor prognosis. Presence of sepsis, MOSF, higher APACHE IV scores and ventilation requirement were correlated with higher mortality in AKI patients in ICU. Early recognisition and intervention improves the outcome.

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