Prognostic factors of early death in children with hemophagocytic lymphohistiocytosis

Abstract BACKGROUND Stereotactic radiosurgery (SRS) is used commonly for patients with brain metastases (BM) to improve intracranial disease control. However, survival of these patients is often dictated by their systemic disease course. The value of SRS becomes less clear in patients with anticipated short survival. OBJECTIVE To evaluate prognostic factors, which may predict early death (within 90 d) after SRS. METHODS A total of 1427 patients with BM were treated with SRS at our institution (2000-2012). There were 1385 cases included in this study; 1057 patients underwent upfront SRS and 328 underwent salvage SRS. The primary endpoint of the study was all-cause mortality within 90 d after first SRS. Multivariate analyses were performed to develop prognostic indices. RESULTS Two hundred sixty-six patients (19%, 95% confidence interval 17%-21%) died within 90 d after SRS. Multivariate analysis of upfront SRS patients showed that Karnofsky Performance Status, primary tumor type, extracranial metastases, age at SRS, boost treatment, total tumor volume, prior surgery, and interval from primary to BM were independent prognostic factors for 90-d mortality. The first 4 factors were also independent predictors in patients treated with salvage SRS. Based on these factors, an index was defined for each group that categorized patients into 3 and 2 prognostic groups, respectively. Ninety-day mortality was 5% to 7% in the most favorable cohort and 36% to 39% in the least favorable. CONCLUSION Indices based on readily available patient, clinical, and treatment factors that are highly predictive of early death in patients treated with upfront or salvage SRS can be calculated and used to define well-separated prognostic groups.

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The Outcome of 129 Korean Children with Hemophagocytic Lymphohistiocytosis.

Blood ◽

10.1182/blood.v108.11.3847.3847 ◽

2006 ◽

Vol 108 (11) ◽

pp. 3847-3847

Author(s):

Hoi Soo Youn ◽

Joon Sup Song ◽

Ho Joon Im ◽

Jong Jin Seo ◽

Hyung Nam Moon ◽

...

Keyword(s):

Prognostic Factors ◽

Diagnostic Criteria ◽

Hemophagocytic Lymphohistiocytosis ◽

Initial Treatment ◽

Disease State ◽

Cns Involvement ◽

Hematopoietic Stem ◽

Korean Children ◽

Female Ratio ◽

Excellent Outcome

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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Difference in causes and prognostic factors of early death between cohorts with de novo and relapsed acute promyelocytic leukemia

Annals of Hematology ◽

10.1007/s00277-017-3216-2 ◽

2017 ◽

Vol 97 (3) ◽

pp. 409-416 ◽

Cited By ~ 3

Author(s):

Hongli Zhao ◽

Yanqiu Zhao ◽

Yingmei Zhang ◽

Jinxiao Hou ◽

Huiyuan Yang ◽

...

Keyword(s):

Prognostic Factors ◽

Acute Promyelocytic Leukemia ◽

De Novo ◽

Early Death ◽

Promyelocytic Leukemia

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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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Familial hemophagocytic lymphohistiocytosis: an entity leading to early death

Annales de biologie clinique ◽

10.1684/abc.2021.1669 ◽

2021 ◽

Vol 79 (5) ◽

pp. 470-471

Author(s):

Linda Khefacha ◽

Nouha Berrayana ◽

Hatem Rouag ◽

Mouna Sassi

Keyword(s):

Hemophagocytic Lymphohistiocytosis ◽

Early Death ◽

Familial Hemophagocytic Lymphohistiocytosis

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