scholarly journals Clinical Features and Prognostic Factors of Early Outcome in Pediatric Hemophagocytic Lymphohistiocytosis

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Yong-Hai Zhou ◽  
Xin-Ru Han ◽  
Fang-Qing Xia ◽  
Neha-Devi Poonit ◽  
Li Liu
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Hua Pan ◽  
Yongmin Huo ◽  
Lirong Sun

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.


Author(s):  
Deepak Kumar ◽  
Suresh Bishnoi ◽  
Durga Shankar Meena ◽  
Gopal Krishana Bohra ◽  
Naresh Midha ◽  
...  

Background: Cryptococcal meningitis is a dreaded complication in HIV infected patients. It was associated with high mortality and morbidity before the advent of Highly Active Anti-Retroviral Therapy. A study was planned in our institute to evaluate the clinical profile, laboratory findings, and prognostic factors for survival in these patients. Settings and Design: A prospective hospital-based observational study, conducted in the Department of Medicine at a tertiary care centre in western Rajasthan. Method and Material: HIV infected patients presenting with clinical features of meningitis, and positive CSF culture for Cryptococcus were included in our study. All cases underwent detailed clinical history, physical examinations and relevant laboratory investigations including CD4 count and CSF examination. Results: 48 HIV infected cryptococcal meningitis patients were analysed, and the most common presenting clinical features were headache (85.42%), and fever (72.92%), followed by neck stiffness (62.50%). CSF examination was also done for Cryptococcal Antigen test which was reactive in all cases (100%), and India ink staining was positive in 43 cases (89.58%). Significant fatal outcome was associated with patients presenting with altered sensorium, loss of consciousness, cranial nerve palsy and CD4 cell count of less than 100. Similarly, on laboratory and imaging diagnosis, cryptococcal antigen test (>3+reactive), fundus examination (papilloedema) and abnormal CT/MRI brain imaging were associated with poor survival. Conclusion: Cryptococcal meningitis is a potentially lethal infection in immunocompromised individuals and should be diagnosed early with high clinical suspicion as around 10% of the cases may not be detected on India ink staining and a large proportion (75%) of cases may have normal imaging at initial evaluation. Early diagnosis, watchful eye on prognostic factors and treatment is vital to improve outcome in these patients.


Open Medicine ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. 169-174
Author(s):  
Lei Lei ◽  
Liu Yang ◽  
Yang-yang Xu ◽  
Hua-fei Chen ◽  
Ping Zhan ◽  
...  

Abstract Hepatoid adenocarcinoma of the lung (HAL) is a rare malignant tumor that is defined as a primary alpha-fetoprotein (AFP)-producing lung carcinoma. We aimed to identify prognostic factors associated with the survival of patients with HAL using data from the Surveillance, Epidemiology, and End Results (SEER) database. We collected data from patients diagnosed with HAL, adenocarcinoma (ADC), and squamous cell carcinoma (SCC) of the lung between 1975 and 2016 from the SEER database. The clinical features of patients with ADC and SCC of the lung were also analyzed. The clinical features of HALs were compared to ADCs and SCCs. A chi-square test was used to calculate the correlations between categorical variables, and a t test or Mann–Whitney U test was used for continuous variables. The Kaplan–Meier method and Cox regression analysis were used to identify the prognostic factors for the overall survival (OS) of HALs. Two-tailed p values < 0.05 were considered statistically significant. Sixty-five patients with HAL, 2,84,379 patients with ADC, and 1,86,494 with SCC were identified from the SEER database. Fewer males, advanced stages, and more chemotherapy-treated HALs were found. Compared to patients with SCC, patients with HAL were less likely to be male, more likely to be in an advanced stage, and more likely to receive chemotherapy (p < 0.05). The American Joint Committee on Cancer staging was the only prognostic factor for OS in patients with HAL, and stage IV was significantly different from other stages (hazard ratio = 0.045, 95% confidence interval: 0.005–0.398, p = 0.005). Males with HAL were more likely to receive radiotherapy compared to females with HAL (61.8 vs 31.5%, p = 0.034). Younger patients with HAL were more likely to receive chemotherapy (59.4 + 10.2 years vs 69 + 11.3 years, p = 0.001). The primary tumor size of HAL was associated with the location of the primary lesion (p = 0.012). No conventional antitumor therapies, including surgery, chemotherapy, and radiotherapy, were shown to have a significant survival benefit in patients with HAL (p > 0.05). This study showed that stage IV was the only prognostic factor for OS in HALs compared to other clinicopathologic factors. Conventional antitumor therapies failed to show survival benefit; thus, a more effective method by which to treat HAL is needed. Interestingly, the clinical features and the location of the primary lesion were shown to be associated with primary tumor size and treatment in patients with HAL, which have not been reported before.


2021 ◽  
Vol 42 (5) ◽  
pp. 103027
Author(s):  
Yi Wang ◽  
Tongli Ren ◽  
Jianghua Jing ◽  
Na Gao ◽  
Hui Zhao ◽  
...  

2016 ◽  
Vol 263 (5) ◽  
pp. 981-990 ◽  
Author(s):  
Cécile-Audrey Durel ◽  
Romain Marignier ◽  
Delphine Maucort-Boulch ◽  
Jean Iwaz ◽  
Emilie Berthoux ◽  
...  

2009 ◽  
Vol 36 (5) ◽  
pp. 1026-1031 ◽  
Author(s):  
TING ZENG ◽  
YU-QIONG ZOU ◽  
MEI-FANG WU ◽  
CHENG-DE YANG

Objective.To describe the onset, clinical features, prognostic factors, and treatment of adult-onset Still’s disease (AOSD) in cases from China.Methods.Sixty-one Chinese patients with AOSD were analyzed retrospectively.Results.Common clinical features were fever (100.0%), rash (88.5%), and arthritis (82.0%). The laboratory findings were as follows: leukocytosis (83.6%), increased erythrocyte sedimentation rate (100.0%), elevated transaminase concentrations (23.0%), elevated ferritin levels (79.6%), negative antinuclear antibody (88.5%), and negative rheumatoid factor (88.5%). Of the 61 patients, 44.3% exhibited a monocyclic disease pattern, 29.5% experienced disease relapse at least once, 16.4% exhibited chronic articular course, and 9.8% died; most deaths were due to pulmonary infection and respiratory failure. Based on the disease course, we divided the 61 patients into 2 groups: those with favorable outcome (cyclic disease course, n = 45) and unfavorable outcome (chronic disease course or death, n = 16). We analyzed the prognostic factors for the 2 groups, and found that pleuritis, interstitial pneumonia, elevated ferritin levels, and failure of fever to subside after 3 days of prednisolone at 1 mg/kg/day were unfavorable prognostic factors for patients with AOSD.Conclusion.Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone.


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