Haematological Malignancies

Background
Anemia is a common blood condition that affects populations in both rich and poor countries.  The most prevalent anemia after iron deficiency anemia is the anemia of chronic disease. It is usually observed as a non-specific anemia in patients with infectious, inflammatory and neoplastic diseases, and may cause diagnostic challenges. Routine diagnostic tests are performed in these patients, but recently, new blood markers such as hepcidin antimicrobial peptide are used to evaluate the severity of the disease. 

Objective
To evaluate the association between inflammatory disease and the potential risk for anemia in pediatric age-groups.

Methods and materials
In this study were involved 64 children aged 0 to 18 years old, hospitalized in ‘’Pediatric Service’’, the University Medical Center of Tirana “Mother Teresa”, during May 2018. Patients were diagnosed with diseases associated with the inflammatory process. Complete blood count (CBC) tests and other tests that detect inflammation such as C-Reactive Protein and Erythrocyte Sedimentation Rate (CRP and ESR), were determined in patients with hemoglobin (Hb)  levels < 11.5 g/dl. The CBC parameters are measured using Automated Cell Counter machines, ESR is measured using Westergren method, CRP test is based on the principle of the latex agglutination, and the colorimetric method is used for serum iron measurement. All the cases involved in this study were evaluated other examinations and laboratory tests, which were significant in the determination of the diagnosis.

Results
The average age of patients included in the study was 2.65 years old among which 56.25%  were male and 44.75% were female. 12.5% of patients had blood malignancies, 34.4% had acute bacterial diseases, 18.7% had chronic bacterial diseases, 23.4% had viral infections and 10.9% had autoimmune diseases. Patients with chronic bacterial diseases and viral infections had significantly higher levels of ESR (ESR=29.83, N=0-13mm/h) than others. Levels of CRP were higher in patients with acute bacterial diseases (CRP=52.84, N=3-20mg/dl), and the levels of hemoglobin were lower in patients with autoimmune diseases (Hb=9.14, N=13-16 ±2 mg/dl). This was presented with a high variance in the values of ESR (σ^2 = 286.09) and  CRP ( σ^2= 2002.28). The evaluations revealed a moderate positive correlation between Hb and RBC (R^2=0.15), a mild negative correlation between Hb and ESR, also between Hb and CRP.



Conclusion
The time span and the degree of the inflammation which coexists with the underlying disease, determine the type of anemia which associates them. These results suggest that in this pediatric age-group, the treatment should focus on the underlying disease in order to correct anemia.

Keywords
anemia, inflammation, pediatric age-group, hemoglobin, CRP, ESR, iron metabolism.

Background: Anthracycline-treated childhood cancer survivors are at higher risk of cardiotoxicity, especially with cumulative doses received above 250 mg/m 2. Dexrazoxane is the only option recommended for cardiotoxicity prevention in high-risk patients supported by randomised trials but its cost-effectiveness in paediatric cancer patients has not been established. Methods: A cost-effectiveness model applicable to different national healthcare system perspectives, which simulates 10,000 patients with either sarcoma or haematologic malignancies, based upon baseline characteristics including gender, age at diagnosis, cumulative anthracycline dose and exposure to chest irradiation. Risk equations for developing congestive heart failure and death from recurrence of the original cancer, secondary malignant neoplasms, cardiac death, pulmonary death, and death from other causes were derived from published literature. These are applied to the individual simulated patients and time until development of these events was determined. The treatment effect of dexrazoxane on the risk of CHF or death was based upon a meta-analysis of randomised and non-randomised dexrazoxane studies in each tumour type. The model includes country specific data for drug and administration costs, all aspects of heart failure diagnosis and management, and death due to different causes for each of the five countries considered; France, Germany, the UK, Italy, and Spain. Results: Dexrazoxane treatment resulted in a mean QALY benefit across the five countries ranging from 0.530 to 0.683 per dexrazoxane-treated patient. Dexrazoxane was cost-effective for paediatric patients receiving anthracycline treatment for sarcoma and for haematologic malignancies, irrespective of the cumulative anthracycline dose received. The Incremental Cost Effectiveness Ratio (ICER) was favourable in all countries irrespective of anthracycline dose for both sarcoma and haematological malignancies (range: dominant to €2196). Individual ICER varied considerably according to country with dominance demonstrated for dexrazoxane in Spain and Italy and ratios approximately double the European average in the UK and Germany.

Loss-of-function mutations of cyclic-AMP response element binding protein, binding protein (CREBBP) are prevalent in lymphoid malignancies. However, the tumour suppressor functions of CREBBP remain unclear. We demonstrate that loss of Crebbp in murine haematopoietic stem and progenitor cells (HSPCs) leads to increased development of B-cell lymphomas. This is preceded by accumulation of hyperproliferative lymphoid progenitors with a defective DNA damage response (DDR) due to a failure to acetylate p53. We identify a premalignant lymphoma stem cell population with decreased H3K27ac, which undergoes transcriptional and genetic evolution due to the altered DDR, resulting in lymphomagenesis. Importantly, when Crebbp is lost later in lymphopoiesis, cellular abnormalities are lost and tumour generation is attenuated. We also document that CREBBP mutations may occur in HSPCs from patients with CREBBP-mutated lymphoma. These data suggest that earlier loss of Crebbp is advantageous for lympho...

Objective:  The purpose of the present study was to compare the
extent of variation in haematologic parameter associated with the
long term use of hormonal and non hormonal contraceptives in urban
population of low socio-economic group.  Type of Study:  Cross
sectional study.  Setting:  Clinical setting.  Patients and Methods:
Fifty-four young females of age ranging from 26-32 years maintained
on Oral contraceptives (OC), Depo-medroxy progesterone (DMPA),
Norethisterone (NET-EN), Implant and non hormonal intrauterine
contraceptive device (IUCD)  for at least one year were invited.
Fasting blood  samples were collected for the analysis of
haematologic parameters. All the results were entered in MS-Excel
and mean ± standard deviation was calculated for each frequency.
Results:  Comparision among different groups was done by
calculating percentile differences among groups. Though the result of
the study does not showed the significant differences in various
indices but the hemoglobin levels in IUCD group was found to be
significantly low as compared to hormonal contraceptives. Use of
hormonal contraceptives was associated with better haematologic
profile whereas IUCDs were found to pose risk of anemia in already
anemic population of low  socio economic group.  Conclusion:
Though the risk in this group of young females maintained on
contraceptives was found to be minimized because of good
monitoring but continuation of IUCDs might cause this slight
predisposition of sub clinical anemia into  a well defined anemic
disease later in their lives. It is therefore recommended to use
intrauterine contraceptive devices with caution in anemic women and
such methods should be used under close monitoring.

Objectives: To measure the psychological effect of an art intervention on hospitalized patients and explore benefits to their quality of life. Methods: We conducted a large prospective randomized trial between July 2006 and August 2009 of an art intervention, Open Window (OW), in patients undergoing stem cell transplantation for a hematological malignancy compared with a control group. The primary endpoint measured the effect of an art intervention on levels of anxiety, depression, and stress using the Hospital Anxiety and Depression Scale and the Distress Thermometer. The secondary endpoint measured the influence of OW on patients' experiences of stem cell transplantation using the OW survey and expectations questionnaires. Results: Of the 199 patients in the study, 96 were randomized to the intervention group and 103 to the control group. Participants in the intervention group had significantly reduced levels of anxiety on the day before transplant (p = 0.001), at day 7 (p = 0.041), and day 60 (p = 0.035). There was a significant reduction in depression before transplant (p = 0.022). Participants in the intervention group reported better experiences (p < 0.005). Qualitative data showed that those in the intervention group commented freely on their likes and dislikes about OW and how it made them feel. Conclusion: An art intervention, OW, had a positive influence on health-related quality of life and patients' experiences of having a stem cell transplant.