14 research outputs found
Chronic immune thrombocytopenic purpura in children overview of 60 patients
Background: A small percentage of children with Immune thrombocytopenic purpura (ITP) suffer from a clinically significant disease with severe thrombocytopenia that requires intervention. Treatment for these children presents a challenge as there are few known therapies that offer long-term remission, and all that are known have significant side effects and toxicities.
Aim of the study: To evaluate the effects of a variety of treatment modalities on the clinical course, and long treatment outcomes in children with chronic ITP.
Patients & methods: A study involved 60 children with chronic ITP who were referred to Hemato-Oncology unit/Children's Welfare Teaching Hospital/Medical City/Baghdad. Treatment of patients included steroid, Intravenous Immunoglobulins, Anti D immunoglobulin, 6-Mercaptopurine, Rituximab and splenectomy. The Period of data collection and analysis was from May 2009 to May 2011.
Results: The most common presenting symptom was skin bleeding, seen in 42 (70%) patients. Thirty-four patients received one or more courses of steroids. Complete response was achieved in 7 (20.5%) patients while there was no response in 12 (35.2%) patients, Intravenous immunoglobulin was used for 5 patients, only one (16%) exhibited a good response. Anti D Immunoglobulin was used in six patients; only one (8.3%) patient got good response. Twelve patients received 6-mercaptopurine, only one (8.3%) patient had a partial response. Six patients received Rituximab; three (50%) had a partial response. Six patients underwent splenectomy; response was noted in 5/6 (83.3%) patients. At the end of the study; complete response was seen in 13 (22.4%) patients, partial in 19 (31.6%), no response in 28 (46.7%) patients.
Conclusions: Splenectomy is the most effective treatment modality when treating children with chronic ITP whose symptoms are severe
Incidence and Predictors of Early Treatment-Related Mortality In Pediatric Acute Lymphoblastic Leukemia In Baghdad (Iraq)
The overall cure rates for children with acute lymphoblastic leukemia (ALL) treated and managed in high-income countries have reached approximately 80% over the last two decades. Unfortunately, these advances in survival have not fully translated into low-income countries where the survival rates remain significantly lower (<35%). Potential reasons for these different results include higher rates of relapse, a high degree of treatment abandonment, insufficient diagnostic work-up procedures, limited availability of effective drugs and supportive measures, and, consequently, high rates of treatment-related mortality (TRM). We examined the incidence, causes and risk factors for early (<60 days) TRM in pediatric patients (15 years) with newly diagnosed ALL managed at the oncology unit of the Children Welfare Teaching Hospital in Baghdad (Iraq), over a 3-year period (2007 – 2009). Data were prospectively collected in Baghdad and analyzed at the GIMEMA Data Center in Rome. From January 2007 to December 2009, a total of 319 children (median age 5.2 years, range 0.3–13.9; 171 males and 148 females) with newly diagnosed ALL were registered; the diagnosis of ALL was confirmed by BM aspirate, according to the FAB classification; patients with L3 morphology (6 cases) were included. The median duration of symptoms prior to diagnosis was 4 weeks, ranging from 1 to 76 weeks. At disease onset, 179 children (56%) presented fever and 30 (9.4%) had hemorrhages; liver and renal functions were impaired in 9/290 (3%) and 21/289 (7.3%) patients with available data. The median Hb level was 7.0 g/dl (range 2.4–14.9), the median WBC count was 16.9 x 109/l (range 0.2–900) and the median platelet count was 35.0 x 109/l (range 0.1–598). CSF was positive in 14/290 children (4.8%). Patients were defined as low (153, 48%), intermediate (127, 40%) or high (33, 10%) risk according to clinical and laboratory parameters (age, hepatosplenomegaly, mediastinal mass, WBC, Hb level, platelet count, CNS and testicular infiltration). Sixteen children were discharged following the parents’ decision (14 before any treatment and 2 after 2 days); 303 children are evaluable for early TRM. Treatment consisted of a modified BFM-95 protocol in the first 31, a modified MRC UKALL-2003 protocol in 266 and the LMB/FAB-96 protocol in the 6 children with ALL-L3 morphology. Up to September 25, 2008 all trials did not include the 7-day steroid pre-phase that was introduced thereafter. A total of 249 children (82%) achieved a complete response in the first 60 days of treatment. The cumulative incidence of early TRM was 16% (48/303); it significantly decreased throughout the study period (2007: 21/88, 24%; 2008: 15/98, 15%; 2009: 12/117, 10% p 0.009). Several variables (sex, age, symptoms duration, hepatosplenomegaly, Hb level, WBC count, platelet count, bleeding, fever, impaired liver and renal function, CNS positivity, risk group, steroid pre-phase and induction complications) were examined as potential predictors of TRM. In univariate analysis, the occurrence of induction complications significantly increased the early TRM: hemorrhage 26% vs 11% p 0.001; infection 18% vs 2% p 0.005. A highly significant favorable impact on early TRM was represented by the 7-day steroid pre-phase; 36/169 children (21%) who did not receive the pre-phase died within the first 60 days of treatment compared to 12/134 children (9%) who underwent the steroid pre-phase (p 0.003). When the steroid pre-phase was placed in multivariable models with each of induction complications or other clinical parameters, it remained an independent predictor of TRM.
Our experience confirms that a protocol-based care of children with ALL has to include the prednisone pre-phase that in low-income countries may contribute to a better risk definition and also to a significant reduction of early TRM
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POSTER: AML-396 Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Initial Results of a Novel, Ongoing BPDCN International Registry Program
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AML-396 Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN): Initial Results of a Novel, Ongoing BPDCN International Registry Program
Comprehensive global collaboration in the care of 1182 pediatric oncology patients over 12 years: The Iraqi–Italian experience
Abstract Background Iraq's health care system has gradually declined after several decades of wars, terrorism, and UN economic sanctions. The Oncology Unit at Children's Welfare Teaching Hospital (CWTH) in Baghdad was lacking basic facilities and support. To address this shortcoming, a humanitarian and educational partnership was established between CWTH and Sapienza University of Rome (SUR). Methods We investigated the outcomes of 80 online and 16 onsite educational sessions and 142 teleconsultation sessions from 2006 to 2014. We also determined the outcomes of pathology reviews by SUR of 1216 tissue specimens submitted by CWTH from 2007 until 2019 for second opinions. The primary outcomes were discordance, concordance, and changes among clinical diagnoses and pathology review findings. The measures included the frequency of teleconsultation and tele‐education sessions, the topics discussed in these sessions, and the number of pathology samples requiring second opinions. Findings A total of 500 cases were discussed via teleconsultations during the study period. The median patient age was 7 years (range, 24 days to 16·4 years), and the cases comprised 79 benign tumors, 299 leukemias, 120 lymphomas, and 97 solid tumors. The teleconsultation sessions yielded 27 diagnostic changes, 123 confirmed diagnoses, and 13 equivocal impacts. The pathology reviews by SUR were concordant for 996 (81·9%) cases, discordant for 186 (15·3%), and inconclusive for 34 (2·8%). The major cause of discordance was inadequate immunohistochemical staining. The percentage of discordance markedly decreased over time (from 40% to 10%). The cause of the improvement is multifactorial: training of two CWTH pathologists at SUR, better immunohistochemical staining, and the ongoing clinical and pathologic telemedicine activities. The partnership yielded 12 publications, six posters, and three oral presentations by CWTH investigators. Interpretation The exchange of knowledge and expertise across continental boundaries meaningfully improved the diagnoses and management of pediatric cancer at CWTH
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Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN):Multi-Center Collaboration and Global Registry Program
Background BPDCN is a rare, aggressive hematologic malignancy derived from the precursors of plasmacytoid dendritic cells (pDC), typically presenting with primary cutaneous dissemination and involvement of bone marrow (BM) as well as extramedullary sites including central nervous system (CNS). In 2008, it was recognized by WHO as a distinct entity and is now recognized under Myeloid/Histiocytic/Dendritic neoplasms in the 2022 5th edition update of WHO Classification. Since the recognition of this unique entity, recent efforts have been put forward for greater understanding and international collaboration, however usually focused on North America and Europe(Pemmaraju at al and Pagano et al), with this current effort representing the largest known international efforts including more parts of the world, including Asia, Middle East and Africa. Objective Due to its historical rarity and heterogeneous presentation of disease, as well as differences in availability of therapies across geo-locations, at present there is no worldwide consensus on the management of BPDCN. The outcomes remain poor, and the optimal therapy of disease remains to be determined. Launched on July 1st, 2022, the aim of our global registry is through a multi-center international collaboration to build a large database of patients with BPDCN in order to generate data-driven diagnostic and treatment recommendations. Materials and Methods The registry collects retrospective and prospective data on clinical presentation, diagnostics, treatment regimens and outcomes of patients diagnosed with BPDCN after January 1st, 2010. The data is collected globally through eCRF and Excel form. The Registry is included in ClinicalTrials.gov database (NCT05430971). Results Through July 15 th, 2023, 15 centers from 11 countries (Armenia, Canada, Cyprus, Egypt, Georgia, India, Italy, Taiwan, Turkey, UK and USA) have joined the registry and another 13 centers from USA, UK, Egypt, Iraq, Canada, Netherlands, Tajikistan, Czech Republic, Guatemala, Brazil, Serbia, Germany are onboarding. 27 retrospective and 2 prospective pts are currently included in the registry. 76 % of pts are male, in keeping with historical expectation BPDCN. Median age at diagnosis was 62 years (4-97). In 90 % of patients the tumor cells were triple positive with CD4+, CD56+, CD123+ immunophenotype. 53.6% of patients presented with cutaneous manifestation. 21% of pts received the initial treatment with AML-based regimens, 66% with ALL-based and 7% with lymphoma-based regimens. 21% of pts underwent alloSCT. 35% of pts were diagnosed after 2018, only 1 patient has received tagraxofusp, a CD123-directed therapy that received FDA approval for previously untreated or relapsed/refractory BPDCN in 2018(Pemmaraju N et al NEJM 2019). 69 % of patients achieved complete response (CR), from which 90 % were treated with ALL-based regimens. 52% of patients experienced relapse. Conclusion Current analysis of a 29-patient worldwide cohort of BPDCN demonstrates a high degree of heterogeneity in treatment regimens based on a multitude of considerations including geographic, socio-economic, drug availability, and varied clinical preferences. The outcome of ALL-based treatment was superior as compared to AML-based, but relapse rate remained high. Further global collaboration is needed to collect additional data and to identify the best diagnostic and therapeutic approaches for this challenging disease
Human Development Index and its association with staff spiritual care provision: a Middle Eastern oncology study
BackgroundAlthough staff spiritual care provision plays a key role in patient-centered care, there is insufficient information on international variance in attitudes toward spiritual care and its actual provision.MethodsA cross-sectional survey of the attitudes of Middle Eastern oncology physicians and nurses toward eight examples of staff provision of spiritual care: two questionnaire items concerned prayer, while six items related to applied information gathering, such as spiritual history taking, referrals, and encouraging patients in their spirituality. In addition, respondents reported on spiritual care provision for their last three advanced cancer patients.ResultsSeven hundred seventy responses were received from 14 countries (25% from countries with very high Human Development Index (HDI), 41% high, 29% medium, 5% low). Over 63% of respondents positively viewed the six applied information gathering items, while significantly more, over 76%, did so among respondents from very high HDI countries (p value range, p<0.001 to p=0.01). Even though only 42-45% overall were positively inclined toward praying with patients, respondents in lower HDI countries expressed more positive views (p<0.001). In interaction analysis, HDI proved to be the single strongest factor associated with five of eight spiritual care examples (p<0.001 for all). Significantly, the Middle Eastern respondents in our study actually provided actual spiritual care to 47% of their most recent advanced cancer patients, compared to only 27% in a parallel American study, with the key difference identified being HDI.ConclusionsA country's development level is a key factor influencing attitudes toward spiritual care and its actual provision. Respondents from lower ranking HDI countries proved relatively more likely to provide spiritual care and to have positive attitudes toward praying with patients. In contrast, respondents from countries with higher HDI levels had relatively more positive attitudes toward spiritual care interventions that involved gathering information applicable to patient care