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In this paper, we report a life‐threatening condition and relate our experience in managing a hemophilia B patient who required three surgical procedures, highlighting the difficulties we encountered in our setting and propose some tangible.

Acute Myeloblastic Leukaemia (AML) is a malignant haemopathy characterized by infiltration and accumulation in the bone marrow by myeloblastic-type blast cells causing medullary insufficiency. At the global level, the search for improved AML treatment is a long-standing concern.PurposeTo evaluate a new protocol AML MA 2003 its therapeutic results and tolerance in de novo AML patients.MethodsIt was a prospective and descriptive unicentre study carried out from January 2003 to December 2010. It included adults of 20 to 60 years with a diagnosis of de novo AML except a promyelocytic leukemia and without treatment by hydroxyurea.ResultsA total of 962 AMLs were diagnosed, 438 met the inclusion criteria, 48.4% women and 51.6% men with a median age of 39.8 years. 36.1% received hydroxyurea in pre-induction. 96.3% patients were induced, 76 (18%) died. A total of 178 (40.6%) of the 438 evaluable patients reached the maintenance phase. The overall survival of all patients was 32.4% at 5 years. Survival without event was 17.6% for all patients.ConclusionThe results of this single-center study illustrate the difficulty of treating AML patients despite the use of drugs recognised for their effectiveness in appropriate doses.

Cytogenetic abnormalities are frequently reported in the literature describing the presence of chromosomal rearrangements in important cases of acute myeloid leukemia (AML); the rate can reach 50–60% of cases of AML. Cytogenetic abnormalities represent an important prognosis factor, their analysis is crucial for AML; cytogenetic study permits to classify prognostic groups and indicate the treatment strategy and helps to improve the outcome of these patients and to increase their chances of cure. Hundreds of uncommon chromosomal aberrations from AML exist. This chapter summarizes chromosomal abnormalities that are common and classifies AML according to the World Health Organization (WHO) classifications from 2008 to 2016; we will discuss briefly gene mutations detected in normal karyotype (NK) AML by cutting-edge next-generation sequencing technology, like FLT3-ITD, nucleophosmin (NPM1), CCAAT/enhancer-binding protein alpha (CEBPA), and other additional mutations.

AIM: The aim of the study was to improve the quality of pain management in Moroccan pediatric oncology units, the Moroccan Society of Paediatric Haematology/Oncology initiated a national quality improvement project in 2014 with the support of the Lalla Salma Foundation for Prevention and Treatment of Cancer. METHODS: To assess the current situation of pain management in Moroccan pediatric oncology patients, two cross-sectional surveys were conducted, involving patient/parental proxies and health-care providers’. RESULTS: The first survey concerned 108 care providers from five institutions. The second survey covered 155 children with cancer from the five Moroccan pediatric oncology units. Among them, 145 reported suffering from pain, which patients/families attributed to the underlying cancer (n = 85), to procedures and treatment (n = 46), or to both the cancer and procedures/treatment (n = 19). Procedural pain was mainly related to lumbar puncture and bone marrow aspirate. The majority of patients/parents reported that pain negatively impacted their emotional, physical, and social functioning. The majority of parents requested further information and communication about pain management. CONCLUSION: Both health-care providers and families of children with cancer in Morocco report need for pain management improvement, including in institutional and educational practices. This current baseline data have informed the development of our ongoing project including continuing education, training, and practice policies development.

According to the World Health Organization, medulloepithelioma belongs to the embryonal neoplasm entity. It is a very rare, highly malignant tumor typically affecting infants and young children. Usually, the tumor arises in the eye or in the central nervous system; a peripheral location has been rarely reported without an established treatment. The recognition and separation of this neoplasm from other differential tumors are mandatory for better understanding of its biology and determination of optimal treatment. This paper reports a case of an ectopic intrapelvic medulloepithelioma with liver metastasis in a 3-year-old girl.

BACKGROUND: Concomitant thymoma and T- lymphoblastic/leukaemia lymphoma is possible. Secondary thymoma after treatment for T-lymphoblastic/leukaemia lymphoma was also occasionally reported, although this is quite rare. CASE REPORT: We report a case of 44-year-old women with secondary thymoma after chemotherapy treatment for T Acute Lymphoblastic leukaemia/lymphoma. Diagnosis of lymphoblastic/leukaemia lymphoma was made in 2015 by morphological and histological study. The patient underwent Moroccan protocol for acute lymphoblastic leukaemia (MARALL) from 2015 to 2017 and achieved complete remission. One year later, the patient developed an anterior mediastinal mass, relapse was suspected, but the surgical biopsy was performed and histological, the mass showed thymoma. CONCLUSION: At the time of diagnosis of thymoma for a patient treated for T-lymphoblastic/leukaemia lymphoma it is necessary to eliminate a relapse because the distinction between thymoma and T-lymphoblastic/leukaemia lymphoma is sometimes difficult, and the association is possible.

Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a non-malignant disease involving clonal proliferation of Langerhans cells. It is an orphan disease affecting mainly the child and the young adult. Its etiology is still unknown, and its clinical spectrum is quite wide. Systemic treatment incorporating steroids and cytostatic drugs for at least one year has improved prognosis of multisystem LCH and represents the current standard of care. We report the case of a multifocal bone LCH in a 2-year-old girl affecting the frontal and parietal bones, associated with emphysematous bulla. The diagnosis was confirmed with histological examination. The evolution was favorable after treatment. Based on this observation, we will make a brief review of the literature to present the clinical, histological, radiological, therapeutic and evolutionary aspects of this orphan disease.

Cutaneous diffuse B-cell lymphoma leg type is a rare entity, it represents approximately 5% of primary cutaneous lymphomas. It has a poor prognosis. We report case of t 58 years old patient with a cutaneous B cell lymphoma leg type with dual location at the upper and lower limbs and bone lysis. Histological examination of the skin biopsy showed diffuse lymphomatous proliferation dissecting collagen, made of large cells with abundant cytoplasm moderately ill-defined, ovoid nuclei and with a discreet nuclear irregularity. The immunohistochemical study revealed lymphoma cells phenotype B with a homogeneous expression of CD 20, a homogeneous expression of CD 3, CD 43 positive. Bcl 2 was positive, asthe Bcl 6 and Mum 1. CD 5, CD10, MPO, CD 34, and Tdt were negative. The Ki 67 was positivein90%. The staging showed extensive osteolysis of the lower end of the humerus and the ulna. The patient received 2 courses RCHOP, with faillure of treatment, and then she received one course of RDHAP before dying. The particularity of this observation consists in the age; our patient was younger than the average, and the double localization of lymphoma in both arm and leg, with osteolysis. We discuss also the difficulties of treatment of this disease.

We conducted a retrospective analysis of 283 patients with acute myeloid leukemia (AML) treated in our unit, we will report the incidence of AML in elderly, describe cytogenetic characteristics of this population, observe rare and novel cytogenetic abnormalities and compare our finding with that previously reported in literature. Among the 283 patients, 157 (54,4%) patients performed the karyotype, cytogenetic analysis failed in 17 cases (11%). Prognostic group distribution was found to be favorable in 8 patients (6%) with 6 cases of t (8; 21) and 2 cases of inv (16), intermediate group in 94 patients (67%), including 58 cases (41,5%) with a normal karyotype, and an unfavorable group in 38 patients (27%) including complex karyotype (15%), -5 or del 5q (3%), -7 or del 7q (3.5%), t(9,22) (2%). Some rare anomalies were observed. However, occurrence of a complex karyotype was more frequent than younger patients. In our unit, elderly benefit from supportive care, our study shows that it is heterogeneous group and our treatment approach have to change especially for patient from favorable risk group who can benefit from intensive chemotherapy. We have to improve our diagnosis with including molecular genetics that provide a documented substrate for a thoughtfully considered treatment plan. AIM: The goals of this paper are: to report the incidence of AML in elderly, to describe cytogenetic characteristics of this population, to observe rare and novel cytogenetic abnormalities and lastly, to compare our finding with that previously reported in the literature. METHODS: We conducted a retrospective analysis of 283 patients with acute myeloid leukaemia (AML) treated in our unit, we will report the incidence of AML in elderly, describe cytogenetic characteristics of this population, observe rare and novel cytogenetic abnormalities and compare our finding with that previously reported in the literature. RESULTS: Among the 283 patients, 157 (54.4%) patients performed the karyotype, the cytogenetic analysis failed in 17 cases (11%). Prognostic group distribution was found to be favorable in 8 patients (6%) with 6 cases of t (8; 21) and 2 cases of inv (16), intermediate group in 94 patients (67%), including 58 cases (41,5%) with a normal karyotype, and an unfavorable group in 38 patients (27%) including complex karyotype (15%), -5 or del 5q (3%), -7 or del 7q (3.5%), t (9; 22) (2%). Some rare anomalies were observed. CONCLUSION: However, the occurrence of a complex karyotype was more frequent than younger patients. In our unit, elderly benefit from supportive care, our study shows that it is a heterogeneous group and our treatment approach have to change especially for the patient from favourable risk group who can benefit from intensive chemotherapy. We have to improve our diagnosis with including molecular genetics that provides a documented substrate for a thoughtfully considered treatment plan.

Treatment of acute myeloblastic leukemia in children, adolescents and young adults (AYA) is a challenge in low-income countries. To evaluate treatment outcomes of children (≤ 15 years) and AYA (15–30 years) diagnosed with novo AML and treated in a single center according to the AML-MA 2011 protocol. From January 2011 to December 2015, eligible patients (age ≤ 30 years) with novo AML had been enrolled on a uniform treatment protocol. The diagnosis was confirmed according to the FAB classification using the WHO 2008 criteria. Patients with WBC ≥ 50 G/L had pretreated 4 days of hydroxyurea followed by two inductions and two consolidations. Supportive care consisted of transfusion of labile blood products, antibiotics and antifungals, and patient and family education by the hygiene team. 155 patients were recruited, 41 were

Bone and joint pain may be a presenting symptom in 25% of patients with acute leukemia; whereas generalised osteopenia and vertebral complications are less common. An 11 years old boy reported a persistent low back pain since 6 weeks. The physical exam showed exquisite vertebral pain. Imaging objectified vertebral body collapse L1 and L2. Count blood cells found pancytopenia without blasts. B acute lymphoblastic leukemia was diagnosed. The boy received chemotherapy with orthopedic treatment. He was followed for two and half a year on complete remission.

Extracellular nucleotides regulate mucociliary clearance in the airways and surfactant secretion in alveoli. Their release is exquisitely mechanosensitive and may be induced by stretch as well as airflow shear stress acting on lung epithelia. We hypothesized that, in addition, tension forces at the air-liquid interface (ALI) may contribute to mechanosensitive ATP release in the lungs. Local depletion of airway surface liquid, mucins, and surfactants, which normally protect epithelial surfaces, facilitate such release and trigger compensatory mucin and fluid secretion processes. In this study, human bronchial epithelial 16HBE14o−and alveolar A549 cells were subjected to tension forces at the ALI by passing an air bubble over the cell monolayer in a flow-through chamber, or by air exposure while tilting the cell culture dish. Such stimulation induced significant ATP release not involving cell lysis, as verified by ethidium bromide staining. Confocal fluorescence microscopy disclosed reversible cell deformation in the monolayer part in contact with the ALI. Fura 2 fluorescence imaging revealed transient intracellular Ca2+elevation evoked by the ALI, which did not entail nonspecific Ca2+influx from the extracellular space. ATP release was reduced by ∼40 to ∼90% from cells loaded with the Ca2+chelator BAPTA-AM and was completely abolished by N-ethylmalemide (1 mM). These experiments demonstrate that in close proximity to the ALI, surface tension forces are transmitted directly on cells, causing their mechanical deformation and Ca2+-dependent exocytotic ATP release. Such a signaling mechanism may contribute to the detection of local deficiency of airway surface liquid and surfactants on the lung surface.

Sickle cell anemia results from a single amino acid substitution in the gene encoding the β-globin subunit. Polymerization of deoxygenated sickle hemoglobin leads to decreased deformability of red blood cells. Hashimoto's thyroiditis is a common thyroid disease now recognized as an auto-immune thyroid disorder, it is usually thought to be haemolytic autoimmune anemia. We report the case of a 32 years old women admitted for chest pain and haemolysis anemia in which Hashimoto's thyroiditis and sickle cell anemia were found. In our observation the patient is a young woman whose examination did not show signs of goitre but the analysis of thyroid function tests performed before an auto-immune hemolytic anemia (confirmed by a high level of unconjugated bilirubin and a Coombs test positive for IgG) has found thyroid stimulating hormone (TSH) and positive thyroid antibody at rates in excess of 4.5 times their normal value. In the same period, as the hemolytic anemia, and before the atypical chest pain and anguish they generated in the patient, the search for hemoglobinopathies was made despite the absence of a family history of haematological disease or painful attacks in childhood. Patient electrophoresis's led to research similar cases in the family. The mother was the first to be analyzed with ultimately diagnosed with sickle cell trait have previously been ignored. This case would be a form with few symptoms because the patient does not describe painful crises in childhood or adolescence.

Introduction :With the improvement in the risk assessment, therapeutic advances and supportive care, about 50% of children and young adults with newly diagnosed acute myeloid leukemia(AML) can be cured in high income countries. However similar survival advantages are not seen in patients from low or middle income countries. In Morocco, the main causes of poor outcome in patients with AML are delayed diagnosis, early (before the start of treatment) and induction death, induction failure and therapy abandonment. In 2011, the National AML-MA-2011 protocol was developed to treat AML patients according to international standards and has focused on improving particularly supportive care, prevention and management of infection, transfusion support, implementation of hand hygiene, and education of patients, families and nurses. The goals of the AML-MA-2011 protocol were to obtain of favorable risk AML more than 70% complete remission rate, less than 10% on therapy mortality and event free survival (EFS) (4 years) of 40%. Aim of this study : To evaluate and compare treatment outcomes (Complete remission rate, overall survival [OS] and EFS) of children (≤ 15 yrs.) and adolescents and young adults (AYA) (15-30 yrs) diagnosed with de novo AML and treated at a single center on AML-MA 2011 Patients and methods : From January 2011 to December 2015, eligible patient (age ≤ 30 yrs) with de novo AMLwere enrolled ona uniform treatment protocol. Patients with secondary AML, Down syndrome, those with acute promyelocytic leukemia or organ dysfunctions were excluded. The diagnosis was confirmed according to the FAB classification using WHO criteria, At diagnosis BM MPO staining, immunophenotyping and lumbrteritisar puncture were performed. Karyotype was performed on marrow sample (with minimum 20 metaphases analyzed) using R banding technique. Patients with hyperleukocytosis (WBC≥ 50G/L) received as a pre-phase 4 days of hydroxyurea to 50mg/kg/day then 2 inductions and 2 consolidations. The two courses of induction associated cytarabine (100mg/m² q 12h for 10 days), Daunorubicin (50 mg/m² on days 2, 4, 6 for the first course, and on days 1, 3, 5 for the second course) and etoposide (100mg/m² for 5 days for the second course of induction). The consolidation included Cytarabine (3g/m²q 12h days1-3 for first and second course) plus Daunorubicin (30mg/m² on days 3, 4 at the first consolidation). L-Asparaginase 6000UI/m² on day 4 was give at second consolidation. All patients received CNS prophylaxis. Patients with CNS disease received addutional intrathecal. The supportive care consisted of blood product transfusion, antibiotic and antifungal, and patient and family education by hygien team. Complete remission (CR) was defined as normal clinical examination, no evidence of chloroma, peripheral counts recovery (ANC ≥ 1.0 G/L ; platelets ≥ 75 G/L without transfusion) and end of induction II bone marrow showed normal hematopoietic elements and Résultats :During the study period a total of 155 patients were enrolled, 41 were < 15 yrs (22 boys ; median age 7.8 yrs.). Of the 114 AYA enrolled, 48 were women and the median age was 23 yrs. In children median hemoglobin was 6.75g/dl and platelet 39.5G/L, 15/41 (36.6%) had AML2 and 36/41 (87.8%) had immunophenotyping. Among young adults 32/114 (28.1%) were hyperleucocytosis, 7.38g/dl and 52.81G/L were median hemoglobin and platelets respectively, 31/114 (29.8%) had AML1 and 89/114 (78.1%) had immunophenotyping. Cytogenetics was performed 90.2% of children and 95.6% of AYA. The median delay from diagnosis to treatment was 11.5 days in children and 29.9 days. Complete remission after two inductions was achieved in 28/41 (68.3%) children and 71/114 (62.3%) adults. The number of failure was 21 in age groups which 4 (9.8%) on children and 17 (14.9%) in young adults. The number of deaths in children is 9 (22%) of which 3 occurred in the first 15 days of induction and 6 from the 15th to 42nd day. AYA 26 (22.8%) deaths had been seen by registered including 5 in the first 15 days and 21 after. The causes of death were dominated by infections including 4 cases in children and 10 adults followed hemorrhage 3 cases among children and 10 adults. Leukostasis cases were recorded in 2 children 6 and young adults. Analysis of the results is summarized in the table. Conclusion : The therapeutic results of the protocol AML-MA 2011 were similar in both year groups, but are far from the goals of treatment. Disclosures No relevant conflicts of interest to declare.

Background : Langerhans Cell Histiocytosis (LCH) is a rare disease with high survival rate. We noted poor outcomes in Morocco and thus this study was conducted to identify causes of preventable treatment failure in children. Patients & Methods : This study includes cases of children LCH followed in six Pediatric Oncology Units part of the Moroccan LCH Study Group (Groupe Marocain d'Etude de l'Histiocytose) between 2000 and 2009. Patients were categorized into risk group or low risk group as per the International Histiocyte Society classification. Results: Forty-two patients were included in the present study. The median age at diagnosis was 20.5 months. The sex-ratio M/F was 5. The onset symptom was a skin lesion in 20% of the cases and bone involvement in 17%. Seventeen patients had unifocal LCH (40%). The most common site of unifocal LCH was bone (65%) and the mean duration of the first episode treatment was 3 months. Complete or partial remission was achieved in 58% of the cases. Among 20 patients who had risk organ involvement, 11 died from progressive disease and 2 died from septic shock. The overall survival rate was 40% at 5 years. Conclusion : The most common cause of treatment failure was refractory disease in 26%. However, toxic death and abandonment caused an additional 24% of patients to fail treatment. Reducing this preventable failure requires carefully designed interventions. Thus, the Moroccan LCH Study Group was established in March 2010 and set as main objective the improvement of the therapeutic management

Introduction: The outcome of acute myeloid leukaemia (AML) in emerging countries is very dismal. In Morocco,the complete remission rate (CR) has improved from less of 10% in 1980s to more than 60% in 2000s. However this improvement is far from satisfactory to improve five year survival rate. The aim of study was to evaluate preliminary outcomes of children ≤15years treated according to AML- MA 11protocol. Methods : Between the first January 2011 to December 2014, we carrieed a prospective study of all children aged 15 years or less, diagnosed AML. The diagnosis was according by cytological study with cytochemestry: MPO positive (FAB classification), supplemented by immunophenotyping. The prognosis is evaluated by conventional cytogenetic study. AML-MA 2011 protocol consisted of prephase(whenwhite blood cell (WBC) count ≥ 50 G/l) by Hydroxyurea (HU) (50 mg/m2/day for 4 days). The response to HU was evaluated after 2 days; patients were considered responsers if >50% reduction of the initial WBC count. Then 2 inductions based on daunorubicin 50 mg/m2 (3 days), cytarabine 100mg/m2/12 hours (10 days) and etoposide 100 mg/m2 (5 days) for the second course, followed by 3 consolidations with high dose of cytarabine 1-3 g/m2/12 hours (3 days), with intrathecal therapy. The supportive care is assured during all phases of treatment. Results: Forty four patients were enrolled with a median age of 8 years [1-15] and M:F ratio of 1.2. The median WBC count was 22710 elements/mm3; it exceeded 50 000/mm3 in 18 % of cases, more than 100 000/mm3 in 13 % of cases.The cytological studies showed the predominance of AML subtype 2 (31%) followed by subtype 5 (11, 3%). The immunophenotyping was done in 88% of cases. Karyotype performed in 40 out of 44 cases revealed: 9 (22,5%) favorable risk group [8 had the t(8,21); 1 had inv16], 25 (62,5%) intermediate risk, and 6 (15%) unfavorable-risk group. Seven patients received hydroxy urea with 5 good response (71% of cases; 2 patients had favorable risk and 3 had intermediate risk). Four of this patients died. Thirty five patients were treated (3 death before treatment, 4 abandonment of treatment, 1 myelodysplasea syndroma, and 1 bad condition). After the induction cycles; complete remission was achieved in 22 cases (62%), failure in 6 cases, and 7 children dead (20%) [4 septic choc, 2 haemorrhage and 1 acute pulmonary edema]. One patient died between 0 to 14 days during the cure and 6 others after 14 days of treatment. After a mean follow-up of 9months (1 to 43 months), continuous complete remission was achieved in 45,7% of cases, failure in 20%, relapse in 11%, and death in 45,45 % of cases. (toxic death in 25% of cases with high frequence in induction phases: 88%). Theoverall survival rate in 2 years was 41,2%. Table. Evolution of patients according to prognostic groups Status/cytogenetic groups CR (%) Failure (%) Death (%) Relapse (%) OS (%) favorable 55 0 11 22 53,3 intermediate 32 32 16 4 34,4 unfavorable 33 16 16 16 55,6 Conclusion: With aggressive chemotherapy, it is feasible to achieve high CR even in emerging countries. If supportive care is optimal with particular focus on prevention and management of infection and with improved transfusion support, then it's feasible to improve the overall outcome of patients. Finally it is necessary to make the molecular biology in our patients topreciselyindividualize the pronostic groups. Disclosures No relevant conflicts of interest to declare.

Introduction: Treatment of Acute Myeloid Leukemia (AML) with in developing countries is challenging. In Morocco, the major causes of therapy failure are delay in diagnosis, early (prior to start of therapy) and induction deaths, induction failures and abandonment of therapy. Improvement of supportive care with particular focus on prevention and management of infection and improved transfusion support is crucial for better overall outcome. Aim of the study: To evaluate the preliminary results (Complete remission, OS and EFS) in children and young adults with favorable risk group AML treated in a single center with AML MA 2011 protocol. Patients and methods: From January 2011 to December 2014, a uniform treatment protocol was conducted to treat patients with age ≤ 30 years with de novo AML with favorable risk group. The diagnosis was done according to FAB classification, MPO done systematically. Karyotype was performed on marrow sample (20 metaphases analyze at least), R banding technique. Patients with hyperleukocytosis (WBC≥ 50G/L) received as a pre-phase 4 days of hydroxyurea to 50mg/kg/day then 2 inductions and 3 consolidations. The two courses of induction associated Cytarabine (100mg/m² q 12h (day 1-10)), Daunorubicin (50 mg/m² (day 2, 4, 6) for the first course, on days 1, 3, 5 for the second course) and etoposide (100mg/m² only at second course of induction). The consolidation included Cytarabine (3g/m²q 12h (day1-3) for first and second course and 1 g/m² (day1-3) on third course) plus Daunorubicin (30mg/m² (day 3-4 and day 1-3) at the first and third consolidation. L-Asparaginase 6000UI/m² on day 4 was give at second consolidation. Patients received CNS prophylaxis. The supportive care consisted of transfusion, antibiotic and patient and family education by hygiene team. Results: 39/159 patients (24.5%) had a favorable prognosis. They were 13 female and 26 male (Male/Female ratio of 2) with a median age of 21 years and the peak frequency was between 20-30 years (21 patients or 53.8%). Two groups were identified. (Table 1) The means WBC were 39.741 G/L (1.134-425.000G/L) and more than 50 G/L for 17 patients. t(8; 21) represented 71.8% of karyotype and was associated 14 times with other abnormalities: 8 loss of sex chromosome (6 -Y and -X 2), 2 deletions, 2 trisomy and 2 add. Inv16 or t (16; 16), 28.2% of case, was associated with a deletion once and once with trisomy. (Table 1) Molecular biology carried 7 times was positive in 4 cases all AML-ETO. Three patients died before treatment, two in hospital by septic shock (1), subarachnoid hemorrhage (1) and one at home. 36/39 patients were evaluable for the protocol. 17 patients with WBC > 50G/L received hydroxyurea prior to chemotherapy with good response for 16 (94.1%) and 1 (5.9%) death. The median average time from start of treatment was 15 days with a range of 1-52 days. After the two inductions 26 (74.3%) obtained a complete remission, 2 (5.7%) were in failure, and 7 (20%) died in hospitalization during induction 1. The cause of death was: 2 hemorrhage, 3 infections, 1 pulmonary embolism and 1 patient died at home. Conclusion: Therapeutic results are yet far from satisfactory. Complete remission could be improved with reduction of infection toxic deaths. Improvement of supportive care therapy may allow treating patients with intensified treatment with better outcome. Hydroxyurea is efficient to reduce WBC and for best conditions to initiate induction therapy. Table 1. Patient's characteristics All patients Group 1(age ≤15years) Group 2(age≥16 years) Number (%) Median age (Years) 39 (100%) 21 9 (23%) 10 30 (77%) 24 Male/Female 2 3.5 1.7 WBC>50000 (G/L) 17 2 7 FAB M1/M2/M4/M4Eo/other Immunophenotype 12/19/2/4/2 28 (71.8%) 1/7/1/0/0 7 (25%) 11/12/1/4/2 21 (75%) t(8 ;21) 28 (71.8%) 8 (88.9%) 20(66.7%) t(8 ;21)+ other anomalies 14 (50%) 5 (62.5%) 9 (45%) Inv 16 or t(16 ;16)Inv16 or t(16 ;16) + other anomaliesTreatmentComplete remission DeathFailureCauses of death infection/Hemorrhage/otherOSES 11 (28.2%) 2 (18.2%) 36*(92.3%) 26 (72.2%) 7 (18%) 2 2/3/2 51% 30.9% 1 (11.1%) 1 (100%) 9 (100%) 8 (88.9%) 1 (11.1%) 0 0/1/0 10 (33.3%) 1 (10%) 26 (86.7%) 18 (69.2%) 6 (23.1%) 2 (7.7%) 2/2/2 *One death after prephase Disclosures No relevant conflicts of interest to declare.

We present the incidence and the immunologic characteristics of acute lymphoblastic leukemia (ALL) subsets in Moroccan children. We studied 279 unselected patients below the age of 18 years with newly diagnosed ALL. Cases were classified according to immunophenotype: 216 (77.42%) precursor B-cell phenotype (pB-cell), mature B-cell in 4 (1.43%), and T-cell in 59 (21.15%) cases. The subclassification using the CD10 antibody revealed 197 cases pB-ALL CD10+ (91.2%) and 9 cases T-ALL CD10+ (19.2%). The age distribution showed a peak in incidence between 3 and 5 years among the pB-cell ALLs subtype. There was a significantly higher frequency of males in the T-ALL subset (M/F ratio: 2.93 : 1) and more females in the T-ALL CD10+ subset when compared with the T-ALL CD10– subset. All tested pB-cell-lineage ALLs expressed CD19, CD79a, and surface CD22, terminal deoxynucleotidyl transferase (TdT) was detectable in 89.9% of cases, and cells in 74.1% of cases express CD34. All tested T-lineage ALL cells have surface CD7 and cytoplasmic CD3 (cCD3) antigens, CD5 was found in 98.2% cases, and 70.5% express TdT. CD1a, surface CD3 (sCD3), and CD4 are detected in more than 80% of cases; this frequency is higher than the 45% generally observed. Myeloid antigens occur more frequently and were expressed in 124 (57.4%) of pB-cell-ALL cases and 20 (33.9%) of T-cell ALL cases. Our results show that the distribution of ALLs in Moroccan children is similar with the general distribution pattern in developed countries except for the high frequency of T-ALL phenotype. The phenotypic profiles of our patients are close to those reported in literature for B-lineage ALLs; for the T-cell ALL subgroup, the blast cells express more CD1a, surface CD3, and CD4 while expressing less TdT. The high frequency of CD1a expression resulted in an excess of the common thymocyte subtype.

Abstract 4712 Background: Patients with hematological malignancies are at risk for Healthcare Associated Infection (HAIs) due to malignancy types, antineoplastic chemotherapies, immunosuppressant treatment, and prolonged hospital day. HAIs cause delay in cancer treatment, increase morbidity or mortality and increase costs of treatment affecting successful outcomes of cancer care. We describe the outcome of the surveillance in adult hematology patients in our hospital and identified groups most at risk for HAIs. We describe the outcome of the surveillance study in adult hematology patients in Morocco. Methods: A prospective surveillance study included all patients admitted to the adult hematology unit from March 2011 to August 2011. They excluded patients from the transplant unit and day hospital. The Center for Disease Control and Prevention standard definitions were used to determine if an infection was hospital acquired. We collected data in demographics, risk factors, the presence or absence of HAIs and its type. Results: A total of 180 patients that represented 377 admissions, with 4438 hospitalization-days, were included in the study. They have a mean age of 35 years (18–78 years). The sex-ratio H/F was 1.7. The distribution of the patient's diagnosis is related in Table1. 63 patients had 110 episodes of HAIs. The incidence rate of HAIs was 24.74/1000 hospitalization-days. The most frequent of infectious episodes were bacteremia (38.18%) and pneumonia (24.54%). The fever of unknown origin represented 35.45%.The distribution of the intrinsic risk factors with regard to episodes of HAIs was the following: 83.48% presented with neutropenia, 59.79% presented with severe neutropenia. With regard to extrinsic risk factors for HAIs, 61.03% of the infectious episodes had central venous catheterization. The mortality rate due to HAIs was 8%. Conclusion: Although the overall HAI rate is in line with published reports in hematology units, our mortality rate is higher. This finding suggests that we must be more aggressive to prevent infections in the patients in these units. Table I: Distribution of patient's diagnosis and incidence rate of HAIs Infection Incidence rate (/1000 hospital day) N n % Age (years) 18–40 41–60 > 60 Hematologic neoplasia 245 97 35 70 31 9 28,57 31,96 25,71 Acute myeloid leukemia 147 98 89,00 35,27 Acute lymphoblastic leukemia 83 5 4,54 Non Hodgkin's lymphoma 102 5 4,54 Hodgkin's disease 36 0 Aplastic anemia 3 2 Others 6 0 Total 377 110 100 Disclosures: No relevant conflicts of interest to declare.

4713 Background AML with t(8;21) is known to have a more favorable outcome when treated with adequate chemotherapy and supportive care. There have been minimal data on the characteristics and outcome of AML with this translocation in low-income countries. Aim We analyze the clinical and biological characteristics of a large population of children and adults with AML in Casablanca who had t(8;21) in relation to response, event-free (EFS) and overall survival (OS). Methods From 4/1/03 to 3/31/09, eligible patients were treated on the AML-MA2003 protocol at a single center. For patients presenting with WBC>50,000, hydroxyurea (HU) was given for 4 days prior to initiation of standard induction. Treatment included two induction courses of cytarabine, 200 mg/m2/d (days 1-7) and daunomycin 50 mg/m2/d (days 1-3); consolidation was stratified by age: those '20 years had two courses of Capizzi high-dose cytarabine, 2 g/m2 q 12h (days 1-4) with L-asparaginase, 6000 units/m2 day 4; those >20 years had one or two courses of high-dose cytarabine, 1 g/m2 q 12h (days 1-4) with daunomycin 50 mg/m2/d (day 1-3), and maintenance with oral 6-mercaptopurine 60 mg/m2/d and weekly sc cytarabine 40 mg/m2 for 18 months. Events included death, relapse, progression, and abandonment. Results 85 of 535 (15.8%) patients with karyotype results had t(8;21), including 40 with only t(8;21), 25 with additional loss of a sex chromosome, 5 with del (9), and 15 with other cytogenetic abnormalities. Characteristics of the t(8;21) group are shown in the Table. Five adults were ineligible for protocol therapy, due to secondary AML (n=1), refused (n=1) and early death (n=3). Of 33 children (age < 20 years), 28 completed all 4 courses of planned therapy. Five deaths occurred on therapy (1 failure, 4 toxicity) and 11/28 relapsed later, 8-14 months after end therapy. Of 47 adults who received the first induction course, 35 went on to 2nd induction, 34 received 1st consolidation, 22 completed both courses of induction and consolidation, and 20 completed maintenance. There were 12 deaths on therapy and 12 relapses after therapy completion. Overall, 30/80 patients did not complete prescribed therapy: 14 deaths, 1 failure, 4 abandonment, 11 complete remission). 7/8 children and 4/4 adults with t(8;21) had an early response (50% decrease in blasts) to HU. The complete remission rate (CR) after two courses of induction therapy was 91% for children and 72.5% for adults. The 2-year EFS and OS for the patients with t(8;21) was 32% (standard error [SE] 6.3%) and 59% (SE 7.3%) respectively. There was not a significant difference in 2-year EFS by sex (p=0.18), age ' 20 years vs older (p= 0.08), or presence of additional cytogenetic abnormalities accompanying the t(8;21) (p=0.98) Conclusions t(8;21) in Morocco occurred in 15.8% of cases, more frequently than the 7 to12% reported. It was associated with a high incidence of extramedullary disease, FAB M2, frequent deletion of a sex chromosome, preponderance of males, and young age. A third of these favorable-risk AML patients survive event-free 2 years from diagnosis despite the problem of abandonment of therapy and suboptimal supportive care. | | All t(8;21) | Children | Adults | |:------------------------- | -------------- | -------------- | --------------- | | t(8;21) (%) | 85 | 33 (38.8%) | 52 (61.2%) | | Median Age (yrs) | 25 (3-69) | 15 (3-20) | 34 (21-69) | | Male:Female | 1.36 | 1.06 | 1.94 | | Median WBC (range) | 16.6 (0.9-237) | 25.0 (0.9-237) | 14.9 (1.6-64.7) | | WBC ≥ 50,000 | 13 (15.3%) | 8 (24.2%) | 4 (7.7%) | | Extramedullary disease | 11 (12.9%) | 10 (30%) | 1 (1.9%) | | FAB M1/M2/other | 17 / 65 / 3 | 3 / 29 / 1 | 14 / 36 / 2 | | t(8;21)/total AML | 85/535 (16%) | 33/122 (27.0%) | 52/413 (12.6%) | | t(8;21) + other anomalies | 45 (52.9%) | 16 (48.5%) | 29 (55.7%) | Table. 85 patients with t(8;21) Disclosures: No relevant conflicts of interest to declare.

Primary pericardial synovial sarcoma is extremely rare, with few published cases in the literature. We report the case of an adolescent aged 13 years with primary pericardial synovial sarcoma discovered during tamponade, confirmed by molecular biology, and for whom treatment combined radiosurgery and adjuvant chemotherapy. The particularity of the case we are reporting stems from the young age of our patient (13 years) as well as the duration of remission, which is quite long (21 months) prior to a superior mediastinal relapse compared to cases reported in the literature.