Primary splenic angiosarcoma revealed by bone marrow metastasis.

Primary splenic angiosarcomas are the most common malignant non-hematopoietic tumors of the spleen. Metastatic diseases were found in 69% of patients in a reported series but the incidence of bone marrow involvement is unclear. We report a rare case of a 25-years-old Moroccan woman with unsuspected primary splenic angiosarcoma revealed by bone marrow metastasis. She presented with serious anemia and splenomegaly. Bone marrow biopsy revealed proliferating spindle cells. Computed tomography scanning showed an enlarged spleen with heterogeneous lesions. Splenectomy was performed and retrospective histological study of the spleen confirmed the diagnosis. She died 1 year after splenectomy.

[Healthcare-associated infections in a paediatric haematology/oncology unit in Morocco]. / Infections associées aux soins dans une unité d'hématologie-oncologie pédiatrique au Maroc.

OBJECTIVE: HAI cause considerable morbidity and mortality and are associated with prolonged hospital stay and increased health care costs. To describe the incidence of HAI in paediatric cancer patients as the first step towards improving infection control policies. METHODS: A prospective surveillance study was performed in the Casablanca university hospital paediatric haematology/oncology unit over an 8-month period from January to August 2011. Data including extrinsic risk factors associated with HAI were recorded. RESULTS: The incidence of HAI was 28 per 1000 patient-days. The median age was 9.6 years and the most frequent diagnosis was acute myeloid leukaemia (32%). Neutropenia at diagnosis was significantly correlated with the risk of HAI. 55.7% of HAls were nosocomial fever of unknown origin. Gram-negative bacteria were the main pathogens (60%), gram-positive cocci were responsible for 26% of HAI and Candida for 14% of HAI. The length of hospital stay for patients with and without infection were 16.5 and 5 days, respectively (P < 0.001). Six of the 11 deaths were related to HAI. CONCLUSION: These findings suggest the need to evaluate infection control measures in order to reduce morbidity and mortality in paediatric haematology/oncology units.

Infections in hospitalized children and young adults with acute leukemia in Morocco.

BACKGROUND: Overall survival from leukemia is less in low and middle-income countries than in high-income countries. Our purpose was to describe the incidence, clinical features, and mortality of febrile illness with or without documented infection in children and young adults treated for AML and ALL in two centers in Rabat and Casablanca during 2011. METHODS: This retrospective cohort study included patients <30 years of age who were newly diagnosed with AML and ALL in 2011 in Casablanca and Rabat. Each patient's chart was evaluated for patient demographics, febrile episodes, chemotherapy regimen, and clinical or microbiological evidence of infection, neutropenia, antibiotics, and mortality. RESULTS: One hundred sixty-six evaluable patients had 228 inpatient febrile episodes. The median number of febrile episodes in AML was three per patient, and for ALL, one per patient. Clinically identified infections mainly included pneumonitis and mucositis. Coagulase negative staphylococcus was the most commonly isolated bacterium, followed by gram-negative bacteria. Fifty-three percent of febrile episodes were classified as fever of undetermined origin. Broad-spectrum antibiotics were routinely used, with the addition of antifungals in 62 episodes and vancomycin in 83 episodes. The rate of deaths per febrile illness was 11.3% (16/141) in patients with AML, and 9.2% (8/87) in patients with ALL. CONCLUSION: The higher rate of infectious deaths in leukemia compared to that reported in high-income countries, suggests that improvements in infection care and prevention, including consistent access to rapid hospitalization, diagnostics and antibiotics; and standardizing quality of patient care are necessary to improve as well as survival in patients with leukemia in Morocco.

Improving the prognosis of pediatric Hodgkin lymphoma in developing countries: a Moroccan Society of Pediatric Hematology and Oncology study.

BACKGROUND: The event-free survival (EFS) of children with Hodgkin lymphoma (HL) exceeds 80% in high income countries (HIC), but little is known about this rate in developing countries. PROCEDURE: A prospective national protocol for children with classical HL was implemented in Morocco to increase EFS by careful risk stratification, providing each cycle of therapy on time, decreasing treatment abandonment, improving communication among healthcare providers, and improving data collection. Patients were stratified into a favorable risk group (Ann Arbor stages I and II, no B symptoms, no bulky disease, and no contiguous (E) lesions) and received four cycles of vinblastine, doxorubicin, methotrexate, and prednisone (VAMP) or an unfavorable risk group (all others) who received two cycles of vincristine, procarbazine, prednisone, and doxorubicin (OPPA) and four cycles of cyclophosphamide, vincristine, procarbazine, and prednisone (COPP). All patients received involved-field radiotherapy 25.5 Gy after completion of chemotherapy. EFS was calculated counting death, relapse/resistant disease, and abandonment as events. RESULTS: From February 2004 to December 2007, 160 patients enrolled; 138 (86%) had unfavorable risk features. Twenty patients (12.5%) abandoned treatment, 16 relapsed or had resistant disease, and 6 died (3 unexplained, 2 varicella, and 1 suicide). The estimated 5-year EFS was 70 ± 4% and overall survival 88 ± 3%. CONCLUSIONS: Good outcomes for pediatric HL patients can be achieved in LMIC using a multidisciplinary team approach, uniform protocol-based therapy, twinning partnership among oncology units in-country and abroad, and a data collection system to monitor compliance and identify gaps in care.

Trends of hematopoietic stem cell transplantation in the Eastern Mediterranean region, 1984-2007.

Hematopoietic stem cell transplantation (HSCT) activity was surveyed in the 9 countries in the World Health Organization Eastern Mediterranean region that reported transplantation activity. Between the years of 1984 and 2007, 7933 transplantations were performed. The number of HSCTs per year has continued to increase, with a plateau in allogeneic HSCT (allo-HSCT) between 2005 and 2007. Overall, a greater proportion of transplantations were allo-HSCT (n = 5761, 77%) compared with autologous HSCT (ASCT) (n = 2172, 23%). Of 5761 allo-HSCT, acute leukemia constituted the main indication (n = 2124, 37%). There was a significant proportion of allo-HSCT for bone marrow failures (n = 1001, 17%) and hemoglobinopathies (n = 885, 15%). The rate of unrelated donor transplantations remained low, with only 2 matched unrelated donor allo-HSCTs reported. One hundred umbilical cord blood transplantations were reported (0.017% of allo-HSCT). Peripheral blood stem cells were the main source of graft in allo-HSCT, and peripheral blood stem cells increasingly constitute the main source of hematopoietic stem cells overall. Reduced-intensity conditioning was utilized in 5.7% of allografts over the surveyed period. ASCT numbers continue to increase. There has been a shift in the indication for ASCT from acute leukemia to lymphoproliferative disorders (45%), followed by myeloma (26%). The survey reflects transplantation activity according to the unique health settings of this region. Notable differences in transplantation practices as reported to the European Group for Blood and Marrow Transplantation over recent years are highlighted.

Status of hematopoietic stem cell transplantation in the WHO Eastern Mediterranean Region (EMRO).

Several centers are now performing allogeneic hematopoietic stem cell transplantation (HSCT) in the World Health Organization Eastern Mediterranean Region (EMRO) but the availability is still limited due to high cost and the need for multi-disciplinary team and an advanced laboratory support. Special issues including compatible donor availability, potential for alternate donor programs, differences in pattern of disease, pre-HSCT general status particularly for patients with BM failure, high sero-positivity for CMV, Hepatitis B and C infection and specific observations about GVHD with its relation to genetically homogeneous community are discussed. A total of 17 HSCT programs (performing five or more HSCTs annually) exist in nine countries of the EM region. Only six programs are currently reporting to EBMT or IBMTR. A total of 7617 HSCTs including 5701 allogeneic HSCTs have been performed. Due to low HSCT team density (1.5583 teams/10 million inhabitants versus 14.4333 in Europe) and very low HSCT team distribution (0.2729 teams/10,000 sq km area versus <1 to 6 teams in Europe) only 70.8% of total population has access to such a program in EM region. GNI/capita had no clear association with low HSCT activity; however improvement in infrastructure and establishment of EM regional HSCT registry need prioritization.

Nutritional status at diagnosis of children with malignancies in Casablanca.

INTRODUCTION: Nutritional assessment is an essential component of the initial assessment of children with cancer. Malnutrition may be present at diagnosis due to the effects of the malignancy or, in low income countries (LIC), due to poverty and an inadequate diet. PURPOSE: The aim of this study is to evaluate the prevalence of malnutrition at diagnosis in children with cancer in Morocco. PROCEDURE: Nutritional status of 100 children aged less than 18 years with newly diagnosed malignancy between January 2005 and January 2006 was evaluated by anthropometric and biochemical parameters before initiating therapy. We measured weight, height, weight-for-height using z-scores index for children and body mass index for adolescents, triceps skinfold thickness and mid-upper arm circumference, and serum albumin. RESULTS: A total of 100 patients were included. The mean age was 7 years (range 1 to 18 years). Sixty percent were boys. The diagnosis was: Burkitt lymphoma (n = 19), acute myeloblastic leukaemia (n = 18), acute lymphoblastic leukaemia (n = 14), rhabdomyosarcoma (n = 13), Ewing sarcoma (n = 7), nephroblastoma (n = 6), Hodgkin disease (n = 5), osteosarcoma (n = 5), retinoblastoma (n = 4), neuroblastoma (n = 3), germ cell tumor (n = 3), orbital lymphoma (n = 1), cerebral lymphoma (n = 1), ependymoma (n = 1). Incidence of malnutrition ranged from 20 to 50%, depending upon the measurement used. CONCLUSION: The prevalence of malnutrition in this study was high, so interventions are being implemented to improve the nutritional status of these patients.

Treatment of B-cell lymphoma with LMB modified protocols in Africa--report of the French-African Pediatric Oncology Group (GFAOP).

BACKGROUND: The French African Paediatric Oncology Group (GFAOP) was set up in October 2000 to improve the quality of care of children with cancer in Africa. Eight pediatric oncology units from Algeria, Cameroon, Madagascar, Morocco, Tunisia, and Senegal have been involved. METHODS: Patients less than 18 years with cytology or histology proven B-cell non-Hodgkin lymphoma were included. Two LMB89 modified regimens were proposed (MAT and GFA). RESULTS: From April 2001 to April 2004, 343 cases were registered. Thirty seven patients were excluded. Thirteen patients were stage I, 26 stage II, 209 stage III and 50 stage IV including 8 L3 acute lymphoblastic leukemia (ALL3) cases. Three year OS of the whole population of patients is 61%. In GFA group 36 months OS is 63.6% in stages I/II, 51.6% in stage III and 35.8% in stage IV. In MAT group, the OS is 84.4% in stages I/II, 76.2% in stage III and 55.6% in stage IV. Seventy one patients died during treatment, 32 at pre-induction phase, 27 at induction and 12 at consolidation. Treatment related mortality decreased during the 3-year inclusion period (first year: 25.7%, second year: 19.1%, third year: 11.6%). The improvement of supportive care translated into an increase of the overall survival rates from 54% in the first year to 73% in the third year. CONCLUSION: These data demonstrate the feasibility of prospective multicentric studies in Africa. An improvement of quality of care has been noticed during the 3 first years.

[Splenectomy in hematologic diseases. A study of 70 cases]. / La splenectomie dans les maladies hematologiques. A propos de 70 cas.

The aim of this work was to report our own clinical experience and to compare it our results with those already published. This is a retrospective study of 70 splenectomies performed for hematologic diseases at the service des urgences viscerales of Ibn Rochd teaching hospital of Casablanca, between 1996 and 2001. The study included 70 patients. Splenectomy was performed for therapeutic reasons by laparotomy in 56 cases (80%) and bym laparoscopy in 14 cases (20%). The post-operative after-effects were mild in 81.4%. A patient died, however of pulmonary embolism. Splenectomy is an important tool in the management of patients suffering from hemolytic anemia, auto-immune thrombopenic purpura and other hemolytic anemias. Management by a multidisciplinary team is required to prevent a maximum of long and short-term post-operative complications and to offer the offer the best conditions for a successful splenectomy.

Association of glutathione S-transferase (GSTM1 and GSTT1) genes with chronic myeloid leukemia.

Chronic myeloid leukemia (CML), as most of cancers results from a complex interaction between genetic or non genetic factors. Exposures to xenobiotics endogenous or exogenous associated with a reduced individual ability in detoxifying activity, constitutes a risk of developing cancer. It is known that polymorphism of glutathione S-transferases (GSTs) genes affects the detoxification of xenobiotics. Thus, we conducted a case-control study in which 92 patients (Mean age ± SD, 40.62 ± 12.7 years) with CML and 93 healthy unrelated controls (Mean age ± SD, 41.38 ± 13.4 years) have participated. GSTM1 and GSTT1 genotypes were determined by multiplex polymerase chain reaction. Logistic regression was used to assess the possible link between GSTM1 and GSTT1 null genotypes and CML as well as between combined genotypes and CML. GSTM1 null genotype frequency was slightly higher in patients than control (48.9% vs. 40.9%) but, it was not associated with CML (OR 95% CI, 1.4, 0.78-2.48; p = 0.271). Moreover, GSTT1 null genotype frequency showed a similar trend between patients and control (17.4% vs. 9.7%; OR 95% CI, 1.97, 0.82-4.71; p = 0.13). Surprisingly, GSTT1 null genotype was significantly associated with the risk of CML in males (OR 95% CI, 5, 1.25-20.1; p = 0.023). The combined GSTM1 present/GSTT1 null genotype was found to have a limited effect against the risk of CML (OR 95% CI, 0.3, 0.08-0.99; p = 0.049). Our findings have shown that GSTT1 null genotype might be a risk factor of CML in males. While, GSTT1 present genotype might be considered as protective against CML. However, further studies with a large sample size are needed to confirm our findings.

Acquired factor VII deficiency associated with acute myeloid leukemia.

Isolated acquired factor VII deficiency is a rare coagulopathy. It has been reported in 31 patients with malignancy, sepsis, postoperatively, aplastic anemia, and during bone marrow transplantation. We discuss, through a new case of acquired factor VII deficiency, the characteristics of this disease when it is associated with acute myeloid leukemia. Acquired factor VII deficiency in hematological diseases can be caused by intensive chemotherapy, infections, or hepatic dysfunction. The best treatment in developing countries remains corticosteroids associated with plasma exchange, frozen plasma, and antibiotics.

Hematopoietic stem cell transplantation in the Eastern Mediterranean Region (EMRO) 2011-2012: A comprehensive report on behalf of the Eastern Mediterranean Blood and Marrow Transplantation group (EMBMT).

OBJECTIVE/BACKGROUND: The Eastern Mediterranean Blood and Marrow Transplantation (EMBMT) group has accumulated over 31 years of data and experience in hematopoietic stem cell transplantation (HSCT), particularly in hemoglobinopathies, severe aplastic anemia, inherited metabolic and immune disorders, in addition to a wide array of hematologic malignancies unique to this region. A regional update in current HSCT trends is highly warranted. We studied the trends of HSCT activities in World Health Organization-Eastern Mediterranean (EMRO) region, surveyed by the EMBMT, between 2011 and 2012. METHODS: Retrospective analysis of the survey data mainly of cumulative number of transplants, types of transplants (autologous vs. allogeneic), types of conditioning such as myeloablative versus reduced intensity was conducted. Also, trends in leukemias, hemoglobinopathies, severe aplastic anemia, inherited bone marrow failure syndromes, amongst others were analyzed. RESULTS: Twenty-one teams from nine EMRO countries reported their data (100% return rate) to the EMBMT for the years 2011-2012, with a total of 3,546 first HSCT (1,670 in 2011; 1,876 in 2012). Allogeneic HSCT (allo-HSCT) represented the majority (62%) in both years. The main indications for allo-HSCT were acute leukemias (988; 46%), bone marrow failure syndromes (421, 20%), hemoglobinopathies (242; 11%), and immune deficiencies (157; 7%). There was a progressive increase in the proportions of chronic myeloid leukemia cases transplanted beyond first chronic phase (37 [7%] of all chronic myeloid leukemia cases in 2011 vs. 39 [29%] in 2012). The main indications for autologous transplants were multiple myeloma/plasma cell disorders (510; 39%), Hodgkin lymphoma (311; 24%), non-Hodgkin lymphoma (259; 20%), and solid tumors (163; 12%). Reduced intensity conditioning continued to show a progressive decrease over years (9.5% in 2011 vs. 7.9% in 2012), yet remained relatively low compared with contemporary practices in Europe published by EBMT. The vast majority (91%) of allo-HSCT source was from sibling donors with continued dominance of peripheral blood (64%) followed by bone marrow (33%).While umbilical cord blood transplants increased to 4% of allo-HSCT, matched unrelated donor remained underutilized and there was no haplo-identical transplant reported. Large centers with >50 HSCT/year, showed a continued increase in the total number of allo-HSCT over the past 2years that may be related to capacity building issues and require further studies. CONCLUSION: There is a discernable increase of HSCT rate in the EMRO region with a significant expansion in utilization of cord blood transplants and allogeneic peripheral blood-HSCT as a valuable source. However, further research of outcome data and the development of regional donor banks (cord blood and matched unrelated donors) may help to facilitate future planning to satisfy the escalating regional needs and augment collaboration within the EMBMT and globally.

[Acute myeloblastic leukemia in adults: evaluation of the AML 06/96 protocol]. / Leucémies aigües myéloblastiques de l'adulte: évaluation du protocole AML 06/96.

The treatment of acute myeloid leukemia (AML) permits in a population of 25 to 60 years, a complete remission (CR) about 60 to 85% with relapse free survival at 5 years from 45 to 60%. We report our therapeutic results during two years, from june 1996 to december 1998. 104 patients with the novo AML treated according to AML 06/96 protocol, the mean age was 32.5 years old, from 16 to 55 years old. The hyperleucocytar form (GB > 50,000 elts/mm3) represented the third of the cases, only 98 patients received the induction. 6 patients died before treatment. The whole rate of CR was 55%. The rate of failure was 16%, the deaths was about 15.5%, the relapse represented 30.6% with mean delay about 14.1 months, from 4 to 35 months. The CR has been maintained in 9 patients with mean recession of 53 months, from 36 months to 62 months. The overall survival at 5 years was 9%. Our results are still unsufficient in comparison with the literature and could be improved by recess of ARA-C and donorubicin doses in induction and consolidation, as well as a good knowledge about the cytogenetical aspect of the treated population.

[Germs that produce the extended spectrum betalactamases]. / Les germes producteurs de bêta lactamases à spectre étendu: épidémie dans un service d'Hémato-Oncologie.

This retrospective study analyses an epidemic with germs ESBL that supervenes at the department of hematology and pediatric oncology in UHC Ibn Rochd of Casablanca. The responsible germ is the ESBL Escherichia coli. Six patients have been infected during the same period that 2 are female and 4 are male. Five patients had acute lenkemia, one patient had a non Hodgkin's disease. All the patients were in the stage of a deep postchermotherapy neutropenia. The picture of all the patients represented a severe infection with suffered fever and acute diarrhea. Five patients died with apicture of septic shock in the 48 to 72 hours after the beginning of the infection and before the identification of the germ. Their treatment consisted in the third generation of cephalosporin and aminoside. One patient who use the imipeneme more the aminoside has been apyrexized the epidemic and severe situation led to the closing of the unit during a week in order to do a disinfection. After 12 monthes of recession, few isolate episodes of infections with enterobacteries ESBL have observed and controlled. The factors that determine the increase and the diffusion of the ESBL germ are numerous and some of them are still not identified, the means of prevention consisted in: the fight against the selection of the resistant germs, the fight against the colonization of the patients by these germs and their transmission between the patients, this requires measures of hygiene and particularly the washing of the hands.

[Urogenital lymphoma presenting with obstructive anuria]. / Anurie obstructive révélatrice d'un lymphome uro-génital.

The authors report a case of urogenital lymphoma with multiple sites in a patient presenting with oligo-anuria. Clinical examination revealed a hard hypogastric and prostatic mass and an enlarged left scrotum. Abdominopelvic and scrotal ultrasound demonstrated a prostatic tumour, a hypogastric mass, hepatic nodular lesions and coeliac lymphadenopathy with bilateral ureterohydronephrosis and a heterogeneous intrascrotal mass in contact with the lower pole of the left testis. The laboratory assessment revealed severe renal failure. After a haemodialysis session and ultrasound-guided right percutaneous nephrostomy, pelvic magnetic resonance imaging (MRI) showed a very large pelvic mass between the bladder and the rectum and transrectal biopsy of the mass confirmed the diagnosis of high-grade malignant non-Hodgkin's lymphoma (NHL) with a type B lymphoblastic phenotype. Treatment consisted of chemotherapy according to the LMB 93 protocol. The course was favourable with return of normal renal function and complete remission 1 month after induction. The patient is currently in complete remission with a follow-up of 12 months. In the light of this case and a review of the literature, the authors discuss the diagnostic, therapeutic and prognostic aspects of this rare site of lymphoma.

Primary plasma cell leukemia presenting as a thoracic mass.

Primary Plasma cell leukaemia (pPCL) is a rare plasma cell (PC) malignancy. The strict criteria for the diagnosis is an absolute PC number greater 2 × 10(9)/L or a plasmocytosis accounting for > 20% of the differential white cell count that does not arise from a pre-existing multiple myeloma. pPCL was associated with aggressive clinic-biological features. Primary Plasma cell leukaemia is more characterised by an extra medullar involvement such as hepatomegaly, splenomegaly, lymphadenopathy, lepto-meningeal infiltration or extramedullary plasmocytomas. The prognosis of pPCL is very poor. We report the case of a fifty eight year-old man directed to the haematology department for diagnosis of pPCL revealed by a thoracic plasmocytomas mimicking a thoracic neoplasm. The patient received chemotherapy including a classic treatment for multiple myeloma but developed a pulmonary embolism. This case illustrates an uncommon presentation of pPCL the difficulty treating by multiple myeloma chemotherapy.

Naso-Sinusal T ALL With Aberrant Expression of CD56 Mimicking NK/T Non Hodgkin Lymphoma.

T-acute lymphoblastic leukemia (T-ALL) represents 25 % of cases of acute lymphoblastic leukemia (ALL) in adults. The clinical presentation is dominated by a elevated number of white blood cells and in immunophenotype the lymphoblasts are generally Tdt positive and variably express CD1a, CD2, CD3, CD4, CD5, CD7, and CD8. NK/T non Hodgkin lymphoma is presented as a single lesion often ulcerated with torpid evolution affecting the nasal cavity, nasopharynx and/or palate. CD56 expression is while characteristic of NK-cells, and is also expressed on a subset of normal T cells. Its expression in ALL does not exclude the diagnosis and seems to be a prognostic factor of this disease. We report the case of a young woman with nasal cavity tumor which was initially diagnosed as T-cell lymphoma. This diagnosis was finally revised to conclude to T-ALL with CD56 aberrant expression.

Functional polymorphism of CYP2B6 G15631T is associated with hematologic and cytogenetic response in chronic myeloid leukemia patients treated with imatinib.

In the spite of the impressive results achieved with imatinib in chronic myeloid leukemia (CML) patients, differences in patient's response are observed, which may be explained by interindividual genetic variability. It is known that cytochrome P450 enzymes play a major role in the metabolism of imatinib. The present study aimed to understand the functional impact of CYP2B6 15631G>T polymorphism on the response of imatinib in CML patients and its relation to CML susceptibility. We have genotyped CYP2B6 G15631T in 48 CML patients and 64 controls by PCR-RFLP. CYP2B6 15631G>T was not found to be a risk factor for CML (OR 95 % CI, 1.12, 0.6-2, p > 0.05). Hematologic response loss was higher in patients with 15631GG/TT genotype when compared with 15631GT (36.8 vs. 13.8 %; X (2) = 3.542, p = 0.063). Complete cytogenetic response was higher in 15631GG/GT genotype groups when compared with 15631TT (X (2) = 3.298, p = 0.024). Primary cytogenetic resistance was higher in patients carrying 15631GG/TT genotype when compared with 15631GT carriers (52.6 vs. 17.2 %; X (2) = 6.692, p = 0.010). Furthermore, side effects were more common for patients carrying 15631GG genotypes when compared with GT/TT carriers (36 vs. 13.8 %; X (2) = 8.3, p = 0.004). In light of our results, identification of 15631G>T polymorphism in CML patients might be helpful to predict therapeutic response to imatinib.

[A congenital α2-antiplasmin deficiency]. / À propos d'un déficit constitutionnel en α2-antiplasmine.

Α 2 antiplasmin congenital deficiency is an uncommun fibrinolysis disorder that is diagnostics after a post-operative surgery. We report two brothers cases of α 2 antiplasmin deficiency diagnosed after a prostadenomectomy bleeding at two years intervals.