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1.
Bull Cancer ; 2024 Sep 05.
Artículo en Francés | MEDLINE | ID: mdl-39242252

RESUMEN

Bone marrow aplasia is a rare and serious hematologic disorder. Although benign, it is a hematologic disorder whose prognosis can be poor and whose spontaneous development can be fatal. Treatment is long, difficult and costly. In developing countries, the mortality rate is high due to the difficulties of therapeutic management, both supportive and specific. We conducted a retrospective study of 92 cases of AM identified in the Pediatric Hematology and Oncology Department of the 20 Août University Hospital in Casablanca over a 10-year period (January 2010-January 2020). In this work, we present an overview of the situation and highlight the difficulties encountered in the management of AM in the Pediatric Hematology and Oncology Department of the University Hospital of Casablanca. In our study, the mean age was 19 years, ranging from 3 months to 29 years, with a peak in the 15-20 age group. The sex ratio (M/F) was 2.06, with a male predominance of 67%. In our series, only 35% of patients had complete bone marrow failure. An anemic syndrome was present in 92% of patients, and hemorrhagic and infectious syndromes were present in 70% and 41% of patients, respectively. The median time from diagnosis to treatment was 82 days. According to the Camitta score, 31% of our patients had mild AM, 41% had severe AM, and 28% had very severe AM. After etiologic evaluation, we concluded that 90% of the patients had idiopathic bone marrow aplasia, 2% had constitutional bone marrow aplasia, and 8% of the patients were suspected to have secondary bone marrow aplasia: post-hepatitis (3 cases), toxic (2 cases), drug-induced (1 case), and aplastic PNH (1 case). Mortality in the first three months after diagnosis was 21%. Sixty-nine percent of our patients received specific treatment: 28 were treated with cyclosporin (CIS) alone as first-line therapy, 20 received a combination of antilymphocyte serum (ALS) and cyclosporin, 2 received hematopoietic stem cell transplantation (HSCT), while 3 were treated with androgens alone. The overall response rate was 30% with CIS, 42% with ALS+CIS and 100% with HSCT. In our study, the overall death rate was 44%, while the one-year survival rate was 40%. It is important to note that septic shock was the leading cause of death (53% of deaths), followed by hemorrhagic shock (24%). This highlights the lack of hemodynamic resuscitation and symptomatic treatment. Our multivariate study defined the following risk factors as predictive of worse survival: age greater than 16 years (RR: 3.28; CI: 1.29-8.33; P=0.012), PNN less than 200 or very severe bone marrow aplasia (RR: 3.01; 1.1-8.08; P=0.028), and failure to receive any specific treatment (RR: 4.07; 1.77-9.35; P=0.0003). The high overall mortality in our series was due to several factors: inaccessibility to effective therapies, delayed diagnosis, failure to initiate specific treatment, inadequate symptomatic treatment, and geographical and financial inaccessibility.

2.
J Pediatr Hematol Oncol ; 46(5): 217-224, 2024 07 01.
Artículo en Inglés | MEDLINE | ID: mdl-38912833

RESUMEN

Anaplastic large-cell lymphoma is a rare disease and account for approximately 10% to 15% of pediatric non-Hodgkin lymphomas. They are characterized by extended stages, a high frequency of B signs and extra nodal involvement. Multiagent chemotherapy cures ∽60% to 75% of patients and relapse occurs in 35% of cases. For relapsed patients, various treatments ranging from vinblastine monotherapy to therapeutic intensification with hematopoietic stem cell transplantation have been evaluated, but there is currently no consensus on the optimal therapeutic strategy. New therapeutic perspectives are being evaluated for relapses and refractory forms as well as high-risk forms including monoclonal antibodies (Anti CD30), ALK inhibitors, and CART cells.


Asunto(s)
Trasplante de Células Madre Hematopoyéticas , Linfoma Anaplásico de Células Grandes , Humanos , Linfoma Anaplásico de Células Grandes/tratamiento farmacológico , Linfoma Anaplásico de Células Grandes/terapia , Linfoma Anaplásico de Células Grandes/patología , Niño , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico
3.
J Med Case Rep ; 17(1): 537, 2023 Dec 12.
Artículo en Inglés | MEDLINE | ID: mdl-38082322

RESUMEN

BACKGROUND: Acute lymphoblastic leukemia is the most common childhood cancer, with an 80% frequency in children between 1 and 10 years old. The outcome and prognosis of acute lymphoblastic leukemia in children depends on various factors, such as age, clinical and biological features, and cytogenetic factors. CASE PRESENTATION: We report the case of a pediatric patient, a 4-year-old Moroccan female who was referred to the Hematology and Oncology Department of 20 August 1953 Hospital in Casablanca and diagnosed with B-cell acute lymphoblastic leukemia associated with a rare genetic chromosomal abnormality. CONCLUSION: Translocation (1;4)(p21;p15) is a relatively rare chromosomal abnormality found in human leukemia and was never described isolated in pediatric B-cell acute lymphoblastic leukemia patients. It showed a good evolution by complete remission and recovery of this patient after receiving all chemotherapy and after 8 years of follow-up.


Asunto(s)
Leucemia-Linfoma Linfoblástico de Células Precursoras , Translocación Genética , Preescolar , Femenino , Humanos , Aberraciones Cromosómicas , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Pronóstico
4.
J Cancer Res Clin Oncol ; 149(20): 18023-18027, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37979055

RESUMEN

PURPOSE: Peripheral neuroblastic tumors are the most common extracranial cancers found in children, and they are characterized by a diverse spectrum of clinical manifestations and heterogeneous behaviors. This study aimed to investigate the epidemiological and clinical characteristics of children with peripheral neuroblastic tumors admitted to the Department of Pediatric Hematology and Oncology of the Hospital August 20 in Casablanca. METHODS: The medical files of 48 children with peripheral neuroblastic tumors addressed to our department between February 2018 and February 2023 were reviewed. The clinical and demographic characteristics of patients were analyzed by the Statistical Package for the Social Sciences (SPSS), survival curves were obtained by Kaplan-Meier technique, and we assigned the tumor stage to patients based on the International Neuroblastoma Risk Group Staging System (INRGSS). RESULTS: The median age of diagnosis was 30 months (1-174), with a ratio F/M of 1.28. 93.75% of patients had neuroblastoma, and the rest had ganglioneuroma. About 64.6% of patients had at their initial presentations stage M of peripheral neuroblastic tumors. The adrenal region made up 71% of the primary tumor site. The bone was one of the most prevalent metastatic sites (54.2%). The five-year overall survival rate was 35.4%. CONCLUSION: Overall, this study revealed a high stage of peripheral neuroblastic tumors in the majority of the diagnosed patients in our Department of Pediatric Hematology and Oncology. Moreover, the heterogeneity of peripheral neuroblastic tumors makes clinical recognition difficult and, in general, too late.


Asunto(s)
Ganglioneuroma , Neuroblastoma , Niño , Humanos , Neuroblastoma/epidemiología , Neuroblastoma/patología , Ganglioneuroma/epidemiología , Ganglioneuroma/patología , Proteínas Represoras , Tasa de Supervivencia
5.
Clin Case Rep ; 8(12): 2995-2999, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33363866

RESUMEN

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.

6.
Bull Cancer ; 107(9): 861-866, 2020 Sep.
Artículo en Francés | MEDLINE | ID: mdl-32863000

RESUMEN

INTRODUCTION: The treatment of chronic myeloid leukemia (CML) has been revolutionized by the advent of tyrosine kinase inhibitors. The results of the IRIS trial demonstrated the efficacy and long-term safety profile of Imatinib. The objective of our work is to report the results at 15 years of treatment of CML in chronic phase with Imatinib in Morocco. PATIENTS AND METHODS: Retrospective study realized at the hematology unit of CHU d'Ibn-Rochd in Casablanca, from January 2003 to September 2018, including all CML patients in the chronic phase at diagnosis, were treated with Imatinib for a minimum duration of 6 months. RESULTS: In total, 318 patients were collected, the median age was 41.5 years, the sex ratio M/F was 0.7, the Sokal score was high in 56% of cases. The complete hematological response at 3 months was 92%, the complete cytogenetic response at 12 months and the cumulative response were obtained in 43% (29/67) and 55% (153/279) of the cases respectively, the molecular response was evaluated in 125 patients witch 85% were on major molecular response. On a median follow-up of 44 months, the OS and EFS at 10 years were 86% and 59%, respectively. DISCUSSION: Our profile is characterized by a young age of the patients, the female predominance and a high Sokal score. The rate of complete cytogenetic response remains lower compared to what is described, however the survival rates as well as the tolerance were similar to those of the literature.


Asunto(s)
Mesilato de Imatinib/uso terapéutico , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Inhibidores de Proteínas Quinasas/uso terapéutico , Adulto , Femenino , Humanos , Masculino , Marruecos , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento
7.
Cancer Genet ; 238: 1-9, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-31425919

RESUMEN

BACKGROUND: We analyzed the cytogenetic characteristics of a representative population of young adults with de novo acute myéloblastic leukemia (AML) treated in a single center institution. METHODS: Patients with de novo AML included were aged between 20 and 60 years. Cytogenetic analysis was done at diagnosis. Twenty cells were analyzed, although examination of lower numbers of metaphases was also acceptable if an abnormal clone was detected. FINDINGS: From 1/1/04 to 31/12/2014, among 1315 adult patients, 1055 (80%) patients were aged between 20 and 60 years. Karyotype was done in 927 (88%) patients and cytogenetic analysis failed in 32 cases (3·4%). Clonal abnormalities were observed in 520 (58%) patients. 175 (19·5%) were classified in the favorable group, 609 were in the intermediate group and 111 (12·5%) were in the adverse group. The most frequent chromosomal abnormality observed was t(8;21) in 112 (12·5%). Thirty three (3·7%) patients had t(15;17) and 30 (3·3%) had inv16, trisomy 8 was found in 47 (5·2%), 11q23 rearrangements in 32 (3·6%), 67 (7·4%) had a complex karyotype, -5/del(5q) and -7/del(7q) were seen in, respectively, 11(1%) and 27 (3%). Monosomy occurred in 115 (13%) patients, 70 (8%) responded to the definition of monosomal karyotypes. INTERPRETATION: This is the largest study done in Morocco, Africa and Middle East. Epidemiological studies involving different ethnic populations and geographic regions of the world should help unfold the true nature of environmental and genetic interplay in the development of AML. Our cytogenetic profile reveals some particularities when compared to other populations; it does not seem to be similar neither to eastern or western countries.


Asunto(s)
Aberraciones Cromosómicas , Leucemia Mieloide Aguda/genética , Adolescente , Adulto , Estudios de Cohortes , Femenino , Humanos , Cariotipificación , Masculino , Persona de Mediana Edad , Marruecos
8.
Open Access Maced J Med Sci ; 6(12): 2328-2332, 2018 Dec 20.
Artículo en Inglés | MEDLINE | ID: mdl-30607185

RESUMEN

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.

9.
Open Access Maced J Med Sci ; 6(12): 2373-2375, 2018 Dec 20.
Artículo en Inglés | MEDLINE | ID: mdl-30607194

RESUMEN

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.

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