Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 20 Moroccan adult patients.

OBJECTIVE: The objective of this study was to describe the clinical and laboratory manifestations, triggers factors, treatment, and outcome of MAS complicating SLE. METHODS: We retrospectively analyzed the medical records of adult patients with SLE for a period of 8 years (2009-2016) and identified patients who had developed MAS. We conducted statistical analysis to identify factors associated with MAS. RESULTS: Among 208 consecutive lupus patients, 20 patients (19 women) were identified having MAS. The mean age of patients was 35.4 ± 10 years. MAS revealed lupus in 7 patients. In the others, the delay between diagnosis of SLE and MAS was 33,3 months. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. An anemia (hemoglobin < 10 g/dL) was found in all patients. A thrombopenia was observed in 19 (95%) cases. Hypertriglyceridemia and hyperferritinemia were present in all patients. All patients had anti-nuclear antibodies and anti-double-stranded DNA antibodies. Bone marrow aspiration showed hemophagocytosis in 15 (94%) cases. The mean SLEDAI was 20.95 corresponding to an SLE of a very high activity. The mean H-Score was 233.85. MAS was associated with a lupus flare in 13 patients. Documented bacterial infections, viral infections, and a breast cancer were respectively diagnosed in 4, 3, and 1 cases respectively. The corticosteroids were administered in all patients. Intravenous cyclophosphamide was used together with corticosteroids in 6 patients, mycophenolate mofetil in 2 cases and azathioprine in 2 cases. Intravenous immunoglobulin was given in 4 cases, etoposide in one case and rituximab was used as the third line treatment in one patient. All infectious episodes were also treated by broad spectrum antibiotics. All patients had a good outcome without any mortality at the management, with a mean follow-up of 24 months. The clinical parameters significantly associated with MAS were fever (p = 0,001), splenomegaly (p < 0.0001), lymphadenopathy (p < 0.0001), oral and/or nasopharyngeal ulceration (p = 0.04), arthritis (p = 0.017), and pulmonary signs (p = 0.003). Laboratory parameters associated with MAS were anemia (p < 0.0001), thrombopenia (p < 0.0001), hyperferritinemia (p < 0.0001), hypertriglyceridemia (p < 0.0001), SLEDAI (p < 0.0001), and H-Score (p < 0.0001). Receiver operating characteristic (ROC) analysis identified optimal cutoff values of ferritin (> 695 ng/mL) and SLEDAI (> 13.5) to predict the occurrence of MAS in SLE. CONCLUSION: MAS was observed in 9.62% Moroccan adult patients with SLE. SLE flare and infection were the common triggers of MAS in our study. Our study indicates that the occurrence of unexplained fever, splenomegaly, lymphadenopathy, profound cytopenia, hyperferritinemia, hypertriglyceridemia, high SLEDAI, and H-Score should raises the possibility of the diagnosis of MAS in SLE patients. Early diagnosis and urgent therapeutic management improves the overall prognosis. Key Points • Macrophage activation syndrome (MAS) is an underdiagnosed complication of systemic lupus erythematosus (SLE). The prevalence of this complication in this study is nearly 10%. • The diagnosis of MAS represents a major challenge for clinicians, as it could mimic a SLE flare up or be confused with infections. Validated diagnostic criteria for MAS in adults secondary to SLE are urgently needed. • In this study, the H-score calculate the individual risk of adult patients having reactive MAS. The cut-off value for the H-score was 190.5 (sensitivity 96.7%, specificity 97.6%). • The prognosis of MAS with SLE is good in our study. However, in the literature MAS may be a fatal condition in SLE patients. Prospective studies are necessary to confirm these results.

Quality of life of Moroccan patients on the palliative phase of advanced cancer.

OBJECTIVE: The aim of this study is to assess the quality of life of caregiver's. The study was conducted at the RABAT National Institute of Oncology in MOROCCO. RESULTS: 120 patients on the palliative phase of advanced cancer were included. Severe fatigue was observed in 64.2% of patients with an average of 90.55 ± 14.7. There was a positive association between functional dimensions and overall quality of life and a negative association between symptoms and overall quality of life. Patients under 30 years had a lower quality of life. According to the multi-varied analysis, physical function, emotional functioning and fatigue were significant predictors of Health related quality of life/overall quality of life (p < 0.05).

[Epidemiological, clinical, cytologic and immunophenotypic aspects of acute leukemia in children: the experience at the hematology laboratory of IBN SINA University Hospital Center]. / Aspects épidémiologiques, cliniques, cytologiques et immunophénotypiques des leucémies aiguës chez les enfants: expérience du laboratoire d'hématologie du Centre Hospitalier Universitaire IBN Sina.

The aim of this study was to describe epidemiological, cytologic and immunophenotypic aspects of acute leukemias (AL) in children diagnosed at IBN SINA University Hospital Center and to determine the concordance between cytology and immunophenotyping results. This is a cross-sectional study conducted in the hematology laboratory of IBN SINA University Hospital Center between June 2012 and May 2014. Among the 104 cases with diagnosed AL, 52% were boys with a sex-ratio H/F= 1.32, the average age was 5.7 years. The distribution of different types of AL was: lymphoid AL (LAL) (74%), myeloid (AML) (20.2%), biphenotypic AL (BAL) (65.8%). Among the LALs, 78% were classified as B LAL and 22% as T LAL. Clinical signs were mainly presented with tumor syndrome (73.1%), fever (61%) and hemorrhagic syndrome (50%). The most common blood count abnormalities were: thrombopenia (89.4%), anemia (86.5%), hyperleukocytosis (79.8%). The rate of peripheral and bone marrow blasts was statistically higher for LAL than for AML and BAL (p <0.001). The rate of relapse and mortality was 21.2% and 16. 3% respectively. Concordance rate between the results of cytology and of immunophenotyping was 92.7% for LAL and 82.6% for AML. Diagnosis of AL is always based primarily on cytology. Immunophenotyping allowed us to make a better distinction between acute leukemias. The management of paediatric AL is a major health problem which requires specialized care centers.

Cost of Treatment of Cerebral Aneurysm Embolization: Study of Associated Factors.

BACKGROUND: Surgical clipping or endovascular coiling are the main procedures used in the treatment of cerebral aneurysms, with a preference for endovascular coiling. In Morocco, the number of patients needing endovascular coiling is growing, but many of them do not have access to this technique. The aim of this study was to determine the main parameters associated with variations in the total cost of this procedure in order to establish the amount (lump sum) that may be reimbursed by health insurance funds. METHODS: One hundred and seventeen patients with 124 aneurysms were admitted for treatment of one or more intracranial aneurysms between January 2010 and December 2015. The overall cost of hospitalization was assessed by using the micro-costing technique. The calculation was based on the tariffs of medical procedures as defined by the Ministry of Health in Morocco. A regression analysis was used to define the correlation between the overall cost and the various parameters. RESULTS: Univariate linear regression showed that the total cost was influenced by overall duration of hospitalization, ICU duration of hospitalization and size of aneurysm. On the other hand, univariate linear regression showed that the total cost was not influenced by sex, localization of aneurysm, and size of the aneurysm's neck. However, multivariate linear regression showed that the total cost was influenced by one type of insurance health, overall duration of hospitalization, ICU duration of hospitalization, size of the aneurysm, and size of the aneurysm's neck. CONCLUSION: Setting a rate for reimbursement of patients who have had coiling treatment for a cerebral aneurysm should take into account the results of our study in order to limit the costs borne by patients. The parameters that influence the overall cost must be reimbursed in each case while the parameters that do not influence treatment costs could be included in a lump sum.

Smoking, passive smoking and lung cancer cell types among women in Morocco: analysis of epidemiological profiling of 101 cases.

BACKGROUND: Recently women's lung cancer mortality rates have dramatically increased in developed countries, contrasting with a levelling off or decrease among men. Descriptive epidemiological data on primary lung cancer in women is scarce in Morocco. The aim of this study, conducted in the National Institute of Oncology in Rabat, was to describe the epidemiological profiling especially for the smoking status, to determine the most frequent type of lung cancer, and to analyse the survival of Moroccan women with lung cancer diagnosis. RESULTS: We found 101 women among 1680 (male and female) cases of lung cancer. The never-smokers were estimated to 75 %. The proportion of adenocarcinoma among never and passive smokers was higher than that of squamous cell carcinoma (SCC) (69.4 versus 30.6 %), while among women who were smokers, the most frequent histological type was SCC (63.6 %). The Cox regression analysis showed that smoking and passive smoking were not significantly associated with survival [HR: 0.62 (95 % CI 0.31, 1.30); p = 0.19] [HR: 0.56 (95 % CI 0.29, 1.08); p = 0.08] respectively. Adenocarcinoma was significantly associated with shorter survival [HR: 1.73 (95 % CI 1.05, 2.85); p = 0.03]. CONCLUSIONS: The majority Moroccan women affected by lung cancer have never smoked (75 %). Environmental exposures, genetic predisposition, hormonal factors, and viral infection may all play a role in lung cancer in this category. The relation between histological type and tobacco found in our series concurred with those reported in the literature--adenocarcinoma appears to be the most frequent cell type affecting never and passive smokers. Adenocarcinoma is significantly associated with poorer survival.