[Incidence and risk factors of exposure keratopathy in intensive care units: About 91 patients]. / Incidence et facteurs de risque des kératopathies d'exposition en milieu de réanimation : à propos de 91 patients.

Exposure keratopathy is a sight-threatening complication which can occur in patients admitted to intensive care units. This is a prospective study over a period of 5 months in the intensive care unit of the specialty hospital of the IBN SINA medical center, with a capacity of 8 beds. The purpose of our study was to determine the incidence of and risk factors for exposure keratopathy in intensive care settings. Forty-two percent of patients developed exposure keratopathy, 48% exhibited chemosis, and 40% showed lagophthalmos with the cornea visible in 30% of cases. The frequency of keratopathy was significantly higher in patients with chemosis and lagophthalmos (P<0.0001). Lagophthalmos was significantly related to chemosis (P<0.0001). Chemosis in the ventilated patient, also known as "ventilator eye," is a serious complication leading to the risk of keratopathy. The risk factors for keratopathy found in our series were chemosis (OR=25.28, 95% CI=[3.339-191.52] P-value=0.002), lagophthalmos (OR=40.8, 95% CI=[4.347-383.666] P-value=0.001) and length of stay in intensive care (OR=12.28, 95% CI=[1.476-102.230] P-value=0.020). Several methods might be used and adapted to each case for prevention of exposure keratopathy, and we found that raising nursing staff awareness is of paramount importance.

First report of pediatric hematopoietic stem cell transplantation activities in the eastern mediterranean region from 1984 to 2011: on behalf of the pediatric cancer working committee of the eastern mediterranean blood and marrow transplantation group.

To describe the hematopoietic stem cell transplantation (HSCT) activities for children in the Eastern Mediterranean (EM) region, data on transplants performed for children less than 18 years of age between 1984 and 2011 in eight EM countries (Egypt, Iran, Jordan, Lebanon, Oman, Pakistan, Saudi Arabia and Tunisia) were collected. A total of 5187 transplants were performed, of which 4513 (87%) were allogeneic and 674 (13%) were autologous. Overall, the indications for transplantation were malignant diseases in 1736 (38.5%) and non-malignant in 2777 (61.5%) patients. A myeloablative conditioning regimen was used in 88% of the allografts. Bone marrow (BM) was the most frequent source of stem cells (56.2%), although an increasing use of PBSC was observed in the last decade. The stem cell source of autologous HSCT has shifted over time from BM to PBSC, and 80.9% of autologous HSCTs were from PBSCs. The donors for allogeneic transplants were matched-related in 94.5% of the cases, and unrelated transplants, mainly cord blood (99%) in 239 (5.5%) cases. This is the first report to describe the pediatric HSCT activities in EM countries. Non-malignant disorders are the main indication for allogeneic transplantation. Frequency of alternate donor transplantation is low.

Quantitative real-time polymerase chain reaction as an efficient molecular tool for detecting minimal residual disease in Moroccan chronic myeloid leukemia patients.

Chronic myeloid leukemia (CML) is characterized by BCR-ABL translocation and an increased number and migration of immature myeloid cells into the peripheral blood. The detection limit of the BCR-ABL transcript, particularly after treatment, is controversial. In the present study, we used quantitative real-time reverse transcription-polymerase chain reaction (RT-qPCR) to monitor BCR-ABL expression in Moroccan CML patients undergoing imatinib treatment, and compared the results with those of conventional PCR and fluorescence in situ hybridization (FISH). The aim of this study was to establish a new molecular tool for in vitro diagnosis of CML. In a retrospective comparative analysis, 20 CML Moroccan patients who had received imatinib treatment (N = 20) were analyzed by real-time PCR, conventional RT-PCR, and FISH. Half of the samples analyzed (N = 10) were positive for BCR-ABL gene expression, while the other half (N = 10) were negative according to conventional PCR. Interestingly, 5 of the 10 samples shown to be negative by conventional PCR showed positive expression of the BCR-ABL gene according to RT-qPCR. The RT-qPCR results were confirmed by FISH, which revealed a high concordance (100%) rate. We found that real-time RT-qPCR is more reliable and should be used in Moroccan biomedical analysis laboratories to monitor CML progression, particularly for minimal residual disease, following imatinib treatment.

Effect of interaction of glutathione S-transferases (T1 and M1) on the hematologic and cytogenetic responses in chronic myeloid leukemia patients treated with imatinib.

The glutathione S-transferases (GSTs) are phase II xenobiotic metabolizing enzymes known to be involved in the detoxification of carcinogens and anticancer drugs. Individual genetic variation linked to inherited polymorphisms of GSTT1 and GSTM1 leading to a complete loss of enzyme activity could expose subjects to develop cancer or to induce drug resistance. Indeed, despite the impressive results obtained with the imatinib, some patients with chronic myeloid leukemia (CML) fail to achieve the expected results or develop resistance. The present study aimed to examine the impact of GSTT1 and GSTM1 polymorphisms on the response to imatinib in patients with CML. Multiplex polymerase chain reaction was used to detect the genotypes of GSTT1 and GSTM1 in 60 CML patients. We found that side effects were more frequent in patients carrying GSTT1 null when compared to GSTT1 present carriers (31 vs. 16.6 %; χ (2) = 6.2; p = 0.013). The loss of hematologic response was statistically greater in patients carrying the combined genotype GSTT1 present/GSTM1 present (26.3 %) when compared to GSTT1 null/GSTM1 present (12.8 %), GSTT1 present/GSTM1 null (8.3 %) and GSTT1 null/GSTM1 null (0 %), (χ (2) = 18.85; p < 0.001). The complete cytogenetic response was higher in patients harboring the GSTT1 null/GSTM1 null (75 %) compared with GSTT1 null/GSTM1 present (55.6 %), GSTT1 present/GSTM1 null (50 %) and GSTT1 present/GSTM1 present (47.8). On the other hand, the frequency of none cytogenetic responders was more common in patients carrying GSTT1 present/GSTM1 present (34.8 %) when compared to other genotype combinations (χ (2) = 20.99; p = 0.05). Moreover, the GSTT1 present/GSTM1 present appeared to be associated with a final dose of 600 or 800 mg of imatinib, but not significantly. Based on these findings, we find that the interaction between GSTT1 and GSTM1 seems to influence treatment outcome in patients with CML. Therefore, further investigations are required to confirm these results, for better genotype-phenotype correlation.

[Retinoblastoma: preliminary results of national treatment protocol at Casablanca university medical center]. / Rétinoblastome : résultats préliminaires du protocole national de prise en charge au CHU de Casablanca.

Retinoblastoma is the most frequent intraocular cancer, affecting almost exclusively children. We report prospective study results assessing the national protocol for retinoblastoma treatment in Morocco. Treatment included, depending on stage and laterality, primary chemotherapy either to facilitate enucleation or to make conservative treatment possible, postoperative chemotherapy, enucleation and conservative treatments such as transpupillary thermotherapy, thermochemotherapy and cryotherapy. Radiation was used in a few cases. Close supervision was performed until the age of 5. The incidence of retinoblastoma within the study period was 18 new cases per year in our department. Observations of 32 children were included in the study: 18 unilateral retinoblastomas (56%) and 14 bilateral retinoblastomas (44%), for a total of 46 eyes. Leucocoria was the most frequent presenting symptom (69%). Buphthalmia or proptosis were present in 47% of cases. The stage of retinoblastoma was V/D or E (Reese-Elsworth/ABC) in 69.5% of cases. Enucleation was necessary for 28 eyes. Transpupillary thermotherapy or thermochemotherapy were used for 13 eyes (11 children) and cryotherapy for 13 eyes (10 children). After an average follow-up period of 52 months, among 32 children, 4 died and 2 abandoned treatment. Ocular salvage rate was 85.7% (12 eyes out of 14, among which 11 without radiation). Retinoblastoma is a genetic tumor, which occurs in two forms: sporadic, always unilateral, and hereditary, often bilateral. The latter is the most challenging case. Current treatment protocols rely primarily on chemotherapy and local treatments. The future is oriented toward purely local treatments such as intra-arterial chemotherapy and intraocular chemotherapy.

Clinical importance of myeloid antigen expression in Moroccan patients with adult B-lineage acute lymphoblastic leukemia.

The prognostic significance of myeloid antigen (MyAg) expression in acute lymphoblastic leukemias (ALL), especially in adult patients, is still controversial. In the present report, frequency and clinical significance of MyAg (CD13 and/or CD33) in blast cells were assessed in 80 consecutive adult (≥18 years) patients with B-lineage acute lymphoblastic leukemia (B-ALL), representing 66.7% of 120 patients diagnosed as having ALL during the study period. Immunophenotyping was used to classify leukemic cells as Bor Tlymphoblasts and to identify the aberrant expression of myeloid-associated antigens. MyAg expression was documented in 52.5% of the 80 B-ALL cases analyzed. CD13 was the most commonly antigen expressed (36.3%) followed by CD33 (28.8%). No significant associations were found between the expression of MyAg and the presence of known adverse prognostic features (eg: age>30 years, male gender, high WBC count and Philadelphia positivity). Also, we failed to observe any statistically significant difference between MyAg-positive and MyAg-negative patients in terms of achievement of complete remission and overall survival at 3 years. This study demonstrates that the presence of MyAg on lymphoblastic cells lacks prognostic value In Moroccan patients with adult B-ALL.

[Disseminated fusariosis during autologous stem cells transplant]. / Fusariose disséminée au cours d'une autogreffe de moelle osseuse.

Fusarium is a filamentous brown fungus found in soil, on plants and outdoors responsible for localized or disseminated infections. Diagnosis is based on blood cultures and skin biopsy. Disseminated fusariosis is a rare and serious fungal infection, that occurs especially in neutropenic immunosuppressed patients. Treatment is difficult and mortality is estimated between 50 and 70% in adult patients. This infection is rare in Morocco. We report a case of systemic fusariosis in patient with multiple myeloma during a second autologous stem cell transplant. At day 4 of the autologous stem cells transplant the patient had febrile neutropenia and diarrhea; he received ceftazidime, metronidazole and amikacin for 2 days. The patient still febrile was treated by imipenem and vancomycin without bacteriological proof. At day 10 the patient presented difficulty of breathing and wheezing on auscultation of the lungs, and received nebulization with salbutamol every 6 hours. The CT scan shows interstitial infiltrate of the right lung with micronodules. At day 11 he was treated by voriconazole with clinical improvement. At day 19, Fusarium sp. was identified on the Sabouraud blood culture. The patient left the transplant unit at day 25, he received 6 weeks of voriconazole with clinical and radiological improvement.

[Successive cutaneous adverse reactions to nilotinib and imatinib in a single patient]. / Toxidermie à l'imatinib suivie d'une toxidermie au nilotinib.

BACKGROUND: Since the advent of targeted molecules, the treatment and prognosis of many cancers, especially chronic myeloid leukemia (CML), have been substantially modified through the introduction of first- and second-generation tyrosine kinase inhibitors. Skin effects constitute the most common adverse effects of these new substances. Although such skin changes are not life-threatening, they can have extensive clinical impact, in some cases leading to discontinuation of treatment. PATIENTS AND METHODS: A 47-year-old woman with no past medical history was followed for chronic phase CML since 26/11/2010 with the presence of the t(9; 22) karyotype. Imatinib (IM) was started at a dose of 400mg/day and haematological response was good. After 4 months of treatment with IM the patient presented with erythematous plaques on both upper limbs and on the oral and vaginal mucosa. These lesions disappeared after discontinuation of IM. The patient was then put on nilotinib 400mg/d and skin lesions reappeared after 3 weeks in the more serious form of erythema multiform with acral distribution, but with no involvement of the mucosa, resulting in immediate cessation of nilotinib. Skin biopsy was consistent with a drug-induced eruption. The lesions disappeared after discontinuation of nilotinib. DISCUSSION: In case of intolerance to IM, a second-generation ITK (dasatinib or nilotinib) may be substituted, and while cross-sensitivities seem infrequent, therapy is problematic in these patients presenting potentially curable blood dyscrasias.

A rare association of Castleman's disease and nephrotic syndrome.

Castleman's Disease (CD) is an uncommon and poorly understood disorder of lymph node hyperplasia of unknown etiology. This entity belongs to the atypical lymphoproliferative disorders, a heterogeneous group of diseases characterized by a hyperplastic reactive process involving the immune system. The association of the nephrotic syndrome and CD is extremely rare and their interrelation remains enigmatic. We report a case of CD of the hyaline-vascular type with unicentric localization complicated by nephrotic syndrome.

[Askin tumor: a case report with literature review]. / Tumeur d'Askin: à propos d'un cas avec revue de la littérature.

Askin tumor is a rare malignant tumor arising from soft tissues of the chest wall, rarely in the lung. It occurs predominantly in young adults. It still raises many questions about its individualisation and its links with Ewing's sarcoma. We report a case of Askin tumor in a 5-year-old child with reviewing the different data from the literature.

Special issues related to hematopoietic SCT in the Eastern Mediterranean region and the first regional activity report.

Although several centers are now performing allogeneic hematopoietic SCT (HSCT) in the Eastern Mediterranean (EM) region, the availability is still limited. Special issues including compatible donor availability and potential for alternative donor programs are discussed. In comparison to Europe and North America, differences in patterns of diseases and pre-HSCT general status, particularly for patients with BM failure, are described. Other differences including high sero-positivity for CMV, hepatitis B and C infection, and specific observations about GVHD and its relation to genetically homogeneous communities are also discussed. We report that a total of 17 HSCT programs (performing five or more HSCTs annually) exist in 9 countries of the EM region. Only six programs are currently reporting to European Group for Blood and Marrow Transplantation or Center for International Blood and Marrow Transplantation Research. A total of 7617 HSCTs have been performed by these programs including 5701 allogeneic HSCTs. The area has low-HSCT team density (1.56 teams per 10 million inhabitants vs 14.43 in Europe) and very low-HSCT team distribution (0.27 teams per 10 000 sq km area vs <1-6 teams in Europe). Gross national income per capita had no clear association with low-HSCT activity. Much improvement in infrastructure and formation of an EM regional HSCT registry are needed.