Polycythemia Vera Masked by Megaloblastic Anemia.

Polycythemia vera (PV) is a chronic myeloproliferative disorder characterized by uncontrolled red blood cell production. Megaloblastic anemia is caused by deficiency of cobalamin (vitamin B12) and/or folate (vitamin B9). While B12 deficiency may be caused by insufficient dietary intake or impairment of its utilization, its association with PV is described without exact knowledge of the physiopathology. We herein report the occurrence of megaloblastic anemia due to Vitamin B12 deficiency in an 85-year-old North African woman patient with PV. This case highlights this atypical presentation of PV and challenges that comes with it causing the delay of diagnosis and the complexity of its diagnosis and treatment. Keywords: megaloblastic anemia, polycythemia vera, association, case report.

[Non-Hodgkin cardiac lymphomas]. / Les lymphomes non-Hodgkiniens cardiaques.

Cardiac non-Hodgkin lymphoma (CNHL) is a rare extranodal lymphoma with a poor prognosis. Secondary cardiac lymphoma is more frequent than primary cardiac lymphoma. CNHL often involves the right side of the heart. Diagnosis of CNHL can be suggested by echocardiography and magnetic resonance imaging. Cytological examination of pericardial fluid or histological of cardiac tissue is essential. The majority of cases are diffuse B-cell lymphoma. A prompt immediate diagnosis and early treatment are essential for better outcome. Management should be individualized and the most effective treatment is chemotherapy containing anthracycline variably combined with radiotherapy. A multidisciplinary approach is essential to the successful treatment of CNHL.

[Nephroblastoma and xeroderma pigmentosum: A rare association]. / Xeroderma pigmentosum et néphroblastome : une association rare.

INTRODUCTION: Xeroderma pigmentosum (XP) is a rare, genetically heterogeneous, autosomal recessive disorder, more common in cases of consanguinity. The basic defect underlying the clinical manifestations is a nucleotide excision repair defect leading to the defective repair of DNA damaged by ultraviolet (UV) radiation. XP is characterized by a high incidence of skin cancer on exposed regions. CASE REPORT: We report the case of a 5-year-old boy, followed for xeroderma pigmentosum since the age of 4 years. His sister also has the same anomaly. He presented an abdominal mass revealed by abdominal pain and vomiting. Radiological examinations revealed a nephroblastoma with lung metastases. He received primary chemotherapy for six cycles (vincristine, and actinomycin-d adriamycin), then surgery with ureteronephrectomy. Pathological examination of the nephrectomy specimen confirmed the diagnosis of Wilms tumor with a diffuse anaplastic component reaching 50%. The patient was treated according to the GFAOP stage III protocol, with high histological risk. The outcome was favorable but complicated by renal failure due to the toxicity of the treatment. He is currently in complete remission at 1 year from the end of treatment. CONCLUSION: The association of xeroderma pigmentosum and nephroblastoma is a rare combination. This case illustrates the problem of management of both severe and difficult conditions.

[Assessment of transfusion practice: assessing nurses' knowledge in transfusion medicine at Mohamed VI Hematology and Oncology Center of Marrakesh, Morocco]. / Évaluation de la pratique transfusionnelle : enquête au sein du personnel infirmier du centre d'oncologie-hématologie du CHU Mohammed VI de Marrakech, Maroc.

UNLABELLED: Blood transfusion is a complex activity, involving many actors. It is a high-risk activity which could not be controlled without the use of specific methods. Health care workers beliefs and organizational factors are two major issues for the blood transfusion safety. PURPOSE: In our medical center, transfusion medicine care practices were evaluated by testing the nursing staff with a list of questions. We carried out a cross-sectional study. The information was gathered by using an anonymous questionnaire. The latter was developed by foreign teams and adapted to the local context. Forty-two nurses have participated to study. Only 25% have appropriate knowledge and practice with no negative consequences for the patient safety. In our sample, poor knowledge and practice concerned mainly (1) pre-transfusion compatibility check when receiving blood units (30%); (2) delay in preservation of blood unit in the ward (65%); and (3) recognition of abnormal reactions after transfusion (40%). These results showed on which topics the teaching program should emphasize so as to improve the quality of blood transfusion in the medical centers according to legal obligations.