[Spinal cord compression caused by extramedullary hematopoiesis foci in the course of thalassemia]. / Compression médullaire par des foyers d'hématopoïèse extra médullaire au cours de la thalassémie.

BACKGROUND: Extramedullary haematopoiesis is a physiological response to chronic anemia, observed frequently during homozygous thalassemia. It is usually asymptomatic but can be manifested by compression of adjacent organs, particularly the spinal cord. CASE REPORT: A 44-year-old woman diagnosed with thalassaemia intermedia, was admitted for difficulties to walk and sphincter disturbances. Neurologic examination suggested spinal cord compression, which is confirmed by dorso-lumbar resonance magnetic imaging. The histology obtained by laminectomy led to the diagnosis of extramedullary hematopoiesis related to thalassemia. A radiotherapy enabled with good outcome. DISCUSSION: Spinal cord compressions by extramedullary hematopoiesis during thalassemia are uncommon (75 cases in the literature) but can induce severe sequelae if the diagnostic is not rapidly obtained. Magnetic resonance imaging is the gold standard allowing precise diagnosis and spreading of extramedullary hematopoiesis. Radiotherapy and more recently hydroxyurea are the first line treatment. CONCLUSION: This observation recalls that extramedullary hematopoiesis is a differential diagnostic of spinal cord compression in patients with thalassemia. A screening of paravertebral localization of extramedullary hematopoiesis should be performed in high risk thalassemic patients.

[An unusual tumor of the larynx: malignant fibrous histiocytoma. Apropos of a case. Review of the literature]. / Une tumeur exceptionnelle du larynx: l'histiocytome fibreux malin. A propos d'une observation et revue de la littérature.

On the basis of one case, the authors report about an exceptional tumor of the larynx: malignant fibrous histiocytoma. This tumor, belonging to the soft-tissue sarcomas, usually develops in the limbs. The histological diagnosis is still difficult, in spite of the help provided by immunocytochemistry and fine structural analysis. A histological grading associated to other deciding prognostic factors, such as the size and depth of the tumor and the quality of exeresis, would demonstrate a prognostic correlation. The evolution is characterized by frequent local recurrence and metastases. The treatment is mainly surgical, with as extensive an exeresis as possible at once. Radiation therapy and chemotherapy, though often associated, have not decisively proven their value.