[ « Le syndrome 5Q-» : A propos d’un cas avec revue de la littérature ]
Volume 29, Issue 3, June 2020, Pages 396–399
ASMA ALLALI1, TAOUFIK ROKNI2, MARYAM MOUAMIN3, Hicham Yahyaoui4, ABDERRAHIM RAISSI5, Mustapha Ait Ameur6, and MOHALED CHAKOUR7
1 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
2 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
3 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
4 Professeur en biologie médicale, Laboratoire d’hématologie, Hôpital militaire Avicenne, Marrakech, Morocco
5 Service d’Hematologie Clinique, Hopital Militaire Avicenne, Marrakech, Morocco
6 Professeur en biologie médicale, Laboratoire d’hématologie, Hôpital militaire Avicenne, Marrakech, Morocco
7 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
Original language: French
Copyright © 2020 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
5q -syndrome is a unique paradigm of myelodysplastic syndrome (MDS) characterized by the correlation between cytology and cytogenetics and the low risk of clinical progression. It is a category apart in myelodysplastic syndromes (MDS) because of its hematological peculiarities, its pathophysiology which remains unknown, and the refractoriness of the anemia with which it is associated. It is a pathology that affects the female sex with predilection. The prognosis of the disease remains good and the risk of malignant transformation into acute leukemia does not exceed 15%. Through this work, we report a typical case of SMD with deletion of the long arm of chromosome 5 in order to describe the observation of this particular entity diagnosed in our formation, rare in our Moroccan context and we must know how to evoke before refractory anemia most often isolated.
Author Keywords: MDS, 5q-, cytology, cytogenetic, prognosis, MLA.
Volume 29, Issue 3, June 2020, Pages 396–399
ASMA ALLALI1, TAOUFIK ROKNI2, MARYAM MOUAMIN3, Hicham Yahyaoui4, ABDERRAHIM RAISSI5, Mustapha Ait Ameur6, and MOHALED CHAKOUR7
1 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
2 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
3 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
4 Professeur en biologie médicale, Laboratoire d’hématologie, Hôpital militaire Avicenne, Marrakech, Morocco
5 Service d’Hematologie Clinique, Hopital Militaire Avicenne, Marrakech, Morocco
6 Professeur en biologie médicale, Laboratoire d’hématologie, Hôpital militaire Avicenne, Marrakech, Morocco
7 Laboratoire d’Hematologie, Hopital Militaire Avicenne, Marrakech, Morocco
Original language: French
Copyright © 2020 ISSR Journals. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
5q -syndrome is a unique paradigm of myelodysplastic syndrome (MDS) characterized by the correlation between cytology and cytogenetics and the low risk of clinical progression. It is a category apart in myelodysplastic syndromes (MDS) because of its hematological peculiarities, its pathophysiology which remains unknown, and the refractoriness of the anemia with which it is associated. It is a pathology that affects the female sex with predilection. The prognosis of the disease remains good and the risk of malignant transformation into acute leukemia does not exceed 15%. Through this work, we report a typical case of SMD with deletion of the long arm of chromosome 5 in order to describe the observation of this particular entity diagnosed in our formation, rare in our Moroccan context and we must know how to evoke before refractory anemia most often isolated.
Author Keywords: MDS, 5q-, cytology, cytogenetic, prognosis, MLA.
Abstract: (french)
Le syndrome 5q - est un paradigme unique de syndrome myélodysplasique (SMD) caractérisé par la corrélation entre la cytologie et la cytogénétique et par le faible risque d’évolutivité clinique. Il s’agit d’une catégorie à part dans les syndromes myélodysplasiques (SMD) en raison de ses particularités hématologiques, de sa physiopathologie qui reste méconnue, et du caractère réfractaire de l’anémie à laquelle il est associé. C’est une pathologie qui touche avec prédilection le sexe féminin. Le pronostic de la maladie reste bon et le risque de transformation maligne en leucémie aigue ne dépasse pas les 15%. A travers ce travail, Nous rapportons un cas typique de SMD avec délétion du bras long du chromosome 5 dans le but de décrire l’observation de cette entité particulière diagnostiquée dans notre formation, rare dans notre contexte marocain et qu’il faut savoir évoquer devant une anémie réfractaire le plus souvent isolée.
Author Keywords: SMD, 5q-, cytologie, cytogénétique, pronostic, LAM.
How to Cite this Article
ASMA ALLALI, TAOUFIK ROKNI, MARYAM MOUAMIN, Hicham Yahyaoui, ABDERRAHIM RAISSI, Mustapha Ait Ameur, and MOHALED CHAKOUR, “«5Q- syndrom» : A case report with review of the literature,” International Journal of Innovation and Applied Studies, vol. 29, no. 3, pp. 396–399, June 2020.
