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New classification for myelodysplasia
In order to properly treat myelodysplasia syndrome, Down syndrome–related diseases have been reclassified as a separate entity.
Sharon M Castellino, MD, FAAP Wake Forest University Health Sciences
Timothy P Cripe, MD, PhD Cincinnati Children’s Hospital Medical Center
Scott C Howard, MD Cincinnati Children’s Hospital Medical Center
The myelodysplasia syndromes (MDS) are clonal stem cell disorders characterized by progressive cytopenia or cytopenias, usually in the presence of a hypercellular bone marrow and multilineage dysplasia. Usually, all 3 cell lines (myeloid/monocyte, erythroid, megakaryocyte) are involved. Myelodysplasia syndrome is rare in childhood, and most children have a rapidly progressive course. Myelodysplasia disorders have been defined by their predilection to evolve into acute myeloid leukemias (AML), yet not all cases terminate in leukemia.
For information on Shwachman-Diamond Syndrome check out Shwachman-Diamond America
Showing posts with label MDS. Show all posts
Showing posts with label MDS. Show all posts
Tuesday, May 27, 2008
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Parvo B19 Infection Reminiscent of Myelodysplastic Syndrome in 3 Children.....
PARVOVIRUS B19 INFECTION REMINISCENT OF MYELODYSPLASTIC SYNDROME IN THREE CHILDREN WITH CHRONIC HEMOLYTIC ANEMIA
Authors: Nee Yaral a; Feride Duru a; Tansu Sipahi a; Abdurrahman Kara a; Tahsin TeziƧ a
Affiliation: a Department or Pediatric Hematology, Dr. Sami Ulus Children's Disease Centre, Ankara, Turkey.
Published in: Pediatric Hematology and Oncology, Volume 17, Issue 6 September 2000 , pages 475 - 482
Subjects: Oncology: Hematologic Oncology; Hematology: Hematologic Oncology; Pediatrics & Child Health;
Abstract
The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.
Keywords: Mds; Pancytopenia; Parvovirus B19
Authors: Nee Yaral a; Feride Duru a; Tansu Sipahi a; Abdurrahman Kara a; Tahsin TeziƧ a
Affiliation: a Department or Pediatric Hematology, Dr. Sami Ulus Children's Disease Centre, Ankara, Turkey.
Published in: Pediatric Hematology and Oncology, Volume 17, Issue 6 September 2000 , pages 475 - 482
Subjects: Oncology: Hematologic Oncology; Hematology: Hematologic Oncology; Pediatrics & Child Health;
Abstract
The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.
Keywords: Mds; Pancytopenia; Parvovirus B19
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