| 词条 | Congenital dyserythropoietic anemia type IV | ||||||||
| 释义 |
| symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} PresentationCDA type IV is characterized by mild to moderate splenomegaly. Hemoglobin is very low and patients are transfusion dependent. MCV is normal or mildly elevated. Erythropoiesis is normoblastic or mildly to moderately megaloblastic. Nonspecific erythroblast dysplasia is present.[2] GeneticsCongenital dyserythropoietic anemia type IV is an autosomal dominant inherited red blood cell disorder characterized by ineffective erythropoiesis and hemolysis resulting in anemia. Circulating erythroblasts and erythroblasts in the bone marrow show various morphologic abnormalities. Affected individuals with CDAN4 also have increased levels of fetal hemoglobin.[3]
Diagnosis{{Empty section|date=December 2017}}TreatmentTreatment consists of frequent blood transfusions and chelation therapy. Potential cures include bone marrow transplantation and gene therapy. See also
References1. ^{{cite web |url=http://bloodjournal.hematologylibrary.org/content/102/13/4576.full.html |title=Archived copy |accessdate=2011-09-26 |deadurl=yes |archiveurl=https://archive.is/20120709115538/http://bloodjournal.hematologylibrary.org/content/102/13/4576.full.html |archivedate=2012-07-09 |df= }} 2. ^{{cite book|url=https://books.google.com/books?id=f5C246w1ec4C&pg=PA161&lpg=PA161&dq=cda+type+v&source=bl&ots=lZ4wsrf_az&sig=SbYiWaDXoaUBJMaWRMmUJ5zsheA&hl=en&ei=d-N_ToeeDNOGsALC5eQl&sa=X&oi=book_result&ct=result&resnum=3&ved=0CCkQ6AEwAg#v=onepage&q=cda%20type%20v&f=false |title=Manual of Pediatric Hematology and Oncology - Google Books |publisher=Books.google.com |date= |accessdate=2015-05-06}}{{dead link|date=May 2015}} 3. ^{{cite web|title=ANEMIA, CONGENITAL DYSERYTHROPOIETIC, TYPE IV; CDAN4|url=http://omim.org/entry/613673|publisher=Omim.org|accessdate=6 May 2015}} Further reading
External links{{Medical resources| ICD10 = D64.4 | ICD9 = {{ICD9|285.8}} }} 2 : Genetic disorders with no OMIM|Anemias |
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