Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[Congenital aplastic anemia, type I].

R Jean, D Dossa, M Navarro

    Archives Francaises De Pediatrie
    |April 1, 1975
    PubMed
    Summary

    This case report details a rare congenital dyserythropoiesis (CD) in a child, presenting with anemia and unique erythrocyte membrane abnormalities. The findings highlight a distinct form of ineffective erythropoiesis with unusual lysis patterns.

    Related Concept Videos

    You might also read

    Related Articles

    Articles linked to this work by shared authors, journal, and citation graph.

    Sort by
    Same author

    Producer satisfaction, efficiency, and investment cost factors of different milking systems.

    Journal of dairy science·2001
    Same author

    [Osteogenesis imperfecta. The effect of intramedullary nails in long tubular bones].

    Ugeskrift for laeger·2001
    Same author

    [Endoscopic closure of cranionasal cerebrospinal fluid fistulas with the use of neuronavigation--"Computer Aided Surgery"].

    Ugeskrift for laeger·2001
    Same author

    Men at risk of being a mutation carrier for hereditary breast/ovarian cancer: an exploration of attitudes and psychological functioning during genetic testing.

    European journal of human genetics : EJHG·2001
    Same author

    Topically applied liposome encapsulated superoxide dismutase reduces postburn wound size and edema formation.

    European journal of pharmaceutical sciences : official journal of the European Federation for Pharmaceutical Sciences·2001
    Same author

    Principles of computer-assisted arthroscopy of the temporomandibular joint with optoelectronic tracking technology.

    Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics·2001

    Area of Science:

    • Hematology
    • Genetics
    • Pediatrics

    Background:

    • Congenital dyserythropoiesis (CD) encompasses rare inherited disorders of red blood cell production.
    • Heimpel and Wendt described Type I CD characterized by specific bone marrow morphological abnormalities.
    • HEMPAS (Hereditary Erythrocytosis with Multiple Algoridian Spheres) is a distinct subtype of CD, Type II.

    Observation:

    • A 7-year-old girl presented with lifelong anemia of variable intensity and moderate reticulocytosis.
    • Bone marrow examination revealed marked erythroblastosis with abnormal morphology, including internuclear chromatin bridges.
    • Erythrokinetics indicated ineffective erythropoiesis, consistent with congenital dyserythropoiesis.

    Findings:

    • The patient's condition aligns with Heimpel and Wendt's Type I congenital dyserythropoiesis.
    • Erythrocytes exhibited lysis in acidified sera, indicating a membrane anomaly.
    • This membrane anomaly's lysis characteristics differed from those observed in Type II (HEMPAS) erythrocytes.

    Implications:

    • This case expands the understanding of congenital dyserythropoiesis, particularly Type I.
    • The unusual membrane anomaly and lysis pattern in Type I CD warrant further investigation.
    • Differentiating between CD subtypes is crucial for accurate diagnosis and potential therapeutic strategies.

    Related Experiment Videos