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Fanconi's constitutional panmyelopathy

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Abstract

Fanconi's constitutional panmyelopathy is a form of aplastic anemia characterised by marked pallor, increased pigmentation, short stature, skeletal anomalies and hypogonadism. Consanguinity is mostly present. Chromosomal abnormalities are frequently seen and are diagnostic. Peripheral smear and bone marrow studies help to confirm the diagnosis. In spite of treatment with androgens and steroids, the mortality rate continues to be high although these children do have long periods of remission. Bone marrow transplant is a useful recent addition to management.

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Authors and Affiliations

  1. Department of Pediatrics, Institute of Child Health and Hospital for Children, 600 008, Madras, Tamil Nadu

    V. Pushpa

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  1. V. Pushpa
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Pushpa, V. Fanconi's constitutional panmyelopathy. Indian J Pediatr 54, 775–778 (1987). https://doi.org/10.1007/BF02751303

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  • DOI: https://doi.org/10.1007/BF02751303

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