Paris-Trousseau syndrome platelets in a child with Jacobsen's syndrome

L. Krishnamurti, J. P. Neglia, R. Nagarajan, S. A. Berry, J. Lohr, B. Hirsch, J. G. White

Research output: Contribution to journalArticlepeer-review

57 Scopus citations

Abstract

The thrombocytopenia in an infant with clinical features of Jacobsen's syndrome characterized by multiple congenital anomalies, cardiac defects, psychomotor retardation, and deletion of chromosome 11 at 11q23.3 has been evaluated. Study of his platelets in the electron microscope revealed giant alpha granules in his cells identical in appearance to those reported in the family with Paris-Trousseau syndrome. As a result, the Paris-Trousseau syndrome appears to be a variant of the Jacobsen syndrome, and the thrombocytopenia observed in all cases of chromosome 11q23.3 deletion due to dysmega-karyopoieses. Giant alpha granules are frequently observed in normal platelets during long-term storage and may form in Jacobsen and Paris-Trousseau platelets during prolonged residence in the bone marrow.

Original languageEnglish (US)
Pages (from-to)295-299
Number of pages5
JournalAmerican Journal of Hematology
Volume66
Issue number4
DOIs
StatePublished - Mar 24 2001

Keywords

  • Blood platelet disorders
  • Chromosome deletion
  • Chromosomes, human, pair 11
  • Growth disorders
  • Psychomotor disorders
  • Thrombocytopenia

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