SYNDROME MYELOPROLIFERATIF FAMILIAL

Translated title of the contribution: Familial myeloproliferative syndrome

V. Tatti, P. Marfurt, G. Sirchia

Research output: Contribution to journalArticlepeer-review

Abstract

The case histories of three patients with hematologic disorders are reviewed. The patients are members of the same family composed of three brothers and two sisters. Two have osteomyelofibrosis and one essential thrombocythemia. Hematologic, enzymatic, cytogenetic, immunocytologic and immunogenetic investigations allow the following conclusions: - all the 5 siblings have identical blood group and rhesus factor (0+); - there is an HLA identity in the 5 siblings characterized by the alleles: A2, A3, B5, B7 (w4, w6); - the white blood cell alkaline phosphatase is not increased; - no monoclonality can be shown; - the chromosome Ph1 is absent. In the second (osteomyelofibrosis) and third (essential thrombocythemia) patient an additional chromosome has been found, while the caryotype of the other three siblings, including the first patient with osteomyelofibrosis, is normal.

Translated title of the contributionFamilial myeloproliferative syndrome
Original languageFrench
Pages (from-to)196-204
Number of pages9
JournalSchweizerische Medizinische Wochenschrift
Volume114
Issue number6
Publication statusPublished - 1984

ASJC Scopus subject areas

  • Medicine(all)

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