Albinism Associated with Hemorrhagic Diathesis and Unusual Pigmented Reticular Cells in the Bone Marrow: Report of Two Cases with Histochemical Studies

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  • F. HERMANSKY
    First Medical Clinic, Charles University, Prague, and The Institute for Hematology and Blood Transfusion, Clinical Department, Prague, Czechoslovakia.
  • P. PUDLAK
    First Medical Clinic, Charles University, Prague, and The Institute for Hematology and Blood Transfusion, Clinical Department, Prague, Czechoslovakia.

抄録

<jats:title>Abstract</jats:title> <jats:p>1. A description is given of two unrelated albinos with hemorrhagic diathesis and peculiar, pigmented reticular cells in the bone marrow.</jats:p> <jats:p>2. On examining hemostasis, the only consistent abnormal laboratory finding was a prolonged bleeding time, so that the hemorrhagic disorder fell into the group of so-called pseudohemophilias.</jats:p> <jats:p>3. Unusual reticuloendothelial cells in the bone marrow were packed with blackish or greenish blue granules or particles. According to the histochemical study, the substance in the cytoplasm was probably of a lipid nature. These cells could not be identified with similar cells of this kind which have so far been described.</jats:p> <jats:p>4. The combination of the above described congenital abnormalities (albinism, pseudohemophilia and unusual pigmented macrophages in the bone marrow) in two unrelated patients suggests that a common syndrome is present.</jats:p>

収録刊行物

  • Blood

    Blood 14 (2), 162-169, 1959-02-01

    American Society of Hematology

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