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Der sogenannte infantile Typ der chronischen myeloischen Leukämie

The “Juvenile” type of chronic myelogenous leukemia

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Abstract

The juvenile type of chronic myelogenous leukemia (CML) is characterized by hemorrhagic diathesis, hepatosplenomegaly, generalized enlargement of lymph nodes, reduced erythro- and thrombopoiesis, leukemic infiltration of bone marrow, and decreased activity of leukocyte alkaline phosphatase. The disorder differs from the adult type by the early manifestation of anemia and thrombocytopenia, the minor degree of leucocytosis, the failure to demonstrate the Philadelphia chromosome, and the presence of fetal markers in the patients' erythrocytes. The persistence of fetal markers in erythrocytes and the clinical manifestation in infancy and early childhood suggest that the disease is congenital in origin.

The occurrence of CML of the juvenile type in an 8-year-old boy, which is reported in this paper, favors a postnatal acquisition of the disorder. The significance of hematological and cytogenetic findings and new aspects of nosological classification are discussed.

Zusammenfassung

Der juvenile Typ der chronischen myelosichen Leukämie ist gekennzeichnet durch eine hämorrhagische Diathese, eine Hepatosplenomegalie, generalisierte Lymphknotenschwellungen, eine reduzierte Erythro- und Thrombocytopoese, eine leukotische Infiltration des Knochenmarks, eine Verminderung der alkalischen Leukocytenphosphatase. Er unterscheidet sich vom adulten Typ durch das frühzeitige Auftreten von Anämie und Thrombocytopenie, die geringere Ausprägung der Leukocytose, das Fehlen des Philadelphia-Chromosoms und den Nachweis fetaler Erythrocyteneigenschaften. Die Fetalisierung der Erythrocyten und die ausschließliche Manifestation der Erkrankung im Säuglings- und Kleinkindesalter wurden als Hinweise auf die konnatale Natur des Leidens gewertet.

Das Auftreten der Erkrankung bei einem 81/2 Jahre alten Jungen, über den hier berichtet wird, spricht dagegen eher für eine erworbene Ursache. Die Wertigkeit der einzelnen Befunde sowie neuere Aspekte der nosologischen Einordnung im Rahmen der myeloproliferativen Syndrome werden diskutiert.

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Authors and Affiliations

  1. Kinderkrankenhaus der Stadt Köln, Köln, Deutschland

    H. G. Terheggen & H. Haug

  2. Medizinische Universitätsklinik Köln, Köln, Deutschland

    K. P. Hellriegel

  3. Pathologisches Institut der Universität Köln, Köln, Deutschland

    H. E. Schaefer

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  1. H. G. Terheggen
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  2. H. Haug
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  3. K. P. Hellriegel
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  4. H. E. Schaefer
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Terheggen, H.G., Haug, H., Hellriegel, K.P. et al. Der sogenannte infantile Typ der chronischen myeloischen Leukämie. Z. Kinder-Heilk. 119, 123–131 (1975). https://doi.org/10.1007/BF00443566

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