初発時リンパ性白血病の特徴を有し,経過中単球性白血病へ分化したと思われる先天性白血病の1例

書誌事項

タイトル別名
  • Congenital Leukemia: A Case Report with the Evidence of Differentiation from Acute Lymphocytic Leukemia to Acute Monocytic Leukemia

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抄録

A female newborn with congenital leukemia is reported. The patient was referred to our hospital on the 4th day of life because of hepatosplenomegaly and anemia. Hematological findings on admission revealed remarkable leukocytosis (259,400/mm3) with 94.5% blasts, Hb 7.3 g/dl and platelets 56,000/mm3. Her bone marrow was normocellular with 95.6% blasts as seen in the peripheral blood. Morphology of the blasts stained by Giemsa method was compatible with lymphoid leukemia (FAB, L2 type). The blastic cells showed negative peroxidase stain and negative α-naphthyl butyrate esterase (ANBE) stain. The immunological phenotype was sIg-, E-rosette-, EA-rosette-, EAC-rosette-, Ia like antigen+, common ALL antigen+ and myelomonocytoid antigen+.<br>The first remission was achieved by VP therapy, however, on the 9th week of disease the monocytoid blastic cells increased in peripheral blood and bone marrow. At the time of relapse, blastic cells disclosed IgG-Fc-receptors, positive peroxidase reaction and strong positive ANBE reaction, which was completely inhibited with NaF. Chromosomal analyses by G— and Q— bands revealed 46, XX, t (11; 19) (q23; p13), and the time of relapse additional abnormalities, +8 and i (19p), were also disclosed. These results might suggest that the blastic cells of this case were compatible with bone marrow precursor cells or myeloid progenitor cells which were proposed by Parkin et al.<br>The patient died of CNS leukemia and pneumonia at the 7th month of life.

収録刊行物

  • 臨床血液

    臨床血液 26 (5), 766-771, 1985

    一般社団法人 日本血液学会

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