顔面神経麻痺・腫瘤を初発症状とした先天性白血病(Commom ALL)の一例

—自験例とDown症候群に併発しない先天性白血病78例の文献的考察—

抄録

A 2-month-old boy was admitted for evaluation of skin nodules observed at 5 days, a tumor on the head found at 1 month and facial nerve palsy. Hematological findings were as follows; RBC 345×10⁴/cmm, Platelet 2.8×10⁴/cmm, WBC 13,500/cmm with 100% blast cells. Bone marrow specimen revealed blast cells 33%. The leukemic cells gave no reactions with Sudan black, PAS and peroxidase. Surface markers of the blast cell were surface immunoglobulin (-), E-rosette formation (-), and common ALL antigen 50%. Karyotype of the bone marrow was 46 XY/53 XY, +3, +7, +12, +14, +14, +20, +21. He obtained complete remission with new DCVP therapy. But he died 5 months after the onset of the treatment.
Approximately 78 cases of congenital leukemia without Down's syndrome have been reported up to December of 1981 in Japan.
1) The ratio of males/females was 32/36.
2) On admission hepatosplenomegaly was found in 93%, and skin nodules in 26/50 (52%).
3) Laboratory findings at diagnosis; average leukocyte counts were 22.59×10⁴/cmm, average RBC 310×10⁴/cmm, and 15 cases (52%) had platelet count of under 5×10⁴/cmm.
4) Type of leukemia; AML 39 cases (70%), AMoL 9 cases (16%), ALL 5 cases (8%), stem cell leukemia and Null cell leukemia 4 cases (7%).
5) Survival time; About half of patients deid within 1 month.