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One hundred eighty-two children with acute lymphoblastic leukemia (ALL), reticulum cell sarcoma (RCS), and lymphocytic lymphosarcoma (LS) were divided into four groups as follows: Group 1—LS or RCS which never undergoes leukemic conversion; Group 2—Gross nodal enlargement either ALL from diagnosis or localized LCS or RCS subsequently converting to ALL; Group 3—ALL with palpable spleen or liver, but no gross nodal enlargement; Group 4—ALL without palpable spleen, liver, or gross nodal enlargement. It was found that Group 4, despite a superior survival (42.5 months), had a reduced incidence (31.8%) of central nervous system leukemia. Leukemic “conversion” of LS or RCS and ALL with gross nodal enlargement (Group 2) had the shortest survival (median 8.5 months) but a relatively high percentage (58.8%) with CNS disease. In non-converting LS or RCS (Group 1) only 20% had CNS involvement. On histologic criteria alone, 14.3% with RCS had CNS disease, whereas 57.9% of those with LS were affected. It is felt that a distinct adverse relationship exists between extra medullary enlargement of spleen, liver, and lymph nodes at diagnosis and the subsequent involvement of the CNS in ALL and transforming LS.
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Fitzpatrick et al. (1974) studied this question.
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