SERUM FREE POLYAMINES (SFPA) LEVELS IN CANCER WPEREOSINOPHILIC SYNDROME (HES) WITH CHROMOSOME 14 583 PATIENTS. R. A. Campbell. F. Bartos. D. Bartos, D.P. 586 MARKER AND BONE MARROW LYMPHOBLASTS. Robert Chilcote. Cretcie,.Spon. by R.C. Neerhout. Eugene Pergament. Judy Mikuda. Roberto Kretschmer, University of Oregon Health Sciences Center, Department of Pedia- Bangaru Jayalakshmamma, Ameets Bamzai. Michael Miller. Pritzker trics, Portland, Oregon. School of Medicine. University of Chicago. Michael Reese Hospital Elevated urinary polyamines have been demonstrated in a varie- and Medical Center. Department of Pediatrics, Chicago ty of untreated clinical cancers. SFPA levels in 20 lambda The HES is characterized by persistent eosinophilia and sig- native serum samples obtained from normal (N) and cancer (CAI nificant organ damage and is likely of diverse etiology. The re- patient specimens were determined by anti-spermine antibody radio lationship of HES to leukemic disorders has been particularly immunoassay (RIA). Levels of PA are expressed as nanogram of free difficult to clarify. We report a case of HES presenting with an spermine base per ml of fluid. - eosinophil count in excess of 100.000/mm3, congestive heart Study Group Range n x SE failure and bone marrow - but not peripheral blood - lymphoblasts. I N. Children 10-92 75 45.9 2.1 Parasitic and isohemagglutinin titers were not elevated; IgE was I1 N. Adult 15-95 74 44.0 2.7 normal. Bone marrow blasts were separated from eosinophils by 111 CA. Children 47-850 16 206 49 density gradient and tested for cytologic, immunologic and IV CA. Post Mastectomy 45-171 12 105 12.9 chromosomal markers. Lymphoblasts possessing neither T nor B V CA. Adult Female 17-150 9 95 41.6 cell markers were identified. Quinacrine band fluorescence VI CA. Adult Male 41-990 12 196 78.4 preparations disclosed that 86% of the metaphasea from the bone Values as high as 30 times normal have been encountered in pedia- marrow lymphoblasts contained an extra C group chromosome and a tric and adult age cancer patients. occasionally in those class- marker chromosome 14 (14 q+). Stimulated and unstimulated ified as in remission. All CA patients were unselected. the peripheral blood chromosomes were normal. Eosinophila and opportunity for study occuring at various stages of diagnosis, lymphoblasts disappeared under Vincristine, prednisone and 1-asp clinical course and therapy. Nevertheless, intergroup comparisons induction therapy. Chromosome 14 anomalies have been associated of CA and N subjects revealed p values of 0.05 or less in all with other lymphoproliferative disorders, such as Burkitt's analyses. Preliminary results from a prospective study of newly lymphoma, ataxia-telangiectasia and lymphosarcoma, but not myelo- diagnosed (untreated) lung cancer patients, n-8 demonstrated 718 proliferative states. Our results suggest that a "null cell" with elevated SPPA ..,slues. SFIJA elevations appear to be the ALL-like lymphoproliferative disorder with chromosome 14 marker rule in patients with a variety of liquid and solid tumors. can induce the HES.

,, , ERYTHROCYTE ACID PHOSPHATASE: AN ANALYSIS OF RELA- CIRCULATING COUJNY-FORMING CELLS IN CHILDHOOD 5 8 4 TIONSHIPS WITH SOME NEONATAL VARIABLES. E. Carapella, 587 LEUKEMIA. Sen-Yih Chu, Philip Frciling, De_n_n_~ J. Mortera, P. Lucarelli, F. Gloria-Bottini, R.M. 0_1C_o_n_!or and &c~u_~-Ml!21_fr_es_~. St. Louis University Corbo, R.Pascone, E. Nervegna, and E. Bottini (Spon. by C.D. School of Medicine. Cardinal Glennon Memorial Hospital for Cook). Dept. Genetics, Univ. Camerino; Dept. Pediatrics and Children, Department of Pediatrics, St. Lguis, Missouri. Child Health,Univ. Rane; CNR Center Evol. Genetics, Rome. Serial i! agar culture of peripheral blood was per- Previously we reported a relationship between mean serum bili- formed in children with acute leukemia in various stages af rubin levels (SBL) during the first five days of life and eryth- disease. In acute lymphocytic leukemia, in contrast to the rocyte acid phosphatase (ACP) phenotype (ph) where the CA pheno- depressed colony-forming cells (CFC) in the bone marrow. type showed the highest mean SBL. Further data on the same series circulating CFC were greatly increased in untreated patients of consecutive newborns studied are reported in the table. and patients in relapse. CFC returned to normal after successful ACP Birth Gest. Plac. Mean Max. 1st day Newborn remission induction and while on maintenance therapy. Depressed ph Wt. Age Wt. SBL SBL of SBL No. CFC in marrow and continuous increased CFC in blood after vincristine and prednisone induction may indicate incomplete Kg. Weeks Kg. mg a mq 8 mg r remission in spite of morphological criteria of remission. An A 3.37 40.5 .58 5.2 7.6 3.5 19 increase of CFC in peripheral blood was also observed transient- B 3.29 39.8 .57 5.1 3.2 112 7.7 ly during the early phase of recovery from marrow depression by AB 3.33 40.2 .55 5.1 7.9 3.1 79 either infection or chemotherapy. An increase in circulating CB 3.40 40.5 .56 4.5 3.0 13 6.8 CFC. before clinical and peripheral blood evidence of relapse, CA 2.70 37.7 .48 6.5 9.6 4.1 7 has been observed in one patient off therapy for 11 months. All ph 3.30 39.9 .56 5.1 3.2 230 7.8 Patterns of v_i_t_r_o_ growth may also help to distinguish Variance analysis shows F values significant at 0.05 level for different types of leukemias, especially the undifferentiated gestational age and mean SBL. Correlation analysis shows a cor- leukemias which fail to demonstrate cytochemical markers.. A relation coefficient of 0.18 between gestational age and mean pattern of CFC and colony-stimulation activity similar to SBL. Further analysis shows that the relationship among ACP ph, "preleukemia" in adults was also observed in children with gestational age and mean SBL have comparable strength. Therefore Fanconi 's aplast ic anemia. In conc lus ion, we be l ieve agar the effects of CA ph on SBL may depend on the action of the for- culture of peripheral blood. as well as bone marrow, to be mer on gestational age. useful in diagnosis, confirming the completeness of remission. and assessing prognosis in children with leukemia.

COMPARISON OF ERYTHROCYTE SEDIMENTATION RATES IN THREE GROUPS OF CHILDREN WITH PREDICTION OF PROGNOSISIN APLASTIC ANEMIA BY FERRO- , SICKLE CELL DISEASE (INTER-CRISIS, CRISIS 588 KINETIC STUDIES. Elfrid Cifuentes, Thomas R. Kinney, AND INFECTION). Kenneth P. Carlson, Tito C.M.Sobrinho Frances M..Gi11, and Elias Schwa=. Univ. of Pa. Audrey K. Brown, Department of Pediatrics, S.U.N.Y. Sch. of Med., The Children's Hospital of Phila., Dept. of Peds. Downstate Medical Center, Brooklyn, New York. Criteria are needed to identify at diagnosis patients with idi- It is widely believed that erythrocyte sedimentatin opathic aplastic anemia (AA) who have a poor prognosis, since ear ly bone marrow transplantation may improve their survival. In the rate (ESR) determinations are of little value inackle past 4 yrs we have studied 11 patients, aged 2-14 yrs (median 7 cell disease since the rate is comparatively slow. yrs), with AA. All had pancytopenia and aplastic or markedly hy- Using the Westergren method, uncorrected, we performed poplastic bone marrows. I addition to the usual hematologic ESR's in 44 children with sickle cell disease during evaluation, studies with 5'Fe were done to measure plasma Fe painful crises, periods of infection and during inter- clearance (FCC) and Fe utilization (Feu). All patients were Compar- crisis visits when they were otherwise well. treated with androgens and received supportive care. Six have ison of these three groups revealed that in 19 chil- died (median survival 8 mos). The others have improved and have dren with sickle cell disease the average ESR was 79m a median survival of 25 nos. Significant differences between (range 3-21 nun) during inter-crisis periods. In 10 of survivors and nonsurvivors were found in'initial absolute reticu- 12 children with proven infection, the ESR was above locyte counts (ARC)(p< 025). absolute neutrophil counts (ANC) 20 nun (average 38.3 mm), while among 13 children with (p< .005), and % ?eU (p< .002). Values for nonsurvivors were ARC painful crises the ESR was above 20 nun in only 5(aver- 1.35t0.28(~10~~/1),ANC3.48f0.76(~10~/1), and X Feu 8.0t3.3 (mean age 21 nun). The average values in patients with pain- S.E.M.). Survivors had ARC 3.11f0.84, ANC 7.03t1.14, and Z Feu ful crisis or with infection were significantly higher 48i14. RBC, WBC, and platelet levels had no predictive value for than those in the inter-crisis periods. In general, long term survival. Plasma FeC was prolonged in all, reflecting the ESR's were higher in children with infection than poor Fe uptake. Of 7 patients who had ARC less than 2.5, ANC in those experiencing painful crises. There was no less than 5.0 and less than 25% Feu. 6 died. Feu studies char- relationship between the ESR in these patients and the acterize the total erythropoietic capacity of the marrow better degree of anemia. The findings suggest that an ele- than aspiration, which is subject to sampling error. These data vated ESR (above 20 mm) is a useful diagnostic para- suggest that poor Feu at diagnosis of AA should be used as one meter suggesting infection in patients with sickle criterion for early bone marrow transplantation. cell disease.