Jen-Yih Chu

Metastatic medulloblastoma simulating acute leukemia

3. Wintrobe MM: Clinical hematology, Philadelphia, 1974, Lea & Febiger Publishers, pp 1668-1688. 4. Field M, Block JB, Levin R, and Rail DP: Significance of blood lactate elevations among patients with acute leukemia and other neoplastic proliferative disorders, Am J Med 40:528, 1966. 5. Eisenstaedt RS, and Berkman EM: Rapid cytoreduction in acute leukemia. Management of cerebral leukostasis by cell pheresis, Transfusion 18:113, 1978. 6. Huestis DW, White RF, Price MJ, and Inman M: Use of hydroxyethyl starch to improve granulocyte collection in the Latham blood processor, Transfusion 15:559, 1975. 7. Lowenthal RM: Chronic leukemia: Treatment by leukapheresis, Exp Hematol 5(Suppl 1-2):73, 1977. 8. Meyer R J, Cuttner J, Truog P, Ambinder EP, and Hollard JF: Therapeutic leukapheresis of acute myelo-monocytic leukemia in pregnancy, Med Pediatr Oncol 4:77, 1978. 9. Huestis DW, Corrigan JJ, and Johnson HV: Leukapheresis of a five-year-old girl with chronic granulocytic leukemia, Transfusion 15:489, 1975. 10. Lagreco G, and Sprovieri L: Plasmapheresis on children of less than twenty kilograms weight, in Haemonetics Proceedings of the Advanced Component Seminar, Haemonetics Research Institute, Natick, Mass., vol 4, Item 5, 1975.

Pericarditis as presenting manifestation of acute nonlymphocytic leukemia in a young child

A case of acute nonlymphocytic leukemia presenting as pericarditis is reported in a five‐year‐old boy. Initially, a clinical diagnosis of viral pericarditis was made, because the child did not demonstrate hematologic or clinical manifestations of leukemia. Acute undifferentiated or lymphocytic leukemia was diagnosed one week after admission when his peripheral blood count became abnormal. The patient did not respond to vincristine and prednisone. When cytochemical evaluation indicated acute myelomonocytic leukemia, employment of cytosine arabinoside and 6‐thioguanine was instituted and the child began to improve. Currently, he is still in good remission and has no evidence of recurrence of pericarditis, 1 1/2 years after his initial presentation. In reviewing the literature, we found 17 patients who had leukemic pericardial effusion with cardiac tamponade. There are three reported cases of young children with pericardial effusion as the initial manifestation of acute lymphocytic leukemia, but no reported cases due to nonlymphocytic leukemia, as in this child. Cancer 52:322‐324, 1983.

Restoring Patency of Thrombosed Catheters with Cryopreserved Urokinase

In order to eliminate wasting vials of urokinase, 5000 U/ml aliquots were reconstituted and frozen. The urokinase remained effective for clearing thrombosed catheters for as long as 9 months. When catheters become occluded, patency can be restored by previously frozen urokinase.

Spontaneous resolution of myelodysplastic cytogenetic abnormality developed during the treatment of leukemia.

PURPOSE We describe the spontaneous resolution of a myelodysplastic cytogenetic abnormality developing during the treatment of acute lymphocytic leukemia. PATIENTS AND METHODS A 6-year-old girl with acute lymphocytic leukemia had a clinical picture of myelodysplasia 18 months after diagnosis. The clonal cytogenetic abnormality, 46,XX,del(5)(q12q12), resolved spontaneously 4 months after the discontinuation of chemotherapy. Maintenance chemotherapy was resumed 1 month later and continued for an additional 9 months. Currently, she has been off therapy for 10 months. CONCLUSION A myelodysplastic clonal cytogenetic abnormality developing during treatment may cause some confusion for management. This study demonstrates that spontaneous resolution is possible and that bone marrow transplantation or other intensive treatment may not be necessary.

Iron is sequestered as ferritin in macrophages in skeletal muscle of vitamin E-deficient rabbits.

Summary: Weanling rabbits were fed a purified diet with or without vitamin E supplementation to evaluate the abnormal sequestration of iron in skeletal muscle associated with vitamin E deficiency. A severe myopathy developed in unsupplemented rabbits within 3 to 4 weeks. At this time, the concentration of soluble nonheme iron in biceps femoris muscles had increased from 2.1 ± 0.4 μg/g wet weight (mean ± SD) for six control rabbits to 4.3 ± 1.4 for 10 vitamin E-deficient rabbits, and total nonheme iron had increased from 5.0 ± 1.2 to 8.4 ± 3.3. Soleus muscles had even greater increases in total and soluble nonheme iron concentrations. Intramuscular injection of iron-dextran caused large increases in total and soluble nonheme iron in noninjected muscle of vitamin E-deficient rabbits, which further exaggerated the difference between the two groups. By radioimmunoassay using an antibody to rabbit liver ferritin, the concentration of ferritin in biceps femoris muscles increased from 0.47 ± 0.18 μg/g wet weight for seven control rabbits to 6.34 ± 1.70 for 14 vitamin E-deficient rabbits. Uptake of intravenously injected transferrinbound iron into muscle of vitamin E-deficient rabbits was not increased in a short term experiment (6 h), but radioiron did accumulate in muscle in a long term experiment (6 days). There was no trapping of heat-damaged erythrocytes, no phagocytosis of intravenously injected carbon particles, and no erythrophagocytosis in muscle. An immunohistological staining method designed to detect ferritin in tissue sections stained muscle from normal rabbits very scantily but intensely stained macrophages in the muscle of vitamin E-deficient rabbits. We conclude that macrophages in skeletal muscle of vitamin E-deficient rabbits take up iron from transferrin and incorporate it into ferritin, in which form it is relatively unavailable for erythropoiesis because of slow release.

Congenital Acute Nonlymphoblastic Leukemia with Translocation (9;18)

Chromosome analysis is becoming an increasingly important tool in the diagnosis and treatment of childhood malignancies. This report illustrates a new translocation t(9;18) in a neonate with congenital acute nonlymphocytic leukemia, which predicted a bone marrow relapse in a normal appearing bone marrow.

628 SEQUESTRATION OF IRON IN MONONUCLEAR CELLS IN SKELETAL MUSCLES OF PATIENTS WITH MYOSITIS

Vitamin E deficiency in rabbits causes iron deficient erythropoiesis, severe myopathy and abnormal sequestration of iron in skeletal muscle. We have demonstrated that iron is sequestered as ferritin in macrophages. Our further study also demonstrated that iron is also sequestered as ferritin in macrophages in skeletal muscle of experimental ischemic myopathy. Human muscles were evaluated in this study for possible sequestration of ferritin in infiltrating macrophages. The muscle biopsy specimens were rapidly frozen in isopentane cooled to -160° C with liquid nitrogen and stored at -70° C. Sections of 6μm thickness were cut in cryostat. Localization of ferritin was studied by an immunoperoxidase method. Rabbit anti-ferritin antibody conjugated with peroxidase (Accurate Chemical and Scientific Co.) was used to bind the tissue ferritin and consequently produced a red brownish end product from 3-amino-9-ethyl-carbazole. We observed no ferritin in muscle cells but abundant staining of ferritin in mononuclear cells infiltrating muscle of myositis patients. Normal muscle stains minimally, usually at perivascular areas. This study suggested that similar iron sequestration as ferritin in macrophages probably also exists in the muscles of patients with myositis as in the rabbits. (Supported in part by a grant from Fleur de Lis Fund and Donald Ogle Fund)

SEQUESTRATION OF IRON AS FERRITIN IN MACROPHAGES IN SKELETAL MUSCLE OF VITAMIN E-DEFICIENT RABBITS

Vitamin E deficiency causes iron-deficient erythropoiesis, severe myopathy and abnormal sequestration of iron in skeletal muscle. In the present study we found that both total nonheme iron (5.0±1.2 vs 8.4±3.3μg/g wet biceps femoris muscle) and soluble nonheme iron (2.1±0.4 vs 4.3±1.4) were increased in vitamin E-deficient rabbits. By radioimmunoassay using an antibody to rabbit liver ferritin, muscle ferritin increased from 0.47±0.18 for controls to 6.34±1.70μg/g wet weight in vitamin E-deficient animals. Parenteral iron caused large increases in total and soluble nonheme iron in non-injected muscles of vitamin E-deficient rabbits and exaggerated the difference between the two groups. Uptake of intravenously injected transferrin-bound iron by muscles of vitamin E-deficient rabbits was not increased in a short-term experiment (6 hours), but radioiron accumulated in a long-term experiment (6 days). There was no evidence of erythrocyte sequestration in skeletal muscle. An immunohistological staining method designed to detect ferritin in tissue sections failed to stain muscle from normal rabbits but intensely stained macrophages in the muscle of vitamin E-deficient rabbits. We conclude that macrophages in skeletal muscle of vitamin E-deficient rabbits take up iron from transferrin and incorporate it into ferritin, in which form it is relatively unavailable for erythropoiesis because of slow release.

CIRCULATING COLONY-FORMING CELLS IN CHILDHOOD LEUKEMIA

Serial in vitro agar culture of peripheral blood was performed in children with acute leukemia in various stages of disease. In acute lymphocytic leukemia, in contrast to the depressed colony-forming cells (CFC) in the bone marrow, circulating CFC were greatly increased in untreated patients and patients in relapse. CFC returned to normal after successful remission induction and while on maintenance therapy. Depressed CFC in marrow and continuous increased CFC in blood after vincristine and prednisone induction may indicate incomplete remission in spite of morphological criteria of remission. An increase of CFC in peripheral blood was also observed transiently during the early phase of recovery from marrow depression by either infection or chemotherapy. An increase in circulating CFC, before clinical and peripheral blood evidence of relapse, has been observed in one patient off therapy for 11 months. Patterns of in vitro growth may also help to distinguish different types of leukemias, especially the undifferentiated leukemias which fail to demonstrate cytochemical markers. A pattern of CFC and colony-stimulation activity similar to “pre leukemia” in adults was also observed in children with Fanconis aplastic anemia. In conclusion, we believe agar culture of peripheral blood, as well as bone marrow, to be useful in diagnosis, confirming the completeness of remission, and assessing prognosis in children with leukemia.