Sternal infarction in sickle-cell anemia: concise communication.

Bone scintigraphy in six children with sickle-cell anemia has demonstrated infarction of bony segments of the sternum. Anterior oblique views of the thorax provided best visualization of the infarcts which were seen as areas of decreased bone tracer accumulation.

Successful treatment of varicella pneumonia with prolonged extracorporeal membrane oxygenation in a child with leukemia.

A 5-year-old boy with acute lymphoblastic leukemia in complete continuous remission developed life-threatening varicella pneumonia and acute respiratory insufficiency (ARI). The child recovered after 92 hours of partial venoarterial perfusion with a membrane oxygenator. Functional asplenia developed. Serial pulmonary function tests after perfusion indicate moderately severe restrictive lung disease which has slightly improved during an 18 month period.

Mycoplasma salivarium in the blood of a child with leukemia.

Mycoplasma salivarium was recovered from the blood of a five-year-old girl who had leukemia and subsequently developed pneumonitis. The patient's pneumonitis failed to respond to nafcillin, a cell-wall-active antibiotic, but eventually she recovered from the pneumonia after a regimen of erythromycin. Sputum, oropharyngeal, and nasopharyngeal cultures revealed normal bacterial flora; a blood culture was negative for bacteria. Throat and sputum cultures were negative for mycoplasma; however, M salivarium was recovered from the patient's blood. The patient had a cold hemagglutinin titer of 1:250.

Myelofibrosis in children with chronic renal insufficiency.

During investigation of splenomegaly in a boy with chronic renal failure and osteodystrophy, bone marrow aspirates resulted in "dry taps," whereas biopsied material provided evidence that the marrow had been replaced by fibrous tissue. In a study of six other children with chronic renal failure, similar changes were observed. These findings suggest that the anemia of chronic renal failure may in part be a result of myelofibrosis, and the resulting reduction of functional bone marrow may limit the tolerance to immunosuppressive agents in patients who undergo renal transplantation.

Icteric plasma suggests Gilbert's syndrome in the blood donor.

BACKGROUND: In a recent quality assurance audit of component returns over a 6-month period, 9 of 81 returns were due to icteric plasma. With the sensitive, new methods used to screen donors for anemia and hepatitis, it seemed likely that the icteric discoloration reflected benign unconjugated hyperbilirubinemia (Gilbert's syndrome) in the donor, rather than liver disease or hemolysis. The donors were recalled for repeat blood study to resolve this question. STUDY DESIGN AND METHODS: Seven of the nine donors could be reached, and they submitted blood samples for measurement of serum levels of conjugated (direct-reacting) and total bilirubin and for complete blood and reticulocyte counts. RESULTS: All seven donors had mild unconjugated hyperbilirubinemia, with total bilirubin levels ranging from 1.3 to 2.8 mg per dL. None showed evidence of overt hemolysis. CONCLUSION: All seven donors of the components with icteric plasma have Gilbert's syndrome, a benign genetic anomaly occurring in approximately 3 to 5 percent of the general population. With the sensitive screening tests for viral hepatitis used today, the presence of icteric plasma in a component suggests that the donor has Gilbert's syndrome. Policies about the acceptability of icteric components from blood donors merit reassessment.

Scrotal imaging in the Henoch-Schönlein syndrome.

Testicular involvement in patients with Henoch-Schönlein syndrome may occur in as high as 15 per cent of the cases. Scrotal imaging is extremely accurate in differentiating hyperemia (vasculitis) from impairment of blood flow encountered in torsion of the spermatic cord, the most common cause of acute scrotal swelling in children.

Veno-occlusive disease of the liver in children following chemotherapy for acute myelocytic leukemia.

Three children developed acute veno-occlusive disease of the liver following combination chemotherapy for acute myelocytic leukemia. The clinical presentation was similar in all three, with acute onset of hepatomegaly and thrombocytopenia in the absence of significant transaminasemia or icterus. In all three patients, radionuclide imaging with technetium-99m sulfur colloid showed hepatosplenomegaly, decreased liver uptake, and increased splenic activity. The results of liver biopsy established the diagnosis, revealing marked centrilobular congestion with hemorrhage into the spaces of Disse, atrophy of central hepatic cords, and edema of the walls of the central and sublobular veins. Each patient showed marked improvement following temporary cessation of chemotherapy. The diagnosis of veno-occlusive disease is suggested by the triad of: (1) clinical signs and symptoms; (2) scintigraphic findings; and (3) temporal relationship to chemotherapy.

Immunogenetic determinants of familial acute lymphocytic leukemia.

Acute lymphocytic leukemia developed almost simultaneously in two adolescent brothers, and another brother and both parents had rheumatoid arthritis. Laboratory studies uncovered no evidence for an underlying immunodeficiency state in the family. Immunogenetic evaluation showed the leukemic siblings to be HLA- and mixed-leukocyte-culture identical and homozygous for a recessively inherited locus dictating the presence of antigens on the surface of B-cells. This Ia antigen, as detected by sera from mothers of leukemic children, appeared to be mapped within the major histocompatibility region and may be a human analogue to murine immune-response antigens associated with susceptibility to leukemia.

A new syndrome of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreatic dysfunction.

In the past decade, we have studied four unrelated children with what we believe is a previously unreported disorder affecting the bone marrow and exocrine pancreas. During infancy these patients had the onset of severe, transfusion-dependent, macrocytic anemia plus a variable degree of neutropenia and thrombocytopenia. Their bone marrows had normal cellularity but were characterized by remarkable vacuolization of erythroid and myeloid precursors, hemosiderosis, and ringed sideroblasts. The vacuoles probably represented manifestations of cellular degeneration and death. In two patients, in vitro bone marrow cultures showed abnormal erythroid and myeloid progenitor cell growth and, in one child, abnormal vacuolated erythroid colonies. Family histories were unrevealing, parents were hematologically normal, and both sexes were involved. There was no evidence of specific nutritional deficiencies or exposure to agents associated with marrow vacuolization. A number of therapeutic interventions produced no effect. One child had clinical malabsorption. This child and one other had extensive pancreatic fibrosis at autopsy. The other two patients had findings indicating exocrine pancreatic dysfunction. Two children had splenic atrophy. This new syndrome, with associated bone marrow and exocrine pancreatic dysfunctions, differs in several respects from the syndrome of pancreatic liposis and neutropenia described by Shwachman et all and Bodian et al, and from other conditions with vacuolization of the marrow or sideroblastosis.