摘要:

Neonatal polymorphonuclear leukocytes (PMNs) are deficient in the expression of the adherence protein C3bi (Mol), and are associated with reduced physiological inflammatory responses. We evaluated the priming and direct stimulating effect of recombinant human granulocyte-macrophage colony stimulating factor (rhGM-CSF) on newborn PMN expression of C3bi (Mol), PMN adherence and PMN aggregation. Cord PMNs were incubated with rhGM-CSF (Amgen 4 × 107U/mg) for 0–15 min, and C3bi surface receptor expression measured by immunofluorescence with CD11b, adherence with nylon wool, and aggregation using a Payton aggregometer. RhGM-CSF (15 min) significantly induced Mol expression: 250 pM/L 129.4 ± 5.3% of C (p < 0.001) 500 pM/L 141.5 ± 4. 1% (p < 0.001), 1,000 pM/L 150.2 ± 1.3% (p < 0.0001). RhGM-CSF (1,000 pM/L × 5 min) followed by A23187 also primed newborn PMNs for increased Mol expression 122 ± 4.5% of C (p < 0.001). Additionally, rhGM-CSF (10 min) induced significant PMN adherence 50 pM/L 117.9 ± 8.3% and 100 pM/L 131.5 ± 5.7% of C (p < 0.04). RhGM-CSF additionally primed newborn PMNs (100 pM/L) for increased adherence following A23187 (107.9 ± 0.6% of C), p < 0.02. Lastly, rhGM-CSF primed newborn PMNs for increased aggregation following FMLP: 100 pM/L, 15 min, 138.1 ± 14.1%, p < 0.0001. Co-incubating murine-antihuman GM-CSF AB 100 μg/ml neutralized 86.8 ± 7.0% of newborn PMN Mol up-regulation. These studies demonstrate that rhGM-CSF primes and directly stimulates newborn PMNs for increased in vitro expression of Mol, adherence, and aggregation. This modulation in PMN mobility may prove to have either a beneficial or negative effect in future in vivo studies.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Patients with chronic renal disease in whom erythropoietin production is inadequate invariably experience moderate to severe debilitation-induced fatigue. Unlike the direct humeral control of erythropoiesis, neutropenia in the same cohort of patients appears to be under indirect control, very likely brought about by the suppressive effect of increased levels of low-density lipoprotein (LDL) on granulocyte-monocyte colony formation. Markedly elevated LDL levels were identified in plasma samples obtained from a study population of 179 chronic renal disease patients. The effect of the elevated LDL levels in the plasma of these patients resulted in a >60% decrease in granulocyte-macrophage colony-forming unit in comparison with age-matched plasma from normal individuals. Careful review of all nutritional and therapeutic events in these patients did not offer any evidence, other than the elevated LDL levels, in support of the etiology of the chronically low absolute neutrophil counts.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Growth of the patients with hematological malignancies, aplastic anemia, Fanconi's anemia, and Wiscott-Aldrich syndrome who had been treated with bone marrow transplantation (BMT) was studied. Fourteen out of 21 patients showed suppression of linear growth after BMT. Recovery of the growth velocity after 1–2 years tended to occur if BMT was performed at younger age. Six of eight patients with chronic graft-versus-host-disease (CGVHD) had impaired growth after BMT, whereas eight of 13 (61%) without CGVHD did. Provocative tests for growth hormone (GH) performed 5–72 months after BMT revealed three boys who showed poor response to more than two different stimuli. Two of these three boys had prolonged suppression of growth. Neither the age at BMT, difference in disease, nor presence of posttrans-plant growth retardation gave significant difference in the response of GH to provocative tests. It was concluded that approximately two-thirds of marrow-grafted children experienced transient decrease in growth velocity after BMT.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

From January 1983 to August 1987, 29 evaluable patients with high-grade osteosarcoma were treated in our institution with preoperative intra-atrial cisplatin, 100 mg/m2every 14 days for three courses. Surgery was done on day 42. Surgery consisted of limb salvage in six, amputations in 15, and disarticulations in eight. Postoperative chemotherapy included Adriamycin (ADR), 45 mg/m2for 2 days every 6 weeks, alternated with cisplatin 120 mg/m2every 6 weeks. The nephrotoxicity (18 out 29) was reversible in all cases. Cardiotoxicity was prominent; it was observed in 31% of patients. In six, there was congestive heart failure, but there were no fatal cases. The hematological toxicity was severe. There were three patients with fatal infections who had no evidence of disease after they had finished treatment. Seventeen of 29 patients (58.6%) were good responders and showed 60–100% tumoral necrosis after intra-atrial cisplatin. The 6-year, relapse-free survival rate was 58.6%–70.5% for the good responders and 41.6% for the poor responders (p < 0.05). The size of the tumor was the other important prognostic factor. The rate of 6-year, relapse-free survival was 73.6% for small tumors (those measuring < 100 cm2) and 33.3% for large tumors (p < 0.05).

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

From May 1985 to December 1989, while doing blood counts on hospitalized children in Bombay, over 300 blood smears snowed an impressive number of activated monocytes (AMs) and hemophagocytes (HPs). Many AMs resembled macrophages. The AM-HPs were visible in blood for 1–10 days, accompanied by a neutrophilia and a marked thrombocytopenia. Clinical features associated with these smears were fever, unresponsiveness to antibiotics, symptoms referable to the CNS and respiratory and/or gastrointestinal tracts, and bleeding. Ninety percent of affected children were under 2 years of age. The illness resolved completely or was fatal in 30%, with bleeding or respiratory failure, within 2 weeks. Children older than 2 years had underlying illness and high fever, and 40% died. Surgical candidates had obstructive gastrointestinal pathologic findings, from the stomach to the ileum. Babies under 1 month of age died, with clinical signs of deterioration and bleeding. Bone marrows were unremarkable. Few or no AM-HPs were seen. The fibrin split product tests were positive. Liver function test results were normal. Autopsies on six cases revealed edema and bleeding or thrombosis in the lungs, brain, and gastrointestinal tract. Only one neonate had a mild histiocytic infiltration in the lungs and liver. Features in common with and differences from virus-associated hemophagocytic syndromes are discussed.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Seven children with severe hemophilia A on prophylactic substitution therapy since a mean age of 5 years (group I) were investigated in 1978 (at ages 6–12 years) and in 1988 (at ages 16–22 years). The results were compared with those of seven children aged 5–12 for whom such treatment was started at a mean age of 3 years (group II). In group I, four had each had more than 20 ankle hemarthroses at the first investigation, while in group II, only one boy had experienced such a high bleeding frequency. Radiological changes in ankles were found in one of seven in group II compared with five of seven in group I. Progression of these changes was shown in eight of 10 ankles of group I at reinvestigation. Regular prophylactic therapy must start early, at ages 1–2 years, to prevent changes in ankle joints, and parents and children must learn to recognize ankle bleeding. Modern Factor VIII concentrates must be administered to children two or three times per week in dosages of 3,000 U/kg/year in order to reduce hemarthroses to a minimum. The dosages can probably be lowered if the intervals are shortened. Children on prophylactic treatment can engage in regular sports activities.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

The Pediatric Oncology Group (POG) investigated a high-dose cyclophosphamide (CPM) high-dose methotrexate (MTX) regimen to determine therapeutic efficacy in confirmed advanced nonlymphoblastic non-Hodgkin's lymphoma (NHL) (stages III and IV) and B-cell acute lymphatic leukemia (B-ALL) in children. Another goal was to determine the comparative effectiveness of shortened maintenance treatment (2 versus 6 courses) in the study population. Systemic induction therapy included vincristine, prednisone, cyclophosphamide, and intermediatedose MTX with leucovorin rescue. Superimposed intrathecal (IT) therapy included cytosine arabinoside for 2 successive days followed on day 3 by MTX. Intrathecal MTX was given 3 times during induction. At the end of induction, 2 days of triple (hydrocortisone, MTX, and cytosine arabinoside) therapy were given intrathecally (TIT). All patients then received a consolidation course of 4 doses of TIT, 2 doses of cyclophosphamide, and 4 more courses of vincristine and MTX with leucovorin rescue. Patients were then randomized to receive either 2 or 6 cycles of vincristine plus MTX with leucovorin rescue. The TIT was given with each cycle. Complete response rates by histology and Murphy stage (1) were as follows: undifferentiated lymphoma (DUL) stage III, 84/105 (80%); stage IV, 5/12 (42%); and other NHL [primarily large cell lymphoma (LCL)] stage III, 21/28 (75%); stage IV, 2/3 (67%). Event-free survival (EPS) at >2 years was similar for patients with DUL and LCL, i.e., 65 and 61%, respectively. No significant difference in outcome was noted between patient groups receiving 2 or 6 maintenance treatments (p= .76). Treatment was notable for its modest toxicity following the early change to single-dose CPM therapy. Poor outcome related to renal failure at diagnosis, and to CNS or BM or the involvement of both at diagnosis. The lactic dehydrogenase (LDH) levels were not significantly associated with treatment outcome.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Three siblings with alloimmune neonatal neutropenia are presented. An immunoglobulin G (IgG) antineutrophil antibody specific for NA-2 antigen was demonstrated in maternal serum and in both of the three affected infants who were studied as neonates. The mother's neutrophils were negative for NA-2 antigen. The father and all four children, including the three known to be affected, had neutrophils that expressed NA-2 antigen. One patient studied sequentially demonstrated an inverse relationship between the antineutrophil antibody liter and the absolute neutrophil count. Exchange transfusions did not appear to benefit two of the infants.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

A 19-month-old girl with idiopathic severe aplastic anemia refractory to multi-agent immunosuppressive therapy developed severe platelet alloimmunization following several months of platelet transfusions. She became refractory to human leukocyte antigen (HLA)-matched platelet transfusions and experienced frequent episodes of bleeding. She was treated with intravenous vincristine administered weekly for three doses and showed marked improvement in both clinical and laboratory response to platelet transfusions. When vincristine was held for 3 weeks, she again became refractory to HLA-matched platelet transfusion. Reinstitution of vincristine resulted in cessation of clinical bleeding and improved response to platelet transfusion. The mechanism of response likely involves selective delivery of cytotoxic drug to macro-phages. To our knowledge this is the first reported case of alloimmune thrombocytopenia responsive to vincristine.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID

摘要:

Multiple-organ infiltration by mature, benign erythro-phagocytic histiocytes is the pathologic hallmark of the virus-associated hemophagocytic syndrome (VAHS). Although VAHS has been described in a number of clinical settings, it has been reported following bone marrow transplantation (BMT) only once previously. Our report identifies the clinical and laboratory features associated with VAHS and compares the immune defects described in VAHS with those known to occur following BMT.

ISSN:0192-8562    

出版商:OVID    

年代:1991

数据来源: OVID