Affiliation: University of Michigan
- How to approach neutropeniaLaurence A Boxer
Department of Pediatrics and Communicable Diseases, University of Michigan Health System, Ann Arbor, MI 48109 5718, USA
Hematology Am Soc Hematol Educ Program 2012:174-82. 2012....
- Congenital neutropenia in a newbornK Walkovich
Department of Pediatrics and Communicable Disease, University of Michigan, 1500 E Medical Center Drive, Ann Arbor, MI 48109 5178, USA
J Perinatol 31:S22-3. 2011..Prompt identification and treatment of infants with SCN is critical to minimizing morbidity and mortality; as such, a diagnosis of SCN must be considered in all infants with neutropenia even in the absence of infection...
- Strong evidence for autosomal dominant inheritance of severe congenital neutropenia associated with ELA2 mutationsLaurence A Boxer
Divison of Pediatric Hematology Oncology, Department of Pediatrics, C S Mott Children s Hospital, University of Michigan, Ann Arbor, Michigan 48109 0238, USA
J Pediatr 148:633-6. 2006..To investigate cases of severe congenital neutropenia (SCN) to ascertain SCN inheritance after determining that the same sperm donor was used by 4 different families to impregnate mothers...
- Ceramide 1-phosphate, a mediator of phagocytosisVania Hinkovska-Galcheva
Department of Pediatrics, Division of Hematology Oncology, Virginia Commonwealth University School of Medicine, Richmond, Virginia 23298, USA
J Biol Chem 280:26612-21. 2005..The change in the structural order parameter of the lipid rafts probably contributes to phagocytosis by promoting phagosome formation...
- Phagocytic signaling molecules in lipid rafts of COS-1 cells transfected with FcgammaRIIAPamela J Mansfield
Department of Pediatrics, Division of Hematology/Oncology, University of Michigan, Ann Arbor, MI 48109, USA
Biochem Biophys Res Commun 331:132-8. 2005..These results indicate that signaling proteins for phagocytosis are either constitutively present in, or are recruited to, lipid rafts where they are readily available to activate one another...
- Clinical applications of hematopoietic growth factors in pediatric oncologyJohn E Levine
Division of Pediatric Hematology and Oncology, University of Michigan, Ann Arbor, Michigan 48109 0914, USA
Curr Opin Hematol 9:222-7. 2002....
- Combination therapy for refractory idiopathic thrombocytopenic purpura in adolescentsJames A Williams
University of Michigan Pediatric Hematology Oncology Hospital, Ann Arbor, Michiga 48109 0238, USA
J Pediatr Hematol Oncol 25:232-5. 2003..To investigate combined immunosuppressive therapy with vincristine, methylprednisolone, and prolonged cyclosporine in adolescents with refractory idiopathic thrombocytopenic purpura (ITP)...
- Ceramide kinase promotes Ca2+ signaling near IgG-opsonized targets and enhances phagolysosomal fusion in COS-1 cellsVania Hinkovska-Galcheva
Department of Pediatrics, Division of Hematology Oncology, University of Michigan Medical School, Ann Arbor, MI 48109, USA
J Lipid Res 49:531-42. 2008....
- Rituximab for refractory childhood autoimmune hemolytic anemiaDavid G Motto
Department of Pediatric Hematology Oncology, University of Michigan, Ann Arbor, MI, USA
Isr Med Assoc J 4:1006-8. 2002..Treatment with high dose steroids, splenectomy and frequent blood transfusions results in a myriad of complications including growth failure, bone demineralization, Cushing's syndrome, immunosuppression, and transfusional hemosiderosis...
- Severe congenital neutropenia: genetics and pathogenesisLaurence A Boxer
University of Michigan Hospital, L2110 Women s Hospital, 1500 E Medical Center Drive, Ann Arbor, MI 48109 0238, USA
Trans Am Clin Climatol Assoc 117:13-31; discussion 31-2. 2006..In an unusual clinical set of studies during which SCN developed in five children from four families impregnated by the same sperm donor, we identified the responsible donor by employing genomic DNA studies...
- Introduction of the American Pediatric Society's 2008 John Howland award recipient, Richard B. Johnston Jr, M.DLaurence A Boxer
Department of Pediatrics, University of Michigan, Women's Hospital, Ann Arbor, Michigan 48109, USA
Pediatr Res 64:317-9. 2008
- Large granular lymphocyte leukemia (LGL) in a child with hyper IgM syndrome and autoimmune hemolytic anemiaBrenda J Kitchen
Division of Hematology Oncology, Department of Pediatrics and Communicable Diseases, University of Michigan, Mott Children s Hospital, Ann Arbor, Michigan, USA
Pediatr Blood Cancer 50:142-5. 2008..Flow cytometry of the peripheral blood revealed the presence of a marked predominance of cytotoxic T lymphocytes, shown to be clonal, with concomitant natural killer (NK) antigen expression. She responded to weekly methotrexate therapy...
- Cyclic neutropenia and severe congenital neutropenia in patients with a shared ELANE mutation and paternal haplotype: evidence for phenotype determination by modifying genesPeter E Newburger
Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
Pediatr Blood Cancer 55:314-7. 2010..Mutations of the ELANE gene (the symbol recently replacing ELA2) are considered largely responsible for most cases of CN and SCN, but specific mutations are typically associated with one or the other...
- Neutropenia: causes and consequencesLaurence Boxer
Department of Pediatrics, University of Washington, Seattle, WA 98195-6422, USA
Semin Hematol 39:75-81. 2002..Most severely neutropenic patients have a history of oral ulcers and inflammation and recurrent skin infections. Examination of a bone marrow aspirate and/or biopsy and cytogenetic testing are primary for diagnostic evaluation...
- Regulation of polymorphonuclear leukocyte degranulation and oxidant production by ceramide through inhibition of phospholipase DPamela J Mansfield
Department of Pediatrics, Division of Hematology Oncology, University of Michigan, Ann Arbor 48109, USA
Blood 99:1434-41. 2002..Ceramide may affect protein interactions with PLD in the plasma membrane, thereby attenuating PMN activation...
- Adolescents with cancer: access to clinical trials and age-appropriate carePeter E Newburger
Department of Pediatrics, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
Curr Opin Pediatr 14:1-4. 2002..The challenge before both the medical and pediatric health care communities is to assure adolescent patients the benefits of inclusion in clinical trials and treatment in age-appropriate settings...
- Role of neutrophils in genetic disorders of phagocyte function leading to IBDLaurence A Boxer
Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA
J Pediatr Gastroenterol Nutr 46:E17. 2008
- Neutrophil abnormalitiesLaurence A Boxer
Pediatric Hematology/Oncology, University of Michigan and Women's Hospital, Ann Arbor, MI, USA
Pediatr Rev 24:52-62. 2003
- Granulocyte colony-stimulating factor primes NADPH oxidase in neutrophils through translocation of cytochrome b(558) by gelatinase-granule releasePamela J Mansfield
Department of Pediatrics, Division of Hematology/Oncology, University of Michigan Ann Arbor, 48109, USA
J Lab Clin Med 140:9-16. 2002..We conclude that translocation of cytochrome b(558) was responsible for GCSF priming of NADPH oxidase in neutrophils...
- A molecular classification of congenital neutropenia syndromesLaurence A Boxer
Division of Pediatric Hematology Oncology, Department of Pediatrics, C S Mott Children s Hospital, University of Michigan, Ann Arbor, Michigan, USA
Pediatr Blood Cancer 49:609-14. 2007..This review describes and classifies, on a molecular basis, both primary congenital neutropenia and multifaceted syndromes associated with neutropenia...
- The American Society of Pediatric Hematology/Oncology distinguished career award goes to Laurence A. Boxer, MDPeter E Newburger
Departments of Pediatrics and Cancer Biology, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA
Pediatr Blood Cancer 50:1121-2. 2008
- Mutant elastase in pathogenesis of cyclic and severe congenital neutropeniaAndrew A G Aprikyan
Department of Medicine, University of Washington, 1959 NE Pacific Street, Seattle, WA 98195 6422, USA
J Pediatr Hematol Oncol 24:784-6. 2002..We hypothesize that abnormal processing and subcellular localization of mutant NE might predetermine the etiology of cyclic or severe congenital neutropenia...
- Recruitment and development of academic pediatricians: departmental commitments to promote successAlan H Jobe
Division of Pulmonary Biology, Children's Hospital, Cincinnati, Ohio 45229-3039, USA
Pediatr Res 51:662-4. 2002
- Severe chronic neutropenia: treatment and follow-up of patients in the Severe Chronic Neutropenia International RegistryDavid C Dale
Department of Medicine, Box 356422, University of Washington, Seattle, WA 98195, USA
Am J Hematol 72:82-93. 2003..The risk of leukemia, osteoporosis, other potentially adverse events, and pregnancy outcome need to be further evaluated with continuing long-term observations...
- Neutrophil elastase mutations and risk of leukaemia in severe congenital neutropeniaPhilip S Rosenberg
Biostatistics Branch, Division of Cancer Epidemiology and Genetics, Department of Health and Human Services, National Cancer Institute, National Institutes of Health, Rockville, MD, USA
Br J Haematol 140:210-3. 2008..96). Patients with either mutant or wild-type ELA2 should be followed closely for leukaemic transformation...
- Mutations of the ELA2 gene found in patients with severe congenital neutropenia induce the unfolded protein response and cellular apoptosisDavid S Grenda
Division of Oncology, Washington University School of Medicine, St Louis, MO 63110, USA
Blood 110:4179-87. 2007..Collectively, these data provide strong support for a UPR model of SCN disease pathogenesis and place SCN in a growing list of human diseases caused by misfolded proteins...
- Who will care for tomorrow's children with benign hematological conditions?Max J Coppes
Center for Cancer and Blood Disorders, Children s National Medical Center, and George Washington University, Washington, District of Columbia, USA
Pediatr Blood Cancer 49:224. 2007
- Distinct patterns of mutations occurring in de novo AML versus AML arising in the setting of severe congenital neutropeniaDaniel C Link
Division of Oncology, Department of Medicine, Washington University, St Louis, MO 63110, USA
Blood 110:1648-55. 2007..These data support the hypothesis that mutations of CSF3R may provide the "activated tyrosine kinase signal" that is thought to be important for leukemogenesis...
- Growth factor independence-1 (Gfi-1) plays a role in mediating specific granule deficiency (SGD) in a patient lacking a gene-inactivating mutation in the C/EBPepsilon geneArati Khanna-Gupta
Section of Hematology, Yale University School of Medicine, New Haven, CT 06510, USA
Blood 109:4181-90. 2007..We hypothesize that decreased Gfi-1 levels in our SGD patient, together with the mutant C/EBPepsilon, block SGP expression, thereby contributing to the underlying etiology of the disease in our patient...
- Chemotaxis of non-compressed blood polymorphonuclear leukocytes from an adolescent with severe leukocyte adhesion deficiencyStephen E Malawista
Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut 06520, USA
Am J Hematol 73:115-20. 2003..These findings indicate that not all patients with severe LAD are candidates for early bone marrow transplantation...
- The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapyPhilip S Rosenberg
Biostatistics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Rockville, MD 20852 7244, USA
Blood 107:4628-35. 2006..Risk of MDS/AML may be similar in SDS and SCN. In less-responsive SCN patients, early hematopoietic stem cell transplantation may be a rational option...
- Cellular and molecular abnormalities in severe congenital neutropenia predisposing to leukemiaAndrew A G Aprikyan
Department of Medicine, University of Washington School of Medicine, Seattle, Wash, USA
Exp Hematol 31:372-81. 2003..Furthermore, our results suggest that acquired G-CSFR mutations may initiate signaling events that override the pro-apoptotic effect of mutant NE in primitive progenitor cells, resulting in an expansion of the abnormal AML clone...
- REGULATION OF GRANULOCYTE SECRETIONLaurence Boxer; Fiscal Year: 1991..We will also examine by pulse-chase experiments the synthesis of adhesive glycoproteins and their accumulation on the plasma membrane of normal and deficient canine monocytes...
- REGULATION OF GRANULOCYTE SECRETIONLaurence Boxer; Fiscal Year: 1993..They will provide further biochemical understanding of the factors regulating exocytosis of SG. The knowledge of both oxidative metabolism and degranulation in PMN should lead to novel therapeutic approaches to chronic inflammation...
- REGULATION OF POLYMORPHONUCLEAR LEUKOCYTE PHAGOCYTOSISLaurence Boxer; Fiscal Year: 2003..Specific AIM 3 will examine the cellular targets of ceramide and sphingosine actin during PMN phagocytosis, focusing primarily on activation of mitogen-activated kinase (MAPK), Raf kinase and how they are linked to phagosome generation. ..