|
rdf:type |
|
|
lifeskim:mentions |
|
|
pubmed:issue |
6
|
|
pubmed:dateCreated |
2003-6-9
|
|
pubmed:abstractText |
Amegakaryocytic thrombocytopenia (AMT) associated with systemic sclerosis (SSc) has been described in several case reports, but the underlying mechanisms have not been identified. Here we describe a rare case of SSc accompanied by thrombocytopenia and megakaryocytic hypoplasia, in which autoantibody against thrombopoietin receptor (c-Mpl) was detected. A 61-year-old woman with limited SSc was admitted to our hospital because of severe thrombocytopenia (platelet count 0.2 x 10(4)/mm(3)) with gingival bleeding. Her bone marrow was hypocellular with absent megakaryocytes, consistent with AMT. Treatment with corticosteroids and intravenous immunoglobulin infusions resulted in an increased platelet count, and she sustained a remission over a 1-year period, with a platelet count averaging 10.0 x 10(4)/mm(3). Her serum was strongly positive for anti-c-Mpl antibody, and IgG fraction purified from her serum inhibited thrombopoietin-dependent cell proliferation in vitro. Our case report suggests that AMT in patients with SSc could be mediated by the anti-c-Mpl antibody, which functionally blocks an interaction between thrombopoietin and c-Mpl.
|
|
pubmed:language |
eng
|
|
pubmed:journal |
|
|
pubmed:citationSubset |
AIM
|
|
pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Autoantibodies,
http://linkedlifedata.com/resource/pubmed/chemical/Glucocorticoids,
http://linkedlifedata.com/resource/pubmed/chemical/Immunoglobulins, Intravenous,
http://linkedlifedata.com/resource/pubmed/chemical/MPL protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Methylprednisolone,
http://linkedlifedata.com/resource/pubmed/chemical/Neoplasm Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Proto-Oncogene Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Receptors, Cytokine,
http://linkedlifedata.com/resource/pubmed/chemical/Receptors, Thrombopoietin
|
|
pubmed:status |
MEDLINE
|
|
pubmed:month |
Jun
|
|
pubmed:issn |
0004-3591
|
|
pubmed:author |
|
|
pubmed:issnType |
Print
|
|
pubmed:volume |
48
|
|
pubmed:owner |
NLM
|
|
pubmed:authorsComplete |
Y
|
|
pubmed:pagination |
1647-51
|
|
pubmed:dateRevised |
2006-11-15
|
|
pubmed:meshHeading |
pubmed-meshheading:12794833-Autoantibodies,
pubmed-meshheading:12794833-Bone Marrow Cells,
pubmed-meshheading:12794833-Female,
pubmed-meshheading:12794833-Glucocorticoids,
pubmed-meshheading:12794833-Humans,
pubmed-meshheading:12794833-Immunoglobulins, Intravenous,
pubmed-meshheading:12794833-Injections, Intravenous,
pubmed-meshheading:12794833-Megakaryocytes,
pubmed-meshheading:12794833-Methylprednisolone,
pubmed-meshheading:12794833-Middle Aged,
pubmed-meshheading:12794833-Neoplasm Proteins,
pubmed-meshheading:12794833-Proto-Oncogene Proteins,
pubmed-meshheading:12794833-Pulse Therapy, Drug,
pubmed-meshheading:12794833-Purpura, Thrombocytopenic, Idiopathic,
pubmed-meshheading:12794833-Receptors, Cytokine,
pubmed-meshheading:12794833-Receptors, Thrombopoietin,
pubmed-meshheading:12794833-Remission Induction,
pubmed-meshheading:12794833-Scleroderma, Systemic
|
|
pubmed:year |
2003
|
|
pubmed:articleTitle |
Anti-c-Mpl (thrombopoietin receptor) autoantibody-induced amegakaryocytic thrombocytopenia in a patient with systemic sclerosis.
|
|
pubmed:affiliation |
Department of Internal Medicine, Kawasaki Municipal Hospital, Kanagawa, Japan.
|
|
pubmed:publicationType |
Journal Article,
Case Reports
|
