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Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report
We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20 + ), T lymphocytes (CD3...
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| Published in: | International journal of hematology 2017-11, Vol.106 (5), p.718-724 |
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| cites | cdi_FETCH-LOGICAL-c396t-7690a339fea38aedd19b094e9750260346557257f8801d7658ba5a084e7869bc3 |
| container_end_page | 724 |
| container_issue | 5 |
| container_start_page | 718 |
| container_title | International journal of hematology |
| container_volume | 106 |
| creator | Ohkawara, Hiroshi Furukawa, Miki Ikeda, Kazuhiko Shichishima-Nakamura, Akiko Fukatsu, Masahiko Sano, Takahiro Ueda, Koki Kimura, Satoshi Kanai, Risa Oka, Yuka Murakami, Fumi Suzuki, Osamu Hashimoto, Yuko Ogawa, Kazuei Ikezoe, Takayuki |
| description | We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20
+
), T lymphocytes (CD3
+
, CD8
+
), and plasma cells (CD138
+
). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient’s pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-β in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases. |
| doi_str_mv | 10.1007/s12185-017-2268-3 |
| format | article |
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+
), T lymphocytes (CD3
+
, CD8
+
), and plasma cells (CD138
+
). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient’s pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-β in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases.</description><identifier>ISSN: 0925-5710</identifier><identifier>EISSN: 1865-3774</identifier><identifier>DOI: 10.1007/s12185-017-2268-3</identifier><identifier>PMID: 28584963</identifier><language>eng</language><publisher>Tokyo: Springer Japan</publisher><subject>Anemia, Hemolytic, Autoimmune - blood ; Anemia, Hemolytic, Autoimmune - complications ; Anemia, Hemolytic, Autoimmune - drug therapy ; Anemia, Hemolytic, Autoimmune - pathology ; Biopsy ; Bone marrow ; Bone Marrow - metabolism ; Bone Marrow - pathology ; Case Report ; Case reports ; CD20 antigen ; CD3 antigen ; CD8 antigen ; Drug Resistance - drug effects ; Female ; Fibrosis ; Hematology ; Hepatitis ; Hepatitis, Autoimmune - blood ; Hepatitis, Autoimmune - complications ; Hepatitis, Autoimmune - drug therapy ; Hepatitis, Autoimmune - pathology ; Humans ; Immunotherapy ; Liver ; Lymphocytes ; Lymphocytes B ; Lymphocytes T ; Macrophages ; Medicine ; Medicine & Public Health ; Middle Aged ; Monoclonal antibodies ; Myelofibrosis ; Oncology ; Pancytopenia ; Pathogenesis ; Plasma cells ; Prednisolone ; Prednisolone - administration & dosage ; Primary Myelofibrosis - blood ; Primary Myelofibrosis - complications ; Primary Myelofibrosis - drug therapy ; Primary Myelofibrosis - pathology ; Rituximab ; Rituximab - administration & dosage ; Thrombocytopenia - blood ; Thrombocytopenia - complications ; Thrombocytopenia - drug therapy ; Thrombocytopenia - pathology ; Transforming growth factor ; Transforming Growth Factor beta - biosynthesis ; Transforming growth factor-b</subject><ispartof>International journal of hematology, 2017-11, Vol.106 (5), p.718-724</ispartof><rights>The Japanese Society of Hematology 2017</rights><rights>International Journal of Hematology is a copyright of Springer, 2017.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c396t-7690a339fea38aedd19b094e9750260346557257f8801d7658ba5a084e7869bc3</citedby><cites>FETCH-LOGICAL-c396t-7690a339fea38aedd19b094e9750260346557257f8801d7658ba5a084e7869bc3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,786,790,27957,27958</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/28584963$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ohkawara, Hiroshi</creatorcontrib><creatorcontrib>Furukawa, Miki</creatorcontrib><creatorcontrib>Ikeda, Kazuhiko</creatorcontrib><creatorcontrib>Shichishima-Nakamura, Akiko</creatorcontrib><creatorcontrib>Fukatsu, Masahiko</creatorcontrib><creatorcontrib>Sano, Takahiro</creatorcontrib><creatorcontrib>Ueda, Koki</creatorcontrib><creatorcontrib>Kimura, Satoshi</creatorcontrib><creatorcontrib>Kanai, Risa</creatorcontrib><creatorcontrib>Oka, Yuka</creatorcontrib><creatorcontrib>Murakami, Fumi</creatorcontrib><creatorcontrib>Suzuki, Osamu</creatorcontrib><creatorcontrib>Hashimoto, Yuko</creatorcontrib><creatorcontrib>Ogawa, Kazuei</creatorcontrib><creatorcontrib>Ikezoe, Takayuki</creatorcontrib><title>Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report</title><title>International journal of hematology</title><addtitle>Int J Hematol</addtitle><addtitle>Int J Hematol</addtitle><description>We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20
+
), T lymphocytes (CD3
+
, CD8
+
), and plasma cells (CD138
+
). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient’s pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-β in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases.</description><subject>Anemia, Hemolytic, Autoimmune - blood</subject><subject>Anemia, Hemolytic, Autoimmune - complications</subject><subject>Anemia, Hemolytic, Autoimmune - drug therapy</subject><subject>Anemia, Hemolytic, Autoimmune - pathology</subject><subject>Biopsy</subject><subject>Bone marrow</subject><subject>Bone Marrow - metabolism</subject><subject>Bone Marrow - pathology</subject><subject>Case Report</subject><subject>Case reports</subject><subject>CD20 antigen</subject><subject>CD3 antigen</subject><subject>CD8 antigen</subject><subject>Drug Resistance - drug effects</subject><subject>Female</subject><subject>Fibrosis</subject><subject>Hematology</subject><subject>Hepatitis</subject><subject>Hepatitis, Autoimmune - blood</subject><subject>Hepatitis, Autoimmune - complications</subject><subject>Hepatitis, Autoimmune - drug therapy</subject><subject>Hepatitis, Autoimmune - pathology</subject><subject>Humans</subject><subject>Immunotherapy</subject><subject>Liver</subject><subject>Lymphocytes</subject><subject>Lymphocytes B</subject><subject>Lymphocytes T</subject><subject>Macrophages</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Monoclonal antibodies</subject><subject>Myelofibrosis</subject><subject>Oncology</subject><subject>Pancytopenia</subject><subject>Pathogenesis</subject><subject>Plasma cells</subject><subject>Prednisolone</subject><subject>Prednisolone - administration & dosage</subject><subject>Primary Myelofibrosis - blood</subject><subject>Primary Myelofibrosis - complications</subject><subject>Primary Myelofibrosis - drug therapy</subject><subject>Primary Myelofibrosis - pathology</subject><subject>Rituximab</subject><subject>Rituximab - administration & dosage</subject><subject>Thrombocytopenia - blood</subject><subject>Thrombocytopenia - complications</subject><subject>Thrombocytopenia - drug therapy</subject><subject>Thrombocytopenia - pathology</subject><subject>Transforming growth factor</subject><subject>Transforming Growth Factor beta - biosynthesis</subject><subject>Transforming growth factor-b</subject><issn>0925-5710</issn><issn>1865-3774</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><recordid>eNp1kU1u1TAUhS1ERR-FBTBBlpgwMbXj-I8ZqtqCVKkDyjhykhueq8QOtkN522LAMlgTjlJQVYmRZd3vnHPtg9ArRt8xStVpYhXTglCmSFVJTfgTtGNaCsKVqp-iHTWVIEIxeoyep3RLC0hr9QwdV1ro2ki-Q78-Z4jB9SRCcilbn7FdcnDTtHjA0wHGMLg2hjLEzmOLZ5sdFOrO5f1DdA_rJBfM-h6ff7c-4XTwfQwT4C5M8-g6m6HfhM53EWwqV_gxl-jkgsdhwDeXF-T3zzUp7wG3Yd3Bxhju3pforghwhDnE_AIdDXZM8PL-PEFfLs5vzj6Sq-vLT2cfrkjHjcxESUMt52YAy7WFvmempaYGowStJOW1FEJVQg1aU9YrKXRrhaW6BqWlaTt-gt5uvnMM3xZIuZlc6mAcrYewpIYZKmspJeMFffMIvQ1L9GW7QgmlqJCGFYptVFf-NEUYmjm68sRDw2izltpspTalq2YttVmdX987L-0E_T_F3xYLUG1AKiP_FeKD6P-6_gG1LLBA</recordid><startdate>20171101</startdate><enddate>20171101</enddate><creator>Ohkawara, Hiroshi</creator><creator>Furukawa, Miki</creator><creator>Ikeda, Kazuhiko</creator><creator>Shichishima-Nakamura, Akiko</creator><creator>Fukatsu, Masahiko</creator><creator>Sano, Takahiro</creator><creator>Ueda, Koki</creator><creator>Kimura, Satoshi</creator><creator>Kanai, Risa</creator><creator>Oka, Yuka</creator><creator>Murakami, Fumi</creator><creator>Suzuki, Osamu</creator><creator>Hashimoto, Yuko</creator><creator>Ogawa, Kazuei</creator><creator>Ikezoe, Takayuki</creator><general>Springer Japan</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7T5</scope><scope>7T7</scope><scope>7TM</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>C1K</scope><scope>CCPQU</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20171101</creationdate><title>Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report</title><author>Ohkawara, Hiroshi ; Furukawa, Miki ; Ikeda, Kazuhiko ; Shichishima-Nakamura, Akiko ; Fukatsu, Masahiko ; Sano, Takahiro ; Ueda, Koki ; Kimura, Satoshi ; Kanai, Risa ; Oka, Yuka ; Murakami, Fumi ; Suzuki, Osamu ; Hashimoto, Yuko ; Ogawa, Kazuei ; Ikezoe, Takayuki</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c396t-7690a339fea38aedd19b094e9750260346557257f8801d7658ba5a084e7869bc3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Anemia, Hemolytic, Autoimmune - blood</topic><topic>Anemia, Hemolytic, Autoimmune - complications</topic><topic>Anemia, Hemolytic, Autoimmune - drug therapy</topic><topic>Anemia, Hemolytic, Autoimmune - pathology</topic><topic>Biopsy</topic><topic>Bone marrow</topic><topic>Bone Marrow - metabolism</topic><topic>Bone Marrow - pathology</topic><topic>Case Report</topic><topic>Case reports</topic><topic>CD20 antigen</topic><topic>CD3 antigen</topic><topic>CD8 antigen</topic><topic>Drug Resistance - drug effects</topic><topic>Female</topic><topic>Fibrosis</topic><topic>Hematology</topic><topic>Hepatitis</topic><topic>Hepatitis, Autoimmune - blood</topic><topic>Hepatitis, Autoimmune - complications</topic><topic>Hepatitis, Autoimmune - drug therapy</topic><topic>Hepatitis, Autoimmune - pathology</topic><topic>Humans</topic><topic>Immunotherapy</topic><topic>Liver</topic><topic>Lymphocytes</topic><topic>Lymphocytes B</topic><topic>Lymphocytes T</topic><topic>Macrophages</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Middle Aged</topic><topic>Monoclonal antibodies</topic><topic>Myelofibrosis</topic><topic>Oncology</topic><topic>Pancytopenia</topic><topic>Pathogenesis</topic><topic>Plasma cells</topic><topic>Prednisolone</topic><topic>Prednisolone - 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Academic</collection><jtitle>International journal of hematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ohkawara, Hiroshi</au><au>Furukawa, Miki</au><au>Ikeda, Kazuhiko</au><au>Shichishima-Nakamura, Akiko</au><au>Fukatsu, Masahiko</au><au>Sano, Takahiro</au><au>Ueda, Koki</au><au>Kimura, Satoshi</au><au>Kanai, Risa</au><au>Oka, Yuka</au><au>Murakami, Fumi</au><au>Suzuki, Osamu</au><au>Hashimoto, Yuko</au><au>Ogawa, Kazuei</au><au>Ikezoe, Takayuki</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report</atitle><jtitle>International journal of hematology</jtitle><stitle>Int J Hematol</stitle><addtitle>Int J Hematol</addtitle><date>2017-11-01</date><risdate>2017</risdate><volume>106</volume><issue>5</issue><spage>718</spage><epage>724</epage><pages>718-724</pages><issn>0925-5710</issn><eissn>1865-3774</eissn><notes>ObjectType-Case Study-2</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-4</notes><notes>content type line 23</notes><notes>ObjectType-Report-1</notes><notes>ObjectType-Article-3</notes><abstract>We here report a 47-year-old female with autoimmune myelofibrosis (AIMF) associated with liver damage caused by autoimmune hepatitis and Evans syndrome. Bone marrow biopsy revealed hypocellular marrow with grade 2 reticulin fibrosis and increased levels of B lymphocytes (CD20
+
), T lymphocytes (CD3
+
, CD8
+
), and plasma cells (CD138
+
). Immunohistochemical analysis revealed increased expression of transforming growth factor-β (TGF-β) in infiltrating lymphocytes and macrophages in the bone marrow. She was initially treated with oral prednisolone (PSL) for 2 months, which had a limited effect. However, after treatment with rituximab, the patient’s pancytopenia showed improvement, allowing us to rapidly reduce the PSL dosage. The present case suggests the possibility that increased expression of TGF-β in infiltrating lymphocytes and macrophages of bone marrow may contribute to the pathogenesis of AIMF. Prednisolone combined with rituximab may thus be an effective option for steroid-refractory cases.</abstract><cop>Tokyo</cop><pub>Springer Japan</pub><pmid>28584963</pmid><doi>10.1007/s12185-017-2268-3</doi><tpages>7</tpages></addata></record> |
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| subjects | Anemia, Hemolytic, Autoimmune - blood Anemia, Hemolytic, Autoimmune - complications Anemia, Hemolytic, Autoimmune - drug therapy Anemia, Hemolytic, Autoimmune - pathology Biopsy Bone marrow Bone Marrow - metabolism Bone Marrow - pathology Case Report Case reports CD20 antigen CD3 antigen CD8 antigen Drug Resistance - drug effects Female Fibrosis Hematology Hepatitis Hepatitis, Autoimmune - blood Hepatitis, Autoimmune - complications Hepatitis, Autoimmune - drug therapy Hepatitis, Autoimmune - pathology Humans Immunotherapy Liver Lymphocytes Lymphocytes B Lymphocytes T Macrophages Medicine Medicine & Public Health Middle Aged Monoclonal antibodies Myelofibrosis Oncology Pancytopenia Pathogenesis Plasma cells Prednisolone Prednisolone - administration & dosage Primary Myelofibrosis - blood Primary Myelofibrosis - complications Primary Myelofibrosis - drug therapy Primary Myelofibrosis - pathology Rituximab Rituximab - administration & dosage Thrombocytopenia - blood Thrombocytopenia - complications Thrombocytopenia - drug therapy Thrombocytopenia - pathology Transforming growth factor Transforming Growth Factor beta - biosynthesis Transforming growth factor-b |
| title | Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-β in the bone marrow: a case report |
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