Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant
ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH,...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Sociedade Brasileira de Patologia Clínica
|
| Series: | Jornal Brasileiro de Patologia e Medicina Laboratorial |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307&lng=en&tlng=en |
| _version_ | 1828379237877284864 |
|---|---|
| author | Railene Célia B. Alencar Andréa M. Guimarães Lacy C. Brito Junior |
| author_facet | Railene Célia B. Alencar Andréa M. Guimarães Lacy C. Brito Junior |
| author_sort | Railene Célia B. Alencar |
| collection | DOAJ |
| description | ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of PNH with eculizumab. Clinical stabilization and cessation of symptoms were achieved. |
| first_indexed | 2024-12-10T03:37:18Z |
| format | Article |
| id | doaj.art-3e3d66a546354d7f8100de0da47a0e9a |
| institution | Directory Open Access Journal |
| issn | 1676-2444 1678-4774 |
| language | English |
| last_indexed | 2024-12-10T03:37:18Z |
| publisher | Sociedade Brasileira de Patologia Clínica |
| record_format | Article |
| series | Jornal Brasileiro de Patologia e Medicina Laboratorial |
| spelling | doaj.art-3e3d66a546354d7f8100de0da47a0e9a2022-12-22T02:03:41ZengSociedade Brasileira de Patologia ClínicaJornal Brasileiro de Patologia e Medicina Laboratorial1676-24441678-477452530731110.5935/1676-2444.20160051S1676-24442016000500307Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplantRailene Célia B. AlencarAndréa M. GuimarãesLacy C. Brito JuniorABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of PNH with eculizumab. Clinical stabilization and cessation of symptoms were achieved.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307&lng=en&tlng=enpure red-cell aplasiaparoxysmal hemoglobinurialiver transplantation |
| spellingShingle | Railene Célia B. Alencar Andréa M. Guimarães Lacy C. Brito Junior Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant Jornal Brasileiro de Patologia e Medicina Laboratorial pure red-cell aplasia paroxysmal hemoglobinuria liver transplantation |
| title | Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant |
| title_full | Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant |
| title_fullStr | Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant |
| title_full_unstemmed | Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant |
| title_short | Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant |
| title_sort | report of a case of paroxysmal nocturnal hemoglobinuria pnh with complex evolution and liver transplant |
| topic | pure red-cell aplasia paroxysmal hemoglobinuria liver transplantation |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442016000500307&lng=en&tlng=en |
| work_keys_str_mv | AT raileneceliabalencar reportofacaseofparoxysmalnocturnalhemoglobinuriapnhwithcomplexevolutionandlivertransplant AT andreamguimaraes reportofacaseofparoxysmalnocturnalhemoglobinuriapnhwithcomplexevolutionandlivertransplant AT lacycbritojunior reportofacaseofparoxysmalnocturnalhemoglobinuriapnhwithcomplexevolutionandlivertransplant |