Acquired hemoglobin H disease in a patient with aplastic anemia evolving into acute myeloid leukemia
The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal noctur...
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Published in: | São Paulo medical journal Vol. 122; no. 6; pp. 273 - 275 |
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04-11-2004
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Abstract | The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia.
We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin. |
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AbstractList | CONTEXTThe prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia.CASE REPORTWe report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin. CONTEXT: The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia. CASE REPORT: We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin. CONTEXTO: O prognóstico da anemia aplástica grave melhorou com o advento do transplante de medula óssea e do tratamento imunossupressor com globulina antitimocitária. Em contraste com o sucesso destes protocolos, os estudos com seguimento a longo prazo mostraram a ocorrência de doenças clonais, tais como: hemoglobinúria paroxística noturna, síndrome mielodisplásica e leucemia aguda. RELATO DE CASO: Nós relatamos o primeiro caso descrito no Brasil de um paciente com anemia aplástica que evoluiu para síndrome mielodisplásica e leucemia mielóide aguda associada a presença de hemoglobina H e aumento da hemoglobina fetal. The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia. We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin. CONTEXT: The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia. CASE REPORT: We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin. |
Author | Vicari, Perla Yamamoto, Mihoko Antunes, Sandra Vallin Figueiredo, Maria Stella Kimura, Eliza Yuriko Sugano |
AuthorAffiliation | Universidade Federal de São Paulo |
AuthorAffiliation_xml | – name: Universidade Federal de São Paulo |
Author_xml | – sequence: 1 givenname: Maria Stella surname: Figueiredo fullname: Figueiredo, Maria Stella email: stella@hemato.epm.br organization: Universidade Federal de São Paulo, Escola Paulista de Medicina, São Paulo, Brazil. stella@hemato.epm.br – sequence: 2 givenname: Perla surname: Vicari fullname: Vicari, Perla – sequence: 3 givenname: Eliza Yuriko Sugano surname: Kimura fullname: Kimura, Eliza Yuriko Sugano – sequence: 4 givenname: Sandra Vallin surname: Antunes fullname: Antunes, Sandra Vallin – sequence: 5 givenname: Mihoko surname: Yamamoto fullname: Yamamoto, Mihoko |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/15692723$$D View this record in MEDLINE/PubMed |
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Keywords | Anemia aplástica Disease Hemoglobina H Myeloid leukemia Aplastic anemia Leucemia mielóide Hemoglobin H Doença Síndromes mielodisplásicas Myelodysplastic syndromes |
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References | Socié G (ref3) 2000; 37 Tichelli A (ref2) 1988; 69 Barrett J (ref6) 2000; 37 Tamura S (ref1) 1993; 84 Annino L (ref5) 1984; 72 Kudo S (ref4) 2000; 291 Tichelli, A; Gratwohl, A; Wursch, A; Nissen, C; Speck, B 1988; 69 Barrett, J; Saunthararajah, Y; Molldrem, J 2000; 37 Tamura, S; Kanamaru, A; Takemoto, Y; Kakishita, E; Nagai, K 1993; 84 Socié, G; Rosenfeld, S; Frickhofen, N; Gluckman, E; Tichelli, A 2000; 37 Kudo, S; Harigae, H; Watanabe, N 2000; 291 Annino, L; Di Giovanni, S; Tentori, L 1984; 72 |
References_xml | – volume: 72 start-page: 41 issue: 1 year: 1984 ident: ref5 article-title: Acquired hemoglobin H disease in a case of refractory anemia with excess of blasts (RAEB) evolving into acute nonlymphoid leukemia publication-title: Acta Haemat doi: 10.1159/000206355 contributor: fullname: Annino L – volume: 84 start-page: 219 issn: 0007-1048 issue: 2 year: 1993 ident: ref1 article-title: Clonal evolutions during long-term cultures of bone marrow from de novo acute myeloid leukaemia with trilineage myelodysplasia and with myelodysplastic remission marrow publication-title: Br J Haematol doi: 10.1111/j.1365-2141.1993.tb03055.x contributor: fullname: Tamura S – volume: 37 start-page: 15 issn: 0037-1963 issue: 1 year: 2000 ident: ref6 article-title: Myelodysplastic syndrome and aplastic anemia: distinct entities or diseases linked by a common pathophysiology? publication-title: Semin Hematol doi: 10.1016/S0037-1963(00)90027-1 contributor: fullname: Barrett J – volume: 69 start-page: 413 issn: 0007-1048 issue: 3 year: 1988 ident: ref2 article-title: Late haematological complications in severe aplastic anaemia publication-title: Br J Haematol doi: 10.1111/j.1365-2141.1988.tb02382.x contributor: fullname: Tichelli A – volume: 37 start-page: 91 issn: 0037-1963 issue: 1 year: 2000 ident: ref3 article-title: Late clonal diseases of treated aplastic anemia publication-title: Semin Hematol doi: 10.1016/S0037-1963(00)90033-7 contributor: fullname: Socié G – volume: 291 start-page: 83 issn: 0009-8981 issue: 1 year: 2000 ident: ref4 article-title: Increased HbF levels in dyserythropoiesis publication-title: Clin Chim Acta doi: 10.1016/S0009-8981(99)00186-2 contributor: fullname: Kudo S – volume: 37 start-page: 15 issue: 1 year: 2000 end-page: 29 article-title: Myelodysplastic syndrome and aplastic anemia: distinct entities or diseases linked by a common pathophysiology? publication-title: Semin Hematol contributor: fullname: Barrett, J; Saunthararajah, Y; Molldrem, J – volume: 37 start-page: 91 issue: 1 year: 2000 end-page: 101 article-title: Late clonal diseases of treated aplastic anemia publication-title: Semin Hematol contributor: fullname: Socié, G; Rosenfeld, S; Frickhofen, N; Gluckman, E; Tichelli, A – volume: 69 start-page: 413 issue: 3 year: 1988 end-page: 8 article-title: Late haematological complications in severe aplastic anaemia publication-title: Br J Haematol contributor: fullname: Tichelli, A; Gratwohl, A; Wursch, A; Nissen, C; Speck, B – volume: 84 start-page: 219 issue: 2 year: 1993 end-page: 26 article-title: Clonal evolutions during long-term cultures of bone marrow from de novo acute myeloid leukaemia with trilineage myelodysplasia and with myelodysplastic remission marrow publication-title: Br J Haematol contributor: fullname: Tamura, S; Kanamaru, A; Takemoto, Y; Kakishita, E; Nagai, K – volume: 72 start-page: 41 issue: 1 year: 1984 end-page: 4 article-title: Acquired hemoglobin H disease in a case of refractory anemia with excess of blasts (RAEB) evolving into acute nonlymphoid leukemia publication-title: Acta Haemat contributor: fullname: Annino, L; Di Giovanni, S; Tentori, L – volume: 291 start-page: 83 issue: 1 year: 2000 end-page: 7 article-title: Increased HbF levels in dyserythropoiesis publication-title: Clin Chim Acta contributor: fullname: Kudo, S; Harigae, H; Watanabe, N |
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SubjectTerms | Acute Disease Adult Anemia, Aplastic - blood Anemia, Aplastic - complications Aplastic anemia Disease Fatal Outcome Hemoglobin H Humans Leukemia, Myeloid - blood Leukemia, Myeloid - etiology Male MEDICINE, GENERAL & INTERNAL Myelodysplastic syndromes Myelodysplastic Syndromes - complications Myeloid leukemia Time Factors |
Title | Acquired hemoglobin H disease in a patient with aplastic anemia evolving into acute myeloid leukemia |
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