RESUMO
Recombinant human thrombomodulin (rTM) improves the blood coagulation disorder characteristic of disseminated intravascular coagulation (DIC) as well as, or even better than, other anti-DIC drugs. On post-marketing surveillance, its effectiveness has been recognized for hematologic disorders, sepsis and solid tumor subgroups. However, the effect on hemophagocytic syndrome (HPS) complicated by DIC remains unclear. We treated three HPS patients with rTM in addition to chemotherapy for the underlying diseases including nasal NK/T cell lymphoma, angioimmunoblastic T-cell lymphoma and refractory acute myeloid leukemia post cord blood transplantation. Although being refractory to medical management was suspected in our cases, clinical status rapidly came under control including not only amelioration of the blood coagulation disorder but also inflammatory reactions, such as serum ferritin and lactic acid dehydrogenase abnormalities, which represent HPS activity. These observations suggest that rTM might exert marked synergistic effects on HPS with DIC. Given the results obtained in these three cases, administration of rTM appears to offer a promising method of treating HPS complicated by DIC.
Assuntos
Coagulação Intravascular Disseminada/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/terapia , Trombomodulina/uso terapêutico , Idoso , Coagulação Intravascular Disseminada/complicações , Coagulação Intravascular Disseminada/diagnóstico , Feminino , Humanos , Linfo-Histiocitose Hemofagocítica/complicações , Masculino , Pessoa de Meia-Idade , Proteínas Recombinantes/administração & dosagem , Proteínas Recombinantes/uso terapêutico , Trombomodulina/administração & dosagem , Resultado do Tratamento , Adulto JovemRESUMO
A 26-year-old woman, who developed ALL when she was eighteen years old, achieved remission after chemotherapy. Her ALL relapsed when she was twenty-two years old. After re-induction therapy, she underwent cord blood transplantation. Her bone marrow examination on the 42nd day revealed a lymphoblast count of 16%. She was observed without any therapy, but her bone marrow blast count continued to be around 6% for three years without any symptoms. The bone marrow blast fraction originated from the cord blood. Surface marker analysis of the blast fraction initially revealed a pattern of hematogones that was CD10 and CD19 positive, but then showed a myeloblast pattern that was CD13 and CD33 positive. AML developed as donor cell leukemia. When blasts appear in the early phase after transplantation and persist, an observation period is necessary with molecular chimerism, morphology, and surface marker analysis of the blast fraction to consider relapse, hematogones, or donor cell leukemia.
Assuntos
Doadores de Sangue , Células da Medula Óssea/citologia , Transplante de Células-Tronco de Sangue do Cordão Umbilical/efeitos adversos , Diagnóstico Diferencial , Recidiva Local de Neoplasia/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adulto , Antígenos CD19 , Antígenos CD13 , Contagem de Células , Feminino , Células Precursoras de Granulócitos , Humanos , Neprilisina , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Lectina 3 Semelhante a Ig de Ligação ao Ácido SiálicoRESUMO
We herein describe an unusual case of multicentric Castleman's disease accompanied by thrombocytopenia, ascites, renal failure and myelofibrosis in a Japanese woman. The patient was initially diagnosed as having myelodysplastic syndrome with myelofibrosis. The general condition of the patient deteriorated rapidly; however, treatment with tocilizumab, an anti-interleukin-6 receptor antibody, together with corticosteroids dramatically improved her symptoms. The clinical features of this case were similar to those of three cases previously reported by Takai et al. (Rinsho Ketsueki, 2010, 51:320-5), which were determined to be thrombocytopenia, anasarca, fever, reticulin myelofibrosis and organomegaly (TAFRO) syndrome, a possibly distinct clinical entity.