RESUMO
Hepatitis B virus (HBV) reactivation-related hepatitis is likely to progress to acute liver failure, with high morbidity and mortality, even when nucleoside analogs are administered after the onset of hepatitis. We report a case of adult T-cell leukemia/lymphoma (ATLL) with the development of HBV reactivation-related hepatitis during chemotherapy and successful treatment by a combination of entecavir and short-term intravenous administration of interferon (IFN)-ß 3 MIU twice per day. This outcome suggests that this combination therapy has a potent effect in rapidly suppressing HBV replication in the early phase of hepatitis and may be effective and safe for the treatment of HBV reactivation-related hepatitis.
RESUMO
The JAK2V617F mutation is a driver mutation of myeloproliferative neoplasms (MPNs). V617F allele burden is considered a risk factor for complications associated with MPNs and is a predictor of prognosis. In Japan, V617F allele burden has been measured in laboratory settings using the i-densyTM IS-5320 genetic analyzer with the quenching probe-Tm (QP-Tm) method. However, since 2020, allele-specific quantitative PCR (AS-qPCR) is being performed in clinical settings for measuring V617F allele burden. To investigate the clinical usefulness of the QP-Tm method in patients with MPNs, we evaluated the V617F allele burden measured by both the methods. A good correlation was observed between the V617F allele burden determined using QP-Tm and that determined using AS-qPCR (P<0.001, rs=0.952). The median mutant allele burden, as determined using the QP-Tm method, was significantly higher in patients with polycythemia vera than in those with essential thrombocythemia. The results of this study suggested that the QP-Tm method will continue to be useful clinical ancillary test for measuring V617F allele burden.
Assuntos
Transtornos Mieloproliferativos , Policitemia Vera , Alelos , Humanos , Janus Quinase 2/genética , Mutação , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Policitemia Vera/genética , Reação em Cadeia da Polimerase em Tempo RealRESUMO
Acute myeloid leukemia (AML) with BCR-ABL1 is rare and has a poor prognosis with conventional chemotherapy or ABL tyrosine kinase inhibitors (TKIs) alone. We reported a case of AML with BCR-ABL1 patient who was successfully treated with dasatinib alone; additionally, we previously reported another case of long-term remission maintained with imatinib monotherapy. These results suggested that a treatment with a novel and significantly potent TKI may be effective in AML with BCR-ABL1 patients with low tumor burden and without additional chromosome aberrations and ABL kinase domain mutations.
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Donor cell-derived leukemia and myelodysplastic syndrome (DCL) is a rare complication in patients after allogenic stem cell transplantation (SCT). Since 1971, numerous cases of DCL have been reported, but the detailed mechanisms of DCL are still unclear. A patient with jumping translocations (JTs) of 1q in umbilical cord blood donor cell-derived myelodysplastic syndrome (MDS), which likely occurred due to genetic alterations of TET2 and ASXL1 after cord blood transplantation (CBT), was examined in this study. Previously reported DCL cases after CBT that focused on the cytogenetic and molecular characteristics of these patients and patient outcome were reviewed. A total of 30 cases of DCL after CBT were identified between 2005 and 2018. The median time from CBT to the development of DCL was 16 months. The number of patients with DCL who were diagnosed with acute myeloid leukemia (AML) and MDS was 19 and 8, respectively. JTs were frequently observed in 5 of 27 DCL patients who had cytogenetic abnormalities, including our patient. Molecular abnormalities were described in 7 of the cases, and the most frequent abnormality was an NPM1 mutation. Other gene mutations that were usually found in de novo MDS or AML were observed in JT-DCL after CBT. From these results, chromosomal abnormalities such as JTs that occur subsequent to genetic alterations were seemed an important mechanisms underlying DCL onset in patients after CBT. Further molecular analyses regarding the genetic alterations of JTs are required to understand the pathogenesis of umbilical cord blood-derived JT-DCL.
RESUMO
Autoimmune hemolytic anemia (AIHA) is secondary to underlying diseases, such as autoimmune diseases and lymphoid malignancies. Recently, solid cancers have also been reported to be associated with AIHA, although there is not much information available. In this study, we retrospectively examined the correlation between AIHA and onset of malignancy in 100 patients diagnosed with AIHA based on the broad definition of AIHA at our hospital and cooperating institutions from January 1, 1995 to May 31, 2016. Malignancies were detected in 52 of the 100 patients (hematological malignancies: 39 patients; solid cancers: 22 patients; total malignancies including multiple primary malignancies: 67 patients). Of the 67 patients with malignancies, 28 were diagnosed with malignancies within 6 months of AIHA diagnosis. All patients with cold agglutinin disease (CAD) were associated with malignancies. Compared with warm AIHA, solid cancers were significantly more common among the patients with CAD. These findings emphasize the importance of investigating the malignancies upon diagnosis of AIHA.
Assuntos
Anemia Hemolítica Autoimune/complicações , Neoplasias/complicações , Humanos , Estudos Retrospectivos , Fatores de RiscoRESUMO
BACKGROUND: Primary testicular lymphoma (PTL) is a rare, extranodal lymphoma that often relapses in the contralateral testis. We evaluated outcomes in patients with any stage of PTL who had received CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) with rituximab chemotherapy and prophylactic radiotherapy to the contralateral testis. METHODS: We retrospectively identified 15 patients (median age 66 years; range 39-81) diagnosed with diffuse large B-cell PTL in the period 2000-2014. Characteristics and outcomes of these cases were evaluated. RESULTS: All patients received initial orchiectomy followed by CHOP with or without rituximab. Thirteen patients received prophylactic irradiation to the contralateral testis. During follow-up (median 67 months; range 8-190), one patient died of PTL, three died of other disease, and nine were free from relapse. For stage I-II disease, 5-year progression-free and overall survival rates were 80 and 100%, respectively. For stage III-IV PTL, 5-year progression-free and overall survival rates were 50 and 72%, respectively. Notably, no patient developed contralateral testicular involvement after prophylactic irradiation. CONCLUSIONS: The observed outcomes suggest that the combination of (i) CHOP plus rituximab and (ii) radiotherapy for local recurrence prophylaxis is promising for both stage I-II and stage III-IV PTL.
Assuntos
Linfoma/mortalidade , Linfoma/terapia , Neoplasias Testiculares/mortalidade , Neoplasias Testiculares/prevenção & controle , Testículo , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Monoclonais Murinos/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Ciclofosfamida/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prednisona/administração & dosagem , Radioterapia/métodos , Estudos Retrospectivos , Rituximab , Taxa de Sobrevida , Vincristina/administração & dosagemRESUMO
We herein describe the case of a 60-year-old man with a history of Behçet's disease and myelodysplastic syndrome who received cord blood transplantation (CBT). The patient was given anti-thymocyte globulin conditioning and tacrolimus to prevent graft-versus-host disease. Two months after CBT, his blood Tac concentration measured by an antibody-conjugated magnetic immunoassay (ACMIA) was found to have increased >4-fold, even after the Tac treatment was stopped. This false response was caused by the interference of endogenous heterophilic antibodies with ACMIA. Therefore, physicians must be aware of possible false ACMIA results for patients with a history of autoimmune disease and/or treated by xenogeneic antibody therapy.
Assuntos
Ciclosporina/administração & dosagem , Doença Enxerto-Hospedeiro/prevenção & controle , Imunossupressores/administração & dosagem , Síndromes Mielodisplásicas/terapia , Tacrolimo/administração & dosagem , Síndrome de Behçet/epidemiologia , Transplante de Células-Tronco de Sangue do Cordão Umbilical , Reações Falso-Positivas , Humanos , Imunoensaio , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/epidemiologiaRESUMO
Acute lymphoblastic leukemia (ALL) with chromosome aberration t(8;14)(q11.2;q32) mostly affects patients younger than 20 years old. One third of patients with this translocation have been reported to have Down syndrome. This translocation has been reported rarely in patients over the age of 50. Here we report a 71-year-old male ALL patient who carried t(8;14)(q11.2;q32). Fluorescence in situ hybridization (FISH) analysis revealed the involvement of CCAAT enhancer-binding protein delta (CEBPD) gene on chromosome 8, and IgH gene on chromosome 14. This case provides a new aspect for considering this clinical entity.
Assuntos
Cromossomos Humanos Par 14/genética , Cromossomos Humanos Par 8/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Translocação Genética , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Proteína delta de Ligação ao Facilitador CCAAT/genética , Aberrações Cromossômicas , Evolução Fatal , Genes de Cadeia Pesada de Imunoglobulina , Humanos , Hibridização in Situ Fluorescente , Masculino , Recidiva Local de Neoplasia/patologiaRESUMO
[reaction: see text] The first total synthesis of 5,6,11-trideoxytetrodotoxin (1) and its 4-epimer were achieved. The synthesis is characterized by the stereoselective construction of the quaternary amino carbon center at C8a by an asymmetric transferring Strecker synthesis and the highly efficient conversion of cyanohydrin 4 to 1 via intramolecular cyclization reactions.