RESUMO
Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare life-threatening complication of blood transfusion caused by donor T cells that escape rejection by the recipient immune system. These donor T cells drive recipient tissue damage in response to host antigens. On the other hand, GVHD occurring after allogeneic hematopoietic cell transplantation (HCT-GVHD) is also caused by donor T cells, but its pathophysiology is more complex and differs due to the effects of tissue damage caused by pre-HCT conditioning and profound immunosuppression. Both TA-GVHD and HCT-GVHD can be fatal; however, mortality is higher with TA-GVHD due to the paucity of treatment options. Here, we compare and summarize the presentation, diagnosis, pathophysiology, prevention, and treatment of TA-GVHD and HCT-GVHD.
Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Linfócitos T , Transplante Homólogo/efeitos adversosRESUMO
Acquired factor V deficiency (AFVD) is a rare autoimmune bleeding disorder. Unlike acquired hemophilia, bypass therapies with recombinant activated factor VII and activated prothrombin complex concentrates are ineffective for severe bleeding due to AFVD. Although several treatment strategies have been attempted, a standard of care for severe hemorrhage induced by AFVD is lacking. Herein, we report a case of AFVD with severe bleeding that responded to plasma exchange (PE) combined with immunosuppression. We also reviewed previously reported AFVD cases with severe hemorrhage and suggest that PE may be an effective initial treatment for AFVD-induced severe hemorrhage.
Assuntos
Deficiência do Fator V/complicações , Hemorragia/etiologia , Hemorragia/terapia , Troca Plasmática , Autoimunidade , Biomarcadores , Testes de Coagulação Sanguínea , Deficiência do Fator V/diagnóstico , Deficiência do Fator V/etiologia , Hemorragia/sangue , Hemorragia/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Troca Plasmática/efeitos adversos , Troca Plasmática/métodos , Índice de Gravidade de Doença , Avaliação de Sintomas , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
A 67-year-old man was diagnosed with a submucosal primary gastric T-cell lymphoma (PGTL) via upper gastroenteroscopy following an annual health check-up. He received six cycles of CHOP and achieved a complete remission. However, the patient relapsed 4 months post therapy. A second remission, which was maintained for years, was achieved after surgical gastrectomy followed by adjuvant chemotherapies. Prior reports have shown that surgery combined with chemotherapies was curative for patients with newly-diagnosed PGTL. In this report, surgery combined with chemotherapies was successfully applied for early-relapsed PGTL.
Assuntos
Linfoma de Células T Periférico , Linfoma de Células T , Neoplasias Gástricas , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimioterapia Adjuvante , Gastrectomia , Humanos , Linfoma de Células T Periférico/tratamento farmacológico , Masculino , Recidiva Local de Neoplasia , Neoplasias Gástricas/tratamento farmacológico , Neoplasias Gástricas/cirurgiaRESUMO
A 70-year-old man having a mass lesion on his right lower abdomen for 2 months was admitted to our hospital for diagnosis. Upon admission, the patient experienced bilateral upper and lower limb weakness, which aggravated. He underwent nerve conduction study and was diagnosed with axonal neuropathy. Diagnosis of diffuse large B-cell lymphoma (DLBCL) was accomplished via biopsy of the mass lesion, with positive laboratory tests for anti-ganglioside antibodies. Based on these results, immune-mediated DLBCL-induced polyneuropathy was suspected, and chemotherapy (R-CHOP) was immediately started. Limb weakness improved and completely resolved. After six courses of R-CHOP, no evidence of DLBCL was observed on PET/CT (i.e., complete metabolic remission). The patient lived without DLBCL relapse or neurological symptoms after remission. Only few reports regarding immune-mediated polyneuropathy induced by malignant lymphoma are available in the literature, which, together with this case, suggest that prompt control of malignant lymphoma is crucial for favorable prognosis of neuropathy.
Assuntos
Gangliosídeos/imunologia , Linfoma Difuso de Grandes Células B/imunologia , Polineuropatias/complicações , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica , Ciclofosfamida , Doxorrubicina , Humanos , Linfoma Difuso de Grandes Células B/complicações , Masculino , Recidiva Local de Neoplasia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Rituximab , VincristinaRESUMO
We report the case of a 66-year-old woman who presented with diarrhea and weight loss approximately 14 months after unrelated allogeneic bone marrow transplantation for acute myeloid leukemia. Her early post-transplant course was notable for mild acute skin graft-versus-host disease (GVHD) and biopsy-proven upper gastrointestinal (GI) acute GVHD, both of which resolved with treatment. She then developed weight loss and diarrhea treated with prednisolone for what was thought to be GI late acute GVHD. However, her diarrhea and weight loss persisted. Colonoscopy showed a grossly intact mucosa, and stool studies only confirmed steatorrhea. However, an atrophic pancreas was found on an abdominal computed tomography (CT) scan. Exocrine pancreatic enzymes, such as lipase and pancreatic amylase, were markedly decreased, yet pancreatic endocrine function remained intact. The patient's diarrhea and weight loss improved upon treatment with pancrelipase. Therefore, we suggest that her exocrine pancreatic insufficiency was likely partly caused by atypical chronic GVHD.
RESUMO
We report the case of a 28-year-old woman who developed upper abdominal pain and jaundice after a second unrelated allogeneic hematopoietic cell transplantation (allo-HCT) for acute lymphoid leukemia (ALL). Laboratory data showed elevated levels of liver enzymes, amylase, and lipase. Although acute pancreatitis was suspected, no structural lesions were detected. Liver biopsy was compatible with late-onset acute graft-versus-host disease (GVHD), which resolved following treatment with methylprednisolone (mPSL) and tacrolimus (TAC). In addition, her serum amylase level and abdominal pain rapidly resolved following acute GVHD-directed therapy. Acute pancreatitis concomitant with late-onset acute liver GVHD is extremely rare and has not been documented subsequent to a second allo-HCT.
RESUMO
Cardiotoxicity is a critical complication of allogeneic hematopoietic cell transplantation (allo-HCT). In particular, management of severe cardiotoxicity occurring in the early phases of allo-HCT is challenging. We encountered a case of severe cardiotoxicity resulting from AHF six days after allo-HCT, which resisted catecholamines and diuretics. The patient was treated with anthracycline-containing regimens and underwent myeloablative conditioning, including high-dose cyclophosphamide. As invasive circulatory assisting devices were contraindicated because of his immunocompromised status and bleeding tendency, we successfully treated the patient with ivabradine-containing medications. Ivabradine may therefore be considered an alternative drug for the treatment of severe cardiotoxicity induced by cytotoxic agents.
Assuntos
Doença Enxerto-Hospedeiro , Insuficiência Cardíaca , Transplante de Células-Tronco Hematopoéticas , Cardiotoxicidade , Doença Enxerto-Hospedeiro/etiologia , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Ivabradina/uso terapêutico , Condicionamento Pré-Transplante/métodos , Transplante Homólogo/efeitos adversosRESUMO
BACKGROUND: Chronic myeloid leukemia is a hematologic malignancy associated with the fusion of two genes: BCR and ABL1. This fusion results from a translocation between chromosomes 9 and 22, which is called the Philadelphia chromosome. Although the Philadelphia chromosome is present in more than 90% of patients with chronic myeloid leukemia, 5-8% of patients with chronic myeloid leukemia show complex variant translocations. Herein, we report a unique case of a three-way translocation variant in chronic phase chronic myeloid leukemia. CASE PRESENTATION: A 40-year-old Asian male who presented with leukocytosis was diagnosed with chronic phase chronic myeloid leukemia. Cytogenetic karyotyping analysis showed 46,XY,t(4;9;22)(q21;q34;q11.2). He was treated with bosutinib and then changed to dasatinib because of intolerance, and MR4.5 (BCR-ABL/ABL ⦠0.0032%, international scale) was achieved after 17 months of continuous treatment. CONCLUSION: This was the 14th case of t(4;9;22), in particular, a new variant Ph translocation involved in chromosome 4q21 and the first successful case treated with tyrosine kinase inhibitors in the world. We summarize previous case reports regarding three-way variant chromosome translocation, t(4;9;22) and discuss how this rare translocation is linked to prognosis.
Assuntos
Leucemia Mielogênica Crônica BCR-ABL Positiva , Cromossomo Filadélfia , Adulto , Proteínas de Fusão bcr-abl , Humanos , Cariotipagem , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Masculino , Translocação GenéticaRESUMO
A 28-year-old male visited a nearby hospital with chief complaint of bilateral back pain and fever. He was diagnosed with a right complex renal cyst (Bosniak classification, IIF) with a kidney stone and was referred to our hospital. We first suspected an incarcerated kidney stone and performed flexible transurethral lithotomy; however, his symptoms did not improve. Blood examination revealed prolonged APTT; subsequently, he was diagnosed with von Willebrand disease (VWD). Because he experienced pain due to the hemorrhagic renal cyst, we performed partial nephrectomy. Preoperatively, we supplemented the von Willebrand factor (VWF) based on the VWF activity in the patient. Although intraoperative bleeding was well controlled, he developed bleeding from pseudoaneurysms on the postoperative day (POD) 6. We immediately performed transarterial embolization along with VWF replenishment. VWF supplementation was discontinued on POD 14, and the patient was discharged on POD 23. Since then, he has not experienced a bleeding recurrence or pain. In patients with VWD, the perioperative administration of desmopressin or VWF is recommended. Although several reports showed that surgeries involving these treatments are safe, only three cases with VWD, including the present case where the patient underwent partial nephrectomy, have been reported. In the present case, postoperative bleeding occurred despite exhibiting adequate perioperative VWF activity. Thus, bleeding complications in patients with VWD undergoing partial nephrectomy must be considered and should be carefully followed up.
RESUMO
Blastic plasmacytoid dendritic cell (pDC) neoplasm (BPDCN) is a relatively rare hematological malignancy with significantly complex clinicopathological features that are still unclear. This study aimed to analyze the clinicopathological data of BPDCN and evaluate immunohistochemical detection of minimal bone marrow (BM) involvement. In this study, we examined skin and BM lesions from 6 patients with BPDCN. Neoplastic cells tested positive for CD303 (polyclonal, 100%; monoclonal, 40%) in the skin lesions and for CD303 (polyclonal, 100%; monoclonal, 67%) in the BM clots. Although immunostaining of CD4, CD56, CD123, CD303, and TCLl detected minimal BM involvement in 3 patients, morphological identification was challenging in the BM clots stained with hematoxylin-eosin. In conclusion, our results demonstrate the significance of observing BM smears to detect neoplastic cells and that immunohistochemical examination, including CD303 antibodies, is useful to detect minimal BM involvement. This study is the first to report the expression of thymic stromal lymphopoietin (TSLP) and its receptor in BPDCN cells. Therefore, the TSLP/TSLP receptor axis may be associated with the proliferation of BPDCN, and consequently, the survival of patients.
Assuntos
Células da Medula Óssea , Células Dendríticas , Lectinas Tipo C/metabolismo , Glicoproteínas de Membrana/metabolismo , Proteínas de Neoplasias/metabolismo , Plasmócitos , Receptores Imunológicos/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia por Agulha , Células da Medula Óssea/metabolismo , Células da Medula Óssea/patologia , Células Dendríticas/metabolismo , Células Dendríticas/patologia , Neoplasias Hematológicas/metabolismo , Neoplasias Hematológicas/patologia , Humanos , Imuno-Histoquímica , Masculino , Plasmócitos/metabolismo , Plasmócitos/patologiaRESUMO
The prognosis for myelodysplastic syndrome with bone marrow fibrosis (MDS-F) is worse than the prognosis of MDS without fibrosis. Hematopoietic stem cell transplantation (HSCT) is the only curative therapy; however, the indications and the procedures involved in HSCT remain unclear. We herein describe a 69-year-old Japanese man with MDS-F who received haploidentical HSCT and post-transplantation cyclophosphamide. Although the first HSCT resulted in secondary graft failure, the second HSCT using PTCy led to successful engraftment after early improvement in fibrosis. Since the incidence of graft failure is high in myelofibrosis patients, a secondary HSCT using PTCy may be successful if employed.