Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 91
Filtrar
1.
Wiad Lek ; 73(8): 1785-1789, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33055352

RESUMO

OBJECTIVE: Combination of kaposiform hemangioendothelioma (KHE) and Kasabach-Merritt phenomenon (KMP) in newborn children is a life-threatening constellation. The purpose of the study is the choice of the diagnostic and treatment methods in these patients and evaluating the effectiveness of treatment using radiological methods of investigation. The study enrolled 6 newborn patients with KHE within a period 2013 - 2018. MRI (CT) performed to make the diagnosis and evaluate treatment response. Hypervascular mass accompanied by reticular lymphedema, hyper intensive in T2 WI; isointensive in T1 WI, intense contrast enhancement, heterogeneous diffusion restriction were unique MRI characteristics of KHE. The sustained remission was achieved with treatment by propranolol (n=2), vincristine (n=1), and their combination (n=3).


Assuntos
Hemangioendotelioma , Síndrome de Kasabach-Merritt , Sarcoma de Kaposi , Hemangioendotelioma/complicações , Hemangioendotelioma/diagnóstico por imagem , Hemangioendotelioma/tratamento farmacológico , Humanos , Recém-Nascido , Síndrome de Kasabach-Merritt/complicações , Síndrome de Kasabach-Merritt/tratamento farmacológico , Imagem por Ressonância Magnética , Sarcoma de Kaposi/complicações , Sarcoma de Kaposi/tratamento farmacológico
2.
BMJ Case Rep ; 13(9)2020 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-32933906

RESUMO

Kasabach-Merritt phenomenon (KMP) is consumptive coagulopathy in large vascular lesion. This condition is specific in kaposiform haemangioendothelioma (KHE) and tufted angioma, associated with high mortality rate. This condition often involves the limbs, trunk and retroperitoneum. The clinical features that are usually found are purpura, oedema, induration and ecchymosis. A boy baby aged 9 months presented with a history of violaceous tumour that appeared on his left leg at birth that became enlarged since 6 months ago that caused discrepancy between both of his legs. The patient has his left leg massaged through alternative treatment, causing it became bruised and wounded. Blood examination showed normocytic normochromic anaemia with severe thrombocytopenia and high D-dimer. The patient was diagnosed with KMP with cellulitis and treated with oral corticosteroid, propranolol and antibiotics. Diagnosis of KMP can be made through clinical and laboratory findings. Combination therapy with corticosteroid and propranolol can be considered for KMP treatment.


Assuntos
Celulite (Flegmão)/complicações , Síndrome de Kasabach-Merritt/complicações , Celulite (Flegmão)/tratamento farmacológico , Humanos , Lactente , Síndrome de Kasabach-Merritt/tratamento farmacológico , Masculino
3.
J Craniofac Surg ; 31(4): 1074-1077, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32176003

RESUMO

Kaposiform hemangioendothelioma is an aggressive vascular tumor with infiltrative growth that commonly occurs in infancy and is associated with a life-threatening consumptive coagulopathy, as well as Kasabach-Merritt phenomenon. Recently, promising results have shown that sirolimus had been successfully used to treat Kasabach-Merritt phenomenon without significant toxicity. However, the situation the authors encountered in treating infants was not so satisfactory. Here, the authors present 2 patients younger than 3 months with refractory Kaposiform hemangioendothelioma treated with sirolimus and experienced severe pneumonia. The outcomes suggest that it is necessary to keep an eye on any symptoms indicate the infection of respiratory tract and use the antibiotics in time. The 2 cases also remind us of the potential sign that indicate the recurrence of KMP, which refers to firmer lesion with deepen color, especially when it comes with complications.


Assuntos
Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/uso terapêutico , Transtornos da Coagulação Sanguínea/complicações , Hemangioendotelioma/complicações , Humanos , Lactente , Recém-Nascido , Síndrome de Kasabach-Merritt/complicações , Masculino , Sarcoma de Kaposi/complicações , Neoplasias Vasculares
5.
Dermatology ; 236(3): 262-270, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31896113

RESUMO

BACKGROUND: Mammalian target of rapamycin (mTOR) inhibitors have been shown to have excellent effects in the management of kaposiform hemangioendothelioma (KHE); however, the mechanism of action is unclear. This study identified the expressions of mTOR pathway-related proteins in different vascular tumors to provide insight into the pathogenesis of KHE. METHODS: We retrospectively reviewed the pathologic specimens of 30 patients (KHE, 15; tufted angioma [TA], 5; infantile hemangioma [IH], 5; and lymphatic malformation [LM], 5). The immunohistochemical expression of mTOR-related proteins tuberous sclerosis complex 2 (TSC2), phosphatase and tensin homologue (PTEN), phosphorylated eukaryotic translation initiation factor 4E binding protein 1 (p-4EBP1), phosphorylated mTOR (p-mTOR), and phosphorylated ribosomal protein S6 kinase B1 (p-P70S6K) were analyzed using Image-Pro Plus software. KHE had the following pattern of expression in the spindle vascular endothelial cells: TSC2 (-); PTEN (-); p-4EBP1 (+); p-mTOR (+); and p-P70S6K (+). RESULTS: All 3 patients treated with sirolimus had good responses. The TA results were similar to KHE with no significant differences (p-4EBP1: p = 0.0687; p-mTOR: p = 0.0832). The expressions of TSC2, PTEN, p-4EBP1, p-mTOR, and p-P70S6K were negative or weakly positive in IH with a statistically significant difference compared to KHE (p-4EBP1: p < 0.001; p-mTOR: p < 0.001; p-P70S6K: p < 0.001). LM had no significant differences when compared to KHE. CONCLUSIONS: The absence of TSC2 and PTEN caused abnormal activation of the mTOR signaling pathway and may be involved in the pathogenesis of KHE. The expression of mTOR-related proteins in TA and LM was similar to KHE, unlike IH. The KHE pattern of expression [PTEN (-), TSC2 (-), p-mTOR (+), p-P70S6K (+), and p-4EBP1 (+)] suggested that sirolimus may be a good therapeutic choice.


Assuntos
Hemangioendotelioma/metabolismo , Imuno-Histoquímica/métodos , Síndrome de Kasabach-Merritt/metabolismo , Sarcoma de Kaposi/metabolismo , Serina-Treonina Quinases TOR/biossíntese , Antineoplásicos/uso terapêutico , Células Endoteliais/metabolismo , Hemangioendotelioma/tratamento farmacológico , Hemangioma/metabolismo , Humanos , Síndrome de Kasabach-Merritt/tratamento farmacológico , Anormalidades Linfáticas/metabolismo , Estudos Retrospectivos , Sarcoma de Kaposi/tratamento farmacológico , Transdução de Sinais , Sirolimo/uso terapêutico , Serina-Treonina Quinases TOR/metabolismo
6.
J Pediatr Hematol Oncol ; 42(1): 74-78, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-30044355

RESUMO

Kaposiform hemangioendothelioma (KHE) is a rare infiltrative vascular tumor that may be associated with Kasabach-Merritt Phenomenon (KMP), which is a consumptive coagulopathy with potentially life-threatening thrombocytopenia. Management of KHE and KMP is challenging, and currently, there are no standardized validated treatment protocols. Mammalian target of rapamycin inhibitors have been shown to be effective in the treatment of KHE. We describe a term male who presented as a diagnostic dilemma with life-threatening pleural and pericardial effusions and severe thrombocytopenia. After extensive work-up the etiology for his condition was determined to be KHE with KMP. The patient was commenced on sirolimus and responded well to therapy with resolution of KMP.


Assuntos
Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Derrame Pericárdico/tratamento farmacológico , Derrame Pleural Maligno/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/administração & dosagem , Hemangioendotelioma/diagnóstico , Humanos , Recém-Nascido , Síndrome de Kasabach-Merritt/diagnóstico , Masculino , Derrame Pericárdico/diagnóstico , Derrame Pleural Maligno/diagnóstico , Sarcoma de Kaposi/diagnóstico
7.
Am J Case Rep ; 20: 1923-1929, 2019 Dec 23.
Artigo em Inglês | MEDLINE | ID: mdl-31866667

RESUMO

BACKGROUND Kaposiform hemangioendothelioma is a rare locally aggressive vascular endothelial-derived spindle cells neoplasm. Herein, we report a rare case of bifocal tibial kaposiform hemangioendothelioma. CASE REPORT A 9-year-old female presented with a 2-year history of pain and swelling in the left leg. The patient had a high plasma level of the D-dimer and fibrinogen. Radiography revealed a centric lytic lesion on the left proximal tibial metaphysis and an eccentric lateral distal tibial metaphyseal. Histopathologic examination of the sample taken from the distal tibia revealed a dense spindle cell tumor with lobular architecture composed of compact spindle cells compressing small slit-like vascular spaces, forming glomeruloid nests. No necrosis was identified. Based on these findings and the positive immunohistochemical staining for CD31, CD34, and D2-40, the patient was diagnosed with kaposiform hemangioendothelioma. Treatment was started by using vincristine chemotherapy, after which the patient developed temporary peroneal neuropathy, which improved over the next 3 months. CONCLUSIONS Bifocal tibial kaposiform hemangioendothelioma lesions are unique in pediatric patients and can be successfully treated with vincristine chemotherapy. In these cases, the treating physician should be aware of peroneal neuropathy as a potential complication of vincristine administration.


Assuntos
Antineoplásicos Fitogênicos/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Tíbia/patologia , Vincristina/uso terapêutico , Neoplasias Ósseas/diagnóstico por imagem , Criança , Feminino , Hemangioendotelioma/diagnóstico por imagem , Humanos , Síndrome de Kasabach-Merritt/diagnóstico por imagem , Imagem por Ressonância Magnética , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Radiografia , Sarcoma de Kaposi/diagnóstico por imagem , Tíbia/diagnóstico por imagem , Vincristina/efeitos adversos
8.
J Dermatol ; 46(11): 956-961, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31489702

RESUMO

Mammalian target of rapamycin inhibitors have shown promising results in the management of kaposiform hemangioendothelioma (KHE). The purpose of this study was to present our experience involving sirolimus therapy for KHE. A retrospective study was conducted to review the medical documents of 26 patients with KHE who were treated with sirolimus at our hospital between March 2012 and December 2016. Fifteen males and 11 females manifested KHE in infancy with an average age of 2.9 ± 1.8 months. Multiple anatomical sites were involved. Four patients had multifocal lesions, while 22 patients had solitary lesions. Twenty-five patients had Kasabach-Merritt phenomenon (KMP). Twenty patients completed sirolimus therapy in 28.3 ± 12.5 months. Nineteen KHE lesions reduced to small residuals with platelet counts reaching normal levels 3.7 ± 2.8 weeks after treatment; one KHE lesion had no response to therapy. One patient with multifocal lesions died due to a severe infection, although the patient had previously responded to sirolimus. Five patients remained in treatment and had good responses with normal platelet counts. Nineteen patients with anemia had normal hemoglobin levels after 3.5 ± 1.9 weeks of treatment. Mild side effects were observed. The median follow-up time was 32 months (26-60 months), with no evidence of recurrences. Sirolimus was shown to be efficacious in the management of KHE with an average course of 28 months. The time-to-response was variable, with an average of 1 week. After 4 weeks of treatment, the platelet count and hemoglobin level had normalized. Multifocal KHE with KMP is more severe than solitary KHE.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/uso terapêutico , Feminino , Seguimentos , Hemangioendotelioma/patologia , Humanos , Lactente , Recém-Nascido , Síndrome de Kasabach-Merritt/patologia , Masculino , Estudos Retrospectivos , Sarcoma de Kaposi/patologia
9.
J Dermatol ; 46(10): 898-901, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31373046

RESUMO

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare infiltrative vascular tumors. Currently, no standard treatment regimens exist for KHE/TA. The purpose of our study was to evaluate the efficacy and safety of topical application of tacrolimus for superficial KHE/TA. We examined six patients with superficial KHE/TA. All patients were treated with tacrolimus 0.1% ointment twice daily for at least 12 months. The response rate was 100%, including three nearly complete remissions. Only one patient experienced local pruritus during treatment. The data constituted an intriguing rationale for clinical trials of topical tacrolimus in the treatment of superficial KHE/TA.


Assuntos
Hemangioendotelioma/tratamento farmacológico , Hemangioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Tacrolimo/administração & dosagem , Administração Cutânea , Biópsia , Pré-Escolar , Feminino , Hemangioendotelioma/diagnóstico por imagem , Hemangioendotelioma/patologia , Hemangioma/diagnóstico por imagem , Hemangioma/patologia , Humanos , Lactente , Síndrome de Kasabach-Merritt/diagnóstico por imagem , Síndrome de Kasabach-Merritt/patologia , Masculino , Uso Off-Label , Pomadas , Fotografação , Prurido/induzido quimicamente , Sarcoma de Kaposi/diagnóstico por imagem , Sarcoma de Kaposi/patologia , Pele/diagnóstico por imagem , Pele/efeitos dos fármacos , Pele/patologia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/patologia , Tacrolimo/efeitos adversos , Resultado do Tratamento
10.
Pediatr Blood Cancer ; 66(8): e27790, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31045327

RESUMO

BACKGROUND: Kaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly with significant morbidity and mortality. KLA is characterized by diffuse multifocal lesions comprised of focal areas of "kaposiform" spindled cells accompanying malformed lymphatic channels. The goal of this study was to identify activated signaling pathways in cells isolated from three KLA patients for the purpose of testing new therapies. PROCEDURE: Cells were obtained from the lungs of one patient isolated at autopsy and the spleen of two patients removed in surgery due to disease complications. A protein kinase array was performed on the KLA cell lysates and normal lymphatic endothelial cells. RESULTS: Higher activation of key signaling pathways in the KLA cells, including PRAS40, AKT1/2/3, and ERK-1/2, was identified by protein kinase array and confirmed by Western blot analysis. This indicated a role for highly activated PI3K-AKT and MAPK-ERK-1/2 signaling pathways in KLA cells. Cell proliferation studies assessed PI3K inhibitors (LY294002; BYL719), AKT inhibitor ARQ092, mTOR inhibitor rapamycin, and MAPK inhibitor U0126. These studies demonstrated that PI3K-AKT-mTOR and MAPK signaling are important mediators of KLA cell proliferation. BYL719 and rapamycin were more effective at inhibiting KLA cell proliferation than U0126. CONCLUSIONS: Our studies using cells from KLA patient lesions demonstrate that these cells are highly proliferative and the PI3K-AKT-mTOR and MAPK pathways are promising therapeutic targets. Development and clinical trials of PI3K, AKT, and MAPK inhibitors for cancer treatment and the data in this study lend support for early clinical trials assessing the efficacy of these inhibitors in KLA patients.


Assuntos
Antineoplásicos/farmacologia , Hemangioendotelioma/patologia , Síndrome de Kasabach-Merritt/patologia , Linfangioma/patologia , Sistema de Sinalização das MAP Quinases/efeitos dos fármacos , Fosfatidilinositol 3-Quinases/química , Proteínas Proto-Oncogênicas c-akt/antagonistas & inibidores , Sarcoma de Kaposi/patologia , Serina-Treonina Quinases TOR/antagonistas & inibidores , Adolescente , Proliferação de Células/efeitos dos fármacos , Pré-Escolar , Feminino , Seguimentos , Hemangioendotelioma/tratamento farmacológico , Hemangioendotelioma/metabolismo , Humanos , Lactente , Síndrome de Kasabach-Merritt/tratamento farmacológico , Síndrome de Kasabach-Merritt/metabolismo , Linfangioma/tratamento farmacológico , Linfangioma/metabolismo , Masculino , Inibidores de Proteínas Quinases/farmacologia , Estudos Retrospectivos , Sarcoma de Kaposi/tratamento farmacológico , Sarcoma de Kaposi/metabolismo , Transdução de Sinais/efeitos dos fármacos , Células Tumorais Cultivadas
13.
Hematol Oncol Clin North Am ; 33(3): 455-470, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31030813

RESUMO

Vascular anomalies consist of a diverse group of disorders that are broadly categorized as tumors and malformations. . Recently, there has been significant genomic discovery allowing phenotype/genotype correlation of disease. An increasing number of pediatric hematologists/oncologists are caring for individuals with vascular anomalies as these patients require chronic care and have high medical acuity needs. The advent of new medical therapy options, along with ongoing and upcoming clinical trials, makes the involvement of hematologists/oncologists essential. This article highlights diagnosis and management of complicated vascular anomalies as well as important new treatment options and discoveries.


Assuntos
Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/uso terapêutico , Serina-Treonina Quinases TOR/metabolismo , Malformações Vasculares/tratamento farmacológico , Antibióticos Antineoplásicos/uso terapêutico , Criança , Hemangioendotelioma/patologia , Humanos , Síndrome de Kasabach-Merritt/patologia , Sarcoma de Kaposi/patologia , Transdução de Sinais/efeitos dos fármacos , Malformações Vasculares/patologia
14.
J Int Med Res ; 47(4): 1810-1814, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30806107

RESUMO

OBJECTIVE: Kasabach-Merritt syndrome is a rare disease that mainly occurs in infants and adolescents. It usually manifests as disseminated intravascular coagulation and severe bleeding, and is associated with high mortality. However, its low incidence and clinical rarity in adults mean that there is currently no well-verified treatment regimen for this disease. We report on an effective novel therapeutic regimen in a patient with Kasabach-Merritt syndrome. METHODS: A woman with Kasabach-Merritt syndrome presented with a recurrent subcutaneous mass and disseminated intravascular coagulation, and was treated with prednisone, vincristine and thalidomide. RESULTS: This treatment regimen successfully resolved the patient's symptoms, with tumor regression. The patient remained disease-free after 6 years of follow-up. CONCLUSIONS: Prednisone combined with vincristine and thalidomide may be an effective treatment for Kasabach-Merritt syndrome, but further studies are needed to verify the use of this regimen.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Adulto , Feminino , Humanos , Síndrome de Kasabach-Merritt/patologia , Prednisona/administração & dosagem , Prognóstico , Talidomida/administração & dosagem , Vincristina/administração & dosagem
17.
Eur J Pediatr Surg ; 29(5): 401-407, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30372769

RESUMO

OBJECTIVE: To evaluate the outcome and safety of corticosteroids and vincristine (VCR) in the treatment of kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). MATERIALS AND METHODS: Clinical studies involving corticosteroids and VCR therapies in treating KHE/TA were identified by using PubMed, Cochrane Library, OVID, EBSCO, CNKI, VIP, and Wanfang databases from their establishment date to December 2017. Randomized controlled trials, case-control, or case series with more than five cases were included. The following data were extracted: study sample, demographics, responses rate, recurrence rate, and adverse reactions. Two reviewers completed screening and extraction. Methodological quality was evaluated with quality appraisal tool. RESULTS: A total of 266 studies were found, and 27 studies were finally included in this research; quality of all studies was low. Seven studies with a total of 123 participants, which compared the effect of systemic corticosteroids with that of VCR, were performed for the meta-analysis. The results indicated that the effect of VCR was significantly higher than that of corticosteroids (relative risk [RR] = 2.08, 95% confidence interval [CI]: 1.38-3.16). The recurrence rate of VCR (11.1%) was lower than that of corticosteroids (50%), but there was no statistical difference between the two therapies (p = 0.1312). The result of pooled adverse reactions response rate for VCR was 18.2%, significantly lower than that for corticosteroids, which was 52.0%. CONCLUSION: The present profile shows that VCR is relatively more effective and safer in treating KHE/TA than corticosteroids are. So, we believe VCR could be used as a first-line medication agent in the treatment of KHE/TA.


Assuntos
Corticosteroides/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Hemangioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Vincristina/uso terapêutico , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Observacionais como Assunto , Recidiva , Resultado do Tratamento
18.
J Dermatol ; 45(10): 1203-1206, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30118141

RESUMO

Kasabach-Merritt phenomenon (KMP) occurred uniquely in patients with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). We report the clinical characteristics of two patients with KHE involving the right upper arm. The patients demonstrated rapid enlargement of the lesion with severe KMP shortly after vaccination. Sirolimus was used to treat the KHE with KMP. The patients showed a quick normalization of the platelet level. The follow-up examination revealed that the size of the mass was significantly decreased. This report raises the intriguing possibility that extrinsic factors may contribute to the development of KMP in the context of an already existing KHE.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Hemangioendotelioma/diagnóstico , Síndrome de Kasabach-Merritt/diagnóstico , Sarcoma de Kaposi/diagnóstico , Sirolimo/uso terapêutico , Vacinação/efeitos adversos , Vacina BCG/efeitos adversos , Vacinas contra Difteria, Tétano e Coqueluche Acelular/efeitos adversos , Feminino , Hemangioendotelioma/sangue , Hemangioendotelioma/tratamento farmacológico , Hemangioendotelioma/etiologia , Humanos , Lactente , Síndrome de Kasabach-Merritt/sangue , Síndrome de Kasabach-Merritt/tratamento farmacológico , Síndrome de Kasabach-Merritt/etiologia , Imagem por Ressonância Magnética , Masculino , Sarcoma de Kaposi/sangue , Sarcoma de Kaposi/tratamento farmacológico , Sarcoma de Kaposi/etiologia , Resultado do Tratamento
19.
Pediatr Blood Cancer ; 65(12): e27305, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30070028

RESUMO

A full-term newborn with kaposiform hemangioendothelioma (KHE) affecting the right thigh with thrombocytopenia due to Kasabach-Merritt phenomenon (KMP) was referred to our center. After biopsy, he rapidly evolved to severe thrombocytopenia and severe coagulopathy. Standard therapy was initiated with prednisolone and vincristine. His coagulopathy worsened to life-threatening hemorrhage necessitating aggressive blood products replacement. Sirolimus was added; he became transfusion independent with no further bleeding and reduction in tumor size. Addition of sirolimus to treatment of vascular anomalies with hemostatic complications should be considered as part of early treatment for patients with KMP/KHE.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Hemangioendotelioma/tratamento farmacológico , Hemangioendotelioma/patologia , Síndrome de Kasabach-Merritt/tratamento farmacológico , Síndrome de Kasabach-Merritt/patologia , Sarcoma de Kaposi/tratamento farmacológico , Sarcoma de Kaposi/patologia , Transtornos da Coagulação Sanguínea/sangue , Transtornos da Coagulação Sanguínea/tratamento farmacológico , Transtornos da Coagulação Sanguínea/patologia , Criança , Hemangioendotelioma/sangue , Humanos , Síndrome de Kasabach-Merritt/sangue , Masculino , Prednisolona/administração & dosagem , Sarcoma de Kaposi/sangue , Sirolimo/administração & dosagem , Trombocitopenia/sangue , Trombocitopenia/tratamento farmacológico , Trombocitopenia/patologia , Vincristina/administração & dosagem
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...