RESUMEN
Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disease that causes severe bleeding. The pathogenesis and treatment of AAMT have not yet been defined. We report the case of a 60-year-old woman diagnosed with AAMT, who presented with severe thrombocytopenia, gastroin-testinal bleeding, and significantly reduced bone marrow megakaryocytes. The patient was treated with methylprednisolone, cyclosporin, and intravenous immunoglobulin. After 2 weeks of treatment, her platelet count started to increase, and her bone marrow megakaryocyte count had normalized 3 months after diagnosis. At the time of diagnosis, the patient was seropositive for anti-c-mpl antibody but was seen to be seronegative once the platelet count recovered. In contrast, anti-c-mpl antibodies were not detected in the serum of 3 patients with idiopathic thrombocytopenic purpura. This case study suggests that anti-c-mpl antibody plays an important role in the development of AAMT, and that intensive immunosuppressive treatment is required for autoantibody clearance and recovery of megakaryocyte count.
Asunto(s)
Autoanticuerpos/sangre , Enfermedades de la Médula Ósea , Ciclosporina/administración & dosificación , Inmunoglobulinas Intravenosas/administración & dosificación , Metilprednisolona/administración & dosificación , Púrpura Trombocitopénica , Receptores de Trombopoyetina , Células de la Médula Ósea/metabolismo , Enfermedades de la Médula Ósea/sangre , Enfermedades de la Médula Ósea/diagnóstico , Enfermedades de la Médula Ósea/tratamiento farmacológico , Femenino , Hemorragia Gastrointestinal/sangre , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/tratamiento farmacológico , Humanos , Megacariocitos/metabolismo , Persona de Mediana Edad , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/diagnóstico , Púrpura Trombocitopénica/tratamiento farmacológicoRESUMEN
Essentials Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. Surgery is a possible trigger of acute TTP episodes and no guidelines are available. Six patients with severe ADAMTS-13 deficiency during remission underwent elective surgery. Patients were prophylactically treated to restore ADAMTS-13 activity and no relapses occurred. SUMMARY: Background Severe ADAMTS-13 deficiency has been recognized as the main risk factor for recurrence of thrombotic thrombocytopenic purpura (TTP). Several conditions, including surgery, may influence the levels of ultra-large von Willebrand factor and ADAMTS-13, acting as a trigger for an acute TTP event. Objectives To report our experience of management of six patients with acquired TTP who underwent elective surgery after prophylactic treatment to restore ADAMTS-13 activity levels. Patients Six patients followed for acquired TTP with severe ADAMTS-13 deficiency during remission were candidates for seven elective surgeries (inguinal hernioplasty, cholecystectomy, laparoscopic hysterectomy, oophorectomy, parotidectomy and two total hip arthroplasties). Results Four patients were treated with prophylactic plasma exchange (PEX) therapy immediately before surgery. One patient was treated with PEX therapy before her first surgery and with preemptive rituximab once her second surgery was scheduled. Because rituximab increased ADAMTS-13 levels only partially, she required one PEX procedure the day before her second surgery. One patient was treated with azathioprine after rituximab failure, obtaining a progressive increase of ADAMTS-13 activity to more than 40%. This level allowed her to undergo total hip arthroplasty without additional treatment. All surgeries were successful and no complications or relapses occurred. Conclusions Six patients with acquired TTP underwent seven successful surgical procedures using prophylaxis to restore ADAMTS-13 activity. Further observational studies or randomized clinical trials are needed to confirm whether prophylactic PEX could be the key factor in preventing relapse.
Asunto(s)
Proteína ADAMTS13/sangre , Procedimientos Quirúrgicos Electivos/efectos adversos , Inmunosupresores/administración & dosificación , Intercambio Plasmático , Púrpura Trombocitopénica/prevención & control , Prevención Secundaria/métodos , Proteína ADAMTS13/deficiencia , Adulto , Anciano , Biomarcadores/sangre , Femenino , Humanos , Inmunosupresores/efectos adversos , Masculino , Persona de Mediana Edad , Intercambio Plasmático/efectos adversos , Factores Protectores , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/etiología , Recurrencia , Factores de Riesgo , Resultado del Tratamiento , Regulación hacia ArribaRESUMEN
INTRODUCTION: We have developed hemoglobin vesicles (HbVs) as a substitute for red blood cells (RBCs). We investigated the efficacy of HbV transfusion in the treatment of massive hemorrhage in rabbits in the setting of thrombocytopenic coagulopathy, focusing on the efficacy of hemostasis by subsequent platelet transfusion. METHODS: Thrombocytopenic coagulopathy was induced in rabbits by repeated blood withdrawal and isovolemic retransfusion of autologous RBC (platelet counts <45,000/µL). A penetrating liver injury was then made. For 30âmin, bleeding volume was measured every 10âmin, after which subjects were transfused with an equivalent volume of stored RBC, HbV, or platelet poor plasma (PPP) to compensate for blood loss, simulating initial prehospital resuscitation. Thereafter, we transfused platelet rich plasma (PRP) to stop bleeding, which simulated inhospital resuscitation. RESULTS: During the initial resuscitation, the HbV group was similar to the RBC group (but not the PPP group) in their hemodynamics and tissue circulation/oxygenation as assessed by plasma lactate levels. All rabbits showed similar bleeding volumes (20-30âmL) in this period. HbV-transfused rabbits sustained hemoglobin levels, but showed lower hematocrit levels compared with RBC-transfused rabbits. Subsequent PRP transfusion effectively stopped bleeding in all RBC-transfused rabbits (6/6) and most HbV-transfused rabbits (7/8) but not PPP-transfused rabbits (2/8). In addition, 83% of RBC-transfused rabbits and 75% of HbV-transfused rabbits survived for 24âh, although no PPP-transfused rabbits survived. HbV transfusion did not scavenge nitric oxide in rabbits. CONCLUSIONS: HbV transfusion effectively rescued rabbits from severe hemorrhage with coagulopathy, without disturbing hemostasis after the platelet transfusion. HbV transfusion may be practical and useful in prehospital resuscitation.
Asunto(s)
Hemoglobinas/farmacología , Hemorragia , Homeostasis/efectos de los fármacos , Transfusión de Plaquetas , Púrpura Trombocitopénica , Animales , Hemorragia/sangre , Hemorragia/terapia , Masculino , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/terapia , ConejosRESUMEN
BACKGROUND: Conventional or homeopathic treatment of chronic immune thrombocytopenic purpura (ITP) is often difficult. The use of homeopathic dilutions of patient blood (HPB) for immunomodulation has been described, which inspired us to try the method in an ITP case. CASE REPORT: A 2-year-old girl with chronic ITP was treated with homeopathic dilutions of her own capillary blood, given orally over 5 months. Immediately after treatment onset there was a rapid normalization of the thrombocyte counts. Within 6 weeks, they rose from 15,000/µl to 254,000/µl. After treatment stop, they decreased to 155,000/µl, increased again spontaneously to 270,000/µl and remained within normal range for over 3 years. CONCLUSIONS: Oral administration of homeopathic dilutions of capillary patient blood may possibly be an effective treatment in chronic ITP. If our results can be reproduced, this will revolutionize the treatment of ITP.
Asunto(s)
Homeopatía/métodos , Púrpura Trombocitopénica/terapia , Administración Oral , Preescolar , Femenino , Citometría de Flujo , Humanos , Proyectos Piloto , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Remisión EspontáneaRESUMEN
We report the first case of a patient with hepatitis C virus (HCV) infection and idiopathic thrombocytopenic purpura (ITP), who later developed acquired amegakaryocytic thrombocytopenia (AAMT), with autoantibodies to the thrombopoietin (TPO) receptor (c-Mpl). A 64-year-old woman, with chronic hepatitis C, developed severe thrombocytopenia and was diagnosed with ITP. She died of liver failure. Autopsy revealed cirrhosis and liver carcinoma. In the bone marrow, a marked reduction in the number of megakaryocytes was observed, while other cell lineages were preserved. Therefore, she was diagnosed with AAMT. Additionally, autoantibodies to c-Mpl were detected in her serum. Autoantibodies to c-Mpl are one of the causes of AAMT, acting through inhibition of TPO function, megakaryocytic maturation, and platelet formation. HCV infection induces several autoantibodies. HCV infection might also induce autoantibodies to c-Mpl, resulting in the development of AAMT. This mechanism may be one of the causes of thrombocytopenia in patients with HCV infection.
Asunto(s)
Autoanticuerpos/sangre , Enfermedades de la Médula Ósea/diagnóstico , Hepatitis C Crónica/inmunología , Fallo Hepático/sangre , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica/diagnóstico , Autoanticuerpos/inmunología , Médula Ósea/patología , Células de la Médula Ósea/patología , Enfermedades de la Médula Ósea/sangre , Enfermedades de la Médula Ósea/inmunología , Enfermedades de la Médula Ósea/patología , Diagnóstico Diferencial , Resultado Fatal , Femenino , Hepacivirus/aislamiento & purificación , Hepatitis C Crónica/sangre , Hepatitis C Crónica/virología , Humanos , Hígado/inmunología , Hígado/patología , Fallo Hepático/etiología , Fallo Hepático/inmunología , Fallo Hepático/patología , Megacariocitos/patología , Persona de Mediana Edad , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/inmunología , Púrpura Trombocitopénica/patología , Púrpura Trombocitopénica Idiopática/sangre , Receptores de Trombopoyetina/inmunología , Trombopoyetina/metabolismoAsunto(s)
Carbamazepina/efectos adversos , Genotipo , Polimorfismo de Nucleótido Simple , Púrpura Trombocitopénica , Carbamazepina/administración & dosificación , Niño , Femenino , Humanos , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/inducido químicamente , Púrpura Trombocitopénica/genética , Púrpura Trombocitopénica/patologíaRESUMEN
Kikuchi-Fujimoto disease is a mild and rare idiopathic disease, particularly in children. It is mostly characterized by painful cervical lymphadenopathy and/or prolonged fever and confirmed by histology. We report a case of Kikuchi-Fujimoto disease in a 14-year-old teenager with high procalcitonin concentration and thrombocytopenic purpura.
Asunto(s)
Calcitonina/sangre , Linfadenitis Necrotizante Histiocítica/sangre , Linfadenitis Necrotizante Histiocítica/complicaciones , Precursores de Proteínas/sangre , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/etiología , Adolescente , Péptido Relacionado con Gen de Calcitonina , Humanos , MasculinoRESUMEN
Acquired amegakaryocytic thrombocytopenia (AAT) is a hematologic disorder that presents as thrombocytopenia with absent megakaryocytes in the bone marrow. Causes of AAT include toxins, drugs, viral infections, systemic lupus erythematosus, and cytokine deficiencies. Patients with AAT should be followed for possible progression to aplastic anemia or myelodysplastic syndrome. We present a case of a 61-year-old woman with AAT due to occupational chemical exposure.
Asunto(s)
Enfermedades de la Médula Ósea/inducido químicamente , Enfermedades Profesionales/sangre , Exposición Profesional/efectos adversos , Púrpura Trombocitopénica/inducido químicamente , Recuento de Células Sanguíneas , Médula Ósea/patología , Enfermedades de la Médula Ósea/sangre , Detergentes/efectos adversos , Femenino , Humanos , Persona de Mediana Edad , Recuento de Plaquetas , Transfusión de Plaquetas , Púrpura Trombocitopénica/sangreRESUMEN
The Platelet (PLT) Transfusion Therapy plays an important role in the support of surgical, haematological, oncological and transplant patients. The present study was assigned to find out the post transfusion increment of platelet count among the thrombocytopenic patients in Bangladeshi population. This descriptive study was conducted at the Departments of Haematology and Transfusion Medicine, BSMMU, Dhaka. Total 42 thrmbocytopenic patients were randomly assigned to receive a transfusion when their platelet counts below 10000 per cubic millimeter or with active bleeding. Pre transfusion and post transfusion platelet count were measured in all patients. Out of 42 patients, 26(61.90%) were male and 16(38.10%) were female. Leukemia was the most common cause of thrombocytopenia (47.62%). Most of the patients (71.34%) required transfusion of multiple units of platelet and 12(28.57%) patients required double units. Before transfusion of platelet concentrate <30×108/L, 30-80×108/L and >80×108/L platelet count were found in 30(71.43%), 08(19.05%) and 04(09.52%) patients respectively. After transfusion of platelet concentrate <50×108/L, 50-100×108/L and >100×108/L platelet count were found in 30(71.43%), 08(19.05%) and 04(09.52%) patients respectively. In all patients post transfusion platelet count increases but 2 or multiple units of transfusion were needed.
Asunto(s)
Hemorragia/terapia , Transfusión de Plaquetas , Plasma Rico en Plaquetas , Trombocitopenia/terapia , Adulto , Antineoplásicos/efectos adversos , Bangladesh , Dengue/complicaciones , Femenino , Hemorragia/sangre , Hemorragia/etiología , Humanos , Leucemia/complicaciones , Masculino , Persona de Mediana Edad , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/etiología , Púrpura Trombocitopénica/terapia , Trombocitopenia/sangre , Trombocitopenia/etiología , Resultado del Tratamiento , Adulto JovenRESUMEN
Some thrombopoietin receptor-agonists (TPR-As) have been developed and shown to be highly effective in the treatment of immune thrombocytopenic purpura (ITP). Soluble cytotoxic T-lymphocyte-associated antigen 4 (sCTLA-4) can modulate and terminate the immune response. Several reports have shown that sCTLA-4 levels are elevated in patients with some autoimmune disorders. However, sCTLA-4 levels have not previously been investigated in TPR-A exposed patients with ITP. We investigated the levels of transforming growth factor (TGF) ß(1) and sCTLA-4 in ITP patients to determine the clinical association with TGFß(1) and sCTLA-4 in TPR-A-exposed patients with ITP. Thirty-seven ITP patients were divided into 2 groups (TPR-A-exposed: 13 patients; unexposed: 24 patients). Doses of eltrombopag ranging from 12.5mg to 50mg were administered daily, and biochemical data obtained before and after eltrombopag administration were compared. Eltrombopag therapy significantly increased sCTLA-4 and TGFß(1) levels relative to baseline in patients with ITP. In addition, plasma TGFß(1) was positively correlated with platelet counts and sCTLA-4 in patients with ITP in the eltrombopag-exposed group. However, no significant change in the detection rate for anti-glycoprotein antibody was observed before and 24 weeks after eltrombopag treatment. These results suggest that eltrombopag can partially modulate some immune responses by TGFß(1) and sCTLA-4, but it does not induce immune tolerance by 24 weeks after treatment.
Asunto(s)
Benzoatos/administración & dosificación , Antígeno CTLA-4/sangre , Hidrazinas/administración & dosificación , Púrpura Trombocitopénica/tratamiento farmacológico , Púrpura Trombocitopénica/inmunología , Pirazoles/administración & dosificación , Receptores de Trombopoyetina/agonistas , Factor de Crecimiento Transformador beta1/sangre , Relación Dosis-Respuesta a Droga , Femenino , Humanos , Masculino , Persona de Mediana Edad , Púrpura Trombocitopénica/sangre , Resultado del TratamientoRESUMEN
UNLABELLED: Laparoscopic splenectomy (LS) is emerging as the treatment of choice over open splenectomy (OS) in cases of idiopathic (immune) thrombocytopenic purpura (ITP) that is either steroid resistant or steroid unresponsive.The aim of the present study is to compare therapeutic response and outcome of patients with ITP undergoing LS with a similar group undergoing OS.The study was performed on 63 patients with ITP attending Medical College and Hospital, Kolkata, a tertiary level referral centre in Eastern India during 2005-2009.Twenty-seven patients with ITP underwent LS and the rest underwent OS. Twenty-eight patients were steroid resistant whereas the rest were diagnosed to be refractory to steroids. Parameters assessed were demographic characteristics of patients, peri-operative data, complications and haematological outcome.The patients were followed up for a mean period of 40 months. RESULTS: Demographic characteristics of the patients in LS and OS group were comparable. Patients undergoing LS were found to require a longer operative time but had lower intra-operative blood loss, less postoperative pain, decreased incidence of complications, reduced hospital stay and comparable haematological response as compared to that for patients undergoing OS. LS for ITP is a safe technique associated with lower morbidity and faster convalescence and similar haematological response when compared to OS.
Asunto(s)
Púrpura Trombocitopénica/cirugía , Esplenectomía/métodos , Adolescente , Adulto , Pérdida de Sangre Quirúrgica , Femenino , Humanos , India , Laparoscopía , Tiempo de Internación , Masculino , Persona de Mediana Edad , Tempo Operativo , Dolor Postoperatorio/etiología , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Esplenectomía/efectos adversos , Resultado del Tratamiento , Adulto JovenRESUMEN
Seven patients with chronic refractory immune thrombocytopenic purpura (ITP) received adipose tissue-derived mesenchymal stem cells (AMSC) from haplo-identical family donors. The AMSC dose was 2.0×10(6)/kg. No side effects were noted after the AMSC infusions. Overall responses were reached in all patients and sustained response rate was 57.1% (4/7). The serum levels of transforming growth factor ß1 (TGF-ß1), interleukin (IL)-4, and IL-10 were significantly elevated, whereas those of interferon-γ (IFN-γ) and IL-2 were significantly decreased after AMSC administration, compared with those in the patients with active ITP. During follow-up, the cytokine profiles in patients maintaining sustained response remained stable compared with the post-treatment level, but IFN-γ and IL-2 levels were significantly increased, and those of TGF-ß1, IL-4, and IL-10 were significantly reduced again in relapsed patients. AMSC therapy seems to represent reasonable salvage treatment in severe, chronic refractory ITP by causing a shift in the Th1/Th2 cytokine balance to the same levels as normal controls.
Asunto(s)
Trasplante de Células Madre Mesenquimatosas/métodos , Células Madre Mesenquimatosas/metabolismo , Púrpura Trombocitopénica/terapia , Adulto , Enfermedad Crónica/terapia , Femenino , Estudios de Seguimiento , Humanos , Interferón gamma/sangre , Interleucina-10/sangre , Interleucina-2/sangre , Interleucina-4/sangre , Masculino , Células Madre Mesenquimatosas/citología , Persona de Mediana Edad , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/inmunología , Púrpura Trombocitopénica/patología , Grasa Subcutánea Abdominal/citología , Grasa Subcutánea Abdominal/metabolismo , Grasa Subcutánea Abdominal/trasplante , Factor de Crecimiento Transformador beta1/sangre , Adulto JovenRESUMEN
To explore the role of CD72 in the pathogenesis of immune thrombocytopenia (ITP), we detected CD72, Sema4D, IL-2, IL-4, and IFN-γ mRNA expressions and the levels of plasma Sema4D, IL-2, IL-4, IL-6, and IFN-γ in ITP patients (n = 39) and controls (n = 23). The levels of plasma IL-2, IL-4, and IL-6 were assayed by radioimmunoassay, and the levels of plasma IFN-γ and Sema4D were analyzed by enzyme-linked immunosorbent assay. Sema4D, CD72, IL-2, IFN-γ, and IL-4mRNA expressions were analyzed by real-time quantitative reverse-transcription polymerase chain reaction. The expression of CD72 mRNA in ITP patients (n = 23) with active disease was significantly lower than that in patients in remission (p = 0.029) (n = 16) and controls (p = 0.0296) (n = 23). The IFN-γ/IL-4 mRNA (Th1/Th2) expression in ITP patients with active disease and in remission was significantly higher than that in controls (p = 0.0023, p = 0.0125, respectively). The expression of IL-2 mRNA in ITP patients with active disease was significantly lower than that in patients in remission (p = 0.0418) and controls (p = 0.004). The level of plasma IL-2 in ITP patients with active disease was significantly lower than that in patients in remission (p = 0.0029) and controls (p = 0.0101). The levels of plasma IL-4 in ITP patients with active disease and in remission were significantly higher than that of controls (p = 0.0093, p = 0.0053, respectively). CD72 mRNA expression level might correlate with Sema4D mRNA expression in peripheral blood mononuclear cells and level of plasma IL-2 in active ITP patients (p = 0.024 and p = 0.036). Our findings suggest that CD72 might be involved in the pathophysiological process of the ITP disease by increasing B-cell receptor signals.
Asunto(s)
Antígenos CD/genética , Antígenos de Diferenciación de Linfocitos B/genética , Expresión Génica , Interleucina-2/genética , Púrpura Trombocitopénica/genética , ARN Mensajero/genética , Semaforinas/genética , Enfermedad Aguda , Adulto , Anciano , Antígenos CD/sangre , Antígenos de Diferenciación de Linfocitos B/sangre , Estudios de Casos y Controles , Convalecencia , Femenino , Humanos , Interferón gamma/sangre , Interferón gamma/genética , Interleucina-2/sangre , Interleucina-4/sangre , Interleucina-4/genética , Interleucina-6/sangre , Interleucina-6/genética , Masculino , Persona de Mediana Edad , Púrpura Trombocitopénica/sangre , Semaforinas/sangreRESUMEN
The aim of this study was to investigate the expression of Th17 cells and regulatory T (Treg) cells in peripheral blood of patients with immune thrombocytopenia (ITP) and to clarify the role of the Th17/Treg cell ratio imbalance in pathogenesis of ITP. Patients were divided into the pre-treatment group (active group) (n = 38) and post-treatment group (remission group) according to the platelet count and curative effect. Post-treatment group was further divided into remission group (n = 24), partial remission group (n = 10), and non-remission group (n = 4). 30 healthy subjects were enrolled in control group. Flow cytometry was used to detect the percentages of peripheral blood Th17 cells and Treg cells in CD4(+) T cells from ITP patients and controls respectively. The results showed that the percentages of Th17 cells in active group and non-remission group were significantly higher than those in control group (p < 0.05). The percentages of Th17 cells in remission group, partial-remission group were also higher than those in control group, but there were no statistically significant differences between these groups. The percentage of Th17 cells in remission group was lower than that in active group, but there was also no statistically difference between two groups. The percentages of Treg cells in active group, partial-remission group and non-remission group significantly decreased, compared with in control group (p < 0.01). The percentage of Treg cells in remission group was lower than that in control group, but there was no statistically significant difference. The ratio of peripheral blood Th17/Treg cells in active group, partial-remission group and non-remission group was higher, as compared with in control group. The ratio of peripheral blood Th17/Treg cells in remission group was higher than that in control group, but there was no statistically difference between two groups. It is concluded the percentage of Th17 cells and the ratio of Th17/Treg cells are higher in active group. The percentage of Treg cells is low in active group, partial remission and non-remission groups. The imbalance of Th17/Treg ratio may play a critical role in ITP pathogenesis.
Asunto(s)
Púrpura Trombocitopénica/sangre , Linfocitos T Reguladores/inmunología , Células Th17/inmunología , Adolescente , Adulto , Anciano , Recuento de Células Sanguíneas , Estudios de Casos y Controles , Femenino , Citometría de Flujo , Humanos , Masculino , Persona de Mediana Edad , Adulto JovenAsunto(s)
Linfoma de Células B Grandes Difuso/complicaciones , Púrpura Trombocitopénica/complicaciones , Corticoesteroides/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Hemorragia/patología , Hepatomegalia/patología , Humanos , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/fisiopatología , Masculino , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/tratamiento farmacológico , Púrpura Trombocitopénica/fisiopatología , Esplenomegalia/patología , Adulto JovenRESUMEN
AIM: To observe the impact of human serum immunoglobulin treatment of refractory immune thrombocytopenic purpura in clinical efficacy. METHODS: 220 patients met the diagnostic criteria of refractory immune thrombocytopenic purpura, patients were randomly divided into treatment group and control group, 110 patients in each group. Groups were given prednisolone tablets 1 mg/ (kg x d), 2 times/d, oral, taking for 2 weeks, and than gradually reducing, the maintenance to be suspended; all-trans retinoic acid, each 10 mg, 3 times a day, oral. On this basis, the treatment group increases the employing blood immunoglobulin 400 mg/ (kg x d) infusion qd for 7 days. The two groups are 4 weeks for the course of treatment, a therapeutic effect after treatment. RESULTS: The total effective treatment group and control group were 94.56%, 80.91%, statistically significant differences between two groups (P < 0.05). Treatment serum IL-2, IFN-gamma, IL-4, IL-10, TGF-beta1, significant differences compared to other indicators (p < 0.05). Platelet counts after treatment, the rates of increase, effective hemostasis time of the treatment group compared with the control group, significant difference (p < 0.05). CONCLUSION: The impact of human serum immunoglobulin treatment of refractory immune thrombocytopenic purpura significantly shorten the bleeding time, platelet and platelet rise-time return to normal time.
Asunto(s)
Inmunoglobulinas/uso terapéutico , Púrpura Trombocitopénica/tratamiento farmacológico , Adolescente , Adulto , Anciano , Niño , Preescolar , Citocinas/sangre , Femenino , Hemostasis/efectos de los fármacos , Humanos , Inmunoglobulinas/farmacología , Masculino , Persona de Mediana Edad , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Resultado del Tratamiento , Adulto JovenRESUMEN
PURPOSE: Predicting the response to splenectomy in children with immune thrombocytopenic purpura (ITP) continues to be a clinical challenge. The purpose of this study is to identify preoperative predictors of outcome for splenectomy in children with ITP. METHODS: The charts of 19 children who underwent splenectomy for ITP were retrospectively reviewed. Platelet responses to treatment are categorized as complete response (CR, > or =150,000/microL), partial response (PR, > or =50,000 but <150,000/microL), or nonresponse (NR, <50,000/microL). RESULTS: After splenectomy, 13 patients (68%) had CR, 3 (16%) had PR, and 3 (16%) had NR. No correlation existed between CR to splenectomy and any of the following: age, platelet count at diagnosis, last platelet count before splenectomy, platelet count on postoperative day 1, or responses to preoperative intravenous immunoglobulin, WinRho, or Rituximab. However, all 7 patients who had NR to a full course of steroids subsequently had CR to splenectomy. Nonresponse to steroid therapy was directly correlated with CR to splenectomy (P = .01, Fisher's Exact test). Furthermore, postsplenectomy platelet counts were inversely related to peak platelet response to steroids (correlation coefficient = -0.68, P = .01). CONCLUSIONS: Preoperative responsiveness to steroid therapy, as measured by peak platelet count, predicts NR to splenectomy for ITP in children, whereas NR to steroid therapy is highly correlated with CR to splenectomy. These findings challenge the widely held notion that steroid responsiveness portends a favorable outcome after splenectomy.
Asunto(s)
Recuento de Plaquetas , Púrpura Trombocitopénica/cirugía , Esplenectomía , Adolescente , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales de Origen Murino , Antígenos CD20/uso terapéutico , Niño , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Selección de Paciente , Cuidados Preoperatorios , Pronóstico , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/tratamiento farmacológico , Globulina Inmune rho(D)/uso terapéutico , Rituximab , Esteroides/uso terapéutico , Resultado del TratamientoRESUMEN
The aim of our study was to evaluate the influence of steroid therapy on serum cystatin C (cysC) concentrations in patients with acute lymphoblastic leukemia (ALL) and idiopthiás thrombocytopenias purpura (ITP). We studied 17 patients with ITP (girls: boys =5:12, mean age: 7.6 yrs, range between 1 to 17 years) and 18 patients with ALL (girls: boys =6:12, mean age: 6.3 yrs, range between 2 to 17 years). CysC and white blood cell count (WBC) in both group of patients were determined before and after 300 mg/m(2) cumulative dose of steroid therapy. Corticosteroids increased the level of cysC in both groups of patients, however significant increase was found only in ITP patients between pre- and posttreatment values (0.96 +/-0.27 mg/L vs. 1.16 +/- 0.3 mg/L, p =0,02). Pretreatment cysC concentrations were within the reference range in patients with ITP but not with ALL and were significantly higher in ALL patients, than in ITP patients (1.23 +/- 1,12 mg/L vs. 0.96 +/- 0.27 mg/L, p =0,02). Pretreatment WBC of ALL patients were significantly higher than of ITP patients (22.58 G/L, min. 3.5 G/L, max. 102.1 G/L vs. 7.46 G/L, min. 4.8 G/L, max. 12.3 G/L, p =0.03). We have found significant correlation between pretreatment cysC and WBC values in ALL patients (p = 0.04). Although the concentration of cysC may be slightly and reversibly influenced by corticosteroid treatment, cysC is sensitive to detect early and moderate deterioration of GFR in children with cancer.