Immune thrombocytopenic purpura in a patient with renal cell carcinoma

No abstract available.

Pre-treatment and post-treatment changes in platelet indices in patients with immune thrombocytopenia

To investigate if there is a correlation between pre- and post-treatment mean platelet volume [MPV], platelet size deviation width [PDW], and platecrit [PCT] values and to investigate whether we can use them as laboratory parameters to estimate the relapses of immune thrombocytopenia [ITP] patients. Patients with ITP diagnosed at the Hematology Clinic, School of Medicine, Cumhuriyet University, Sivas, Turkey between January 2005 and December 2011 were evaluated by a retrospective review of our patients' records. Eighty-one patients with ITP were collected. The first relapse was termed as the hospitalization day before second-line therapy, and the second relapse was termed as the hospitalization day before alternate second-line therapy. We provided the following data of ITP patients at diagnosis, before and after first relapses, and before and after second relapses: presenting symptoms, platelet count, MPV, PDW, and PCT values. We obtained significant statistical differences between MPV values after initial treatment and before second-line therapy [first relapse] [p=0.005], between MPV values after splenectomy and before immunosuppressive or immune modulator therapy [second relapse] [p=0.028], and also, between PCT values after splenectomy and before second relapse [p=0.043]. Mean platelet volume is gradually increasing before first and second relapses, and again normal values are being obtained after appropriate therapies. We conclude that MPV is a useful parameter as a predictor of relapses

Clinical Implications of Elevated Antiphospholipid Antibodies in Adult Patients with Primary Immune Thrombocytopenia

BACKGROUND/AIMS: Antiphospholipid antibodies (aPL) have been detected in various proportions of patients with primary immune thrombocytopenia (ITP), but the clinical significance of this is debatable. The present study aimed to determine the frequency and clinical implications of elevated aPL in adult patients with ITP. METHODS: We prospectively studied newly diagnosed adult patients with ITP who were enrolled between January 2003 and December 2008 at Chungnam National University Hospital. They were evaluated for the presence of lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) at diagnosis and were followed for the development of thrombosis. RESULTS: Seventy consecutive patients with ITP (median age, 48 years; range, 18 to 79) were enrolled. Twenty patients (28.5%) were positive for aPL at the time of diagnosis: aCL alone in 15 (75%), aCL and LA in two (10%), and LA alone in three (15%). Patients who had platelet counts < 50,000/microL were administered oral prednisolone with or without intravenous immune globulin. No difference was found between the aPL-positive and -negative groups regarding gender, initial platelet count, and response to the therapy. After a median follow-up of 20 months (range, 2 to 68), two of 20 patients who were aPL-positive (10%) developed thrombosis, whereas no thrombotic event was found among those who were aPL-negative. CONCLUSIONS: Our data suggest that aPL levels should be determined at the initial presentation of ITP and that patients found to be aPL-positive should receive closer follow-up for thrombotic events.

Intravenous immune globulin versus intravenous anti-D immune globulin for the treatment of acute immune thrombocytopenic purpura.

OBJECTIVE: The purpose of this study was to compare the efficacy and side effects of intravenous immunoglobulin (IVIG) with intravenous anti-D immunoglobulin for treatment of newly diagnosed acute childhood Idiopathic thrombocytopenic purpura (ITP). METHODS: Children (6 months to 14 years) with newly diagnosed acute ITP and platelet count below 20,000/ microL were randomized to receive single dose intravenous 75 microg/kg anti-D or 1g/kg IVIG for two consecutive days (total dose 2 g/kg). Response rate defined as a platelet count over 20,000 / microL 72 hours after initial treatment. RESULTS: Eighty one patients (52 male and 29 female) with mean age of 5 years and 3 months randomly divided in anti-D group (n=42) and IVIG group (n=39). Mean baseline (pretreatment) platelet counts were 15406 / microL and 15230/ microL in anti-D and IVIG group, respectively. The response rate in IVIG group (98%) was more significant than anti-D group (76%); (P = 0.017). After 7 days the platelet counts of all patients in IVIG group were more than 20,000/ microL while in anti-D group 12% had platelet counts below 20,000/ microL. CONCLUSION: In acute childhood ITP, initial treatment with IVIG (2g/Kg in divided dose) increased platelet count more rapidly and more significant than intravenous anti-D (single dose of 75 microg/kg) within the first 72 hours.

Attenuated form of Evans syndrome among pediatric ITP patients.

Long term follow up of adult patients with immune thrombocytopenic purpura (ITP) have shown evolvement of secondary autoimmune diseases such as SLE, Evans syndrome, autoimmune neutropenia, Graves disease etc. We studied 30 cases of pediatric ITP patients for evidence of hemolysis to assess the possibility of Evans like syndrome. Measurement of free serum haptoglobin, a sensitive indicator of red cell destruction was used after careful exclusion of micro angiopathic hemolysis, SLE or overt Evans Syndrome. Results showed abnormally low level of free serum haptoglobin in 11 of the 30 (36.7%) patients compared to that in 20 age matched controls (P < 0.001) as an evidence of hemolysis. Our data in pediatric patients is similar to that reported in adult ITP cases and support the observation of Evans made 50 years ago that there is a spectrum like relationship between primary thrombocytopenia and hemolytic anemia. Thus the concept of attenuated form of Evans syndrome could be considered, in group of patients with ITP in pediatric age group.

Características clínicas del púrpura trombocitopénico inmune: revisión de 52 casos / Clinical characteristics of inmune thrombocytopenic purpura

Introducción: El Púrpura Trombocitopénico Inmune (PTI) suele ser autolimitado pero 10-20 por ciento persisten a los 6 meses, es decir de evolución crónica. Su tratamiento es controvertido y existen pocos datos nacionales. Objetivo: Conocer algunas características clínicas y de laboratorio del PTI, su relación con evolución crónica y el manejo actual. Método: Estudio retrospectivo de los 52 pacientes con PTI evaluados en Hospital Luis Calvo Mackenna, entre marzo 1998 y febrero 2003. Se consignó: sexo, edad, manifestaciones clínicas, conducta terapéutica y recuento plaquetario (RP) al diagnóstico, 15-60 días y 6 meses. Se aplicaron Test de Fisher y Odd Ratio. Resultados: Mediana de edad: 4,4 años (0,7 a 16,1), el RP fue < - 20 000 x mm3 en 37/52. No hubo hemorragia del sistema nervioso central. Se manejó con observación clínica 34/52, corticoides 17/52 e inmunoglobulinas endovenosas con corticoides 1/52. Completaron control (6 meses) 48/52 pacientes. Presentaron curso crónico 11/48, asociado a RP 15 días < - 20 000 x mm3 (p = 0,01) OR = 9 (IC95 por ciento: 1,26-80,16) y RP a los 60 días < - 50 000 x mm3 (p = 0,0000003) OR= 124 (IC95 por ciento: 7,77 - 4951,52). Conclusiones: La mayoría de los pacientes requirieron sólo observación clínica. Presentaron evolución crónica 23 por ciento siendo factores de riesgo RP a los 15 días £ 20 000 x mm3 y RP a los 60 días £ 50 000 x mm3.

Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario / Successful treatment of idiopathic acquired refractory thrombotic thrombocytopenic purpura with an association of rituximab - vindesine. Report of one case

We report a 23 years old female who presented a second episode of thrombotic thrombocytopenic purpura (TTP). She was treated with fresh frozen plasma infusions and 14 plasma exchange (PE) sessions without response. Therefore a second-line therapy was started, associating a weekly cycle administration of vindesine (Vds) 2 mg/m2 and rituximab (R) 375 mg/m2. Five cycles of this association plus one cycle of R exclusively, were administered. After the third course, biological signs of improvement were observed and complete normalization of blood cell counts and other specific parameters was seen after 8 weeks. From the beginning of her second relapse we detected a severe deficit (<5%) in von Willebrand-cleaving factor (ADAMTS13) associated to the presence of ADAMTS13 inhibitors. The combined treatment induced an improvement in ADAMTS13 values without detectable inhibitors. After 21 months of follow-up the patient was well, without signs of relapse but ADAMTS13 values were still under normal, which may be an unfavorable prognostic factor. PE is the treatment of choice for acquired idiopathic TTP, but for refractory cases or TTP cases with severe ADAMTS13 values/high inhibitor titers, PE associated to an immunosuppressive treatment should be considered.

Clinical Aspects of Pregnancy and Delivery in Patients with Chronic Idiopathic Thrombocytopenic Purpura (ITP)

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a condition that often develops in young women and, consequently, physicians should frequently manage and monitor pregnant patients with this disorder. METHODS: We reviewed the charts of 30 women with chronic ITP delivered in 31 pregnancies from January 1995 to December 2003. RESULTS: Fifteen patients were diagnosed with ITP before pregnancy and sixteen patients were diagnosed during pregnancy. The mean platelet counts before pregnancy, during pregnancy, and at delivery were 70, 040/mm3, 83, 960/mm3, and 62, 680/mm3, respectively. The symptoms of hemostatic impairment were not noted in most of the pregnancies (77%, 24/31). During pregnancy and at delivery, most of the women (61%, 19/31) received various kinds of treatment to raise platelet counts. At delivery, the most commonly used therapy was platelet transfusion (48.4%, 15/31). Seven pregnancies (22.6%) were treated with corticosteroids during pregnancy and at delivery. Five pregnancies (16.1%) were treated with IV IgG during pregnancy and at delivery. Fifteen deliveries (51.7%) were performed by cesarean section and fourteen (48.3%) with vaginal delivery. Bleeding was uncommon at delivery. There were no cases of infants with any clinical signs of hemorrhage. CONCLUSION: Our current results suggest that ITP in pregnancy can proceed safely with low hemorrhagic risk in both infants and mothers, and that mothers with ITP can deliver healthy infants without serious hemorrhagic complications.

Lupus anticoagulant in immune thrombocytopenic purpura.

OBJECTIVE: Antibodies against phospholipid antigens (APA) have been demonstrated in adult idiopathic(immune) thrombocytopenic purpura (ITP), but their clinical and pathogenetic significance has remained elusive. Also there are no such studies available in pediatric ITP cases. In this study, the prevalence and clinical significance of APAs were investigated in pediatric patients with ITP. METHODS: Forty newly diagnosed ITP patients (age 2-13 years) were prospectively studied. They were evaluated for the presence of lupus anticoagulant (LA). RESULTS: Eleven patients (27.5%) were LA positive at the time of diagnosis. No statistically significant differences were found between the LA-positive and LA-negative groups regarding gender, initial platelet counts, or response to methyl prednisolone therapy. After 6 months of follow up, 5 of the 11 LA-positive cases were still positive for LA. The frequency of LA positivity found in this pediatric age group was similar to that reported in adult patients. CONCLUSION: In view of the fact that in adult patients with ITP, the persistent presence of APAs is an important risk factor for the development of antiphospholipid syndrome, the same may also hold true for pediatric ITP patients, and thus 'demands long term follow up for these patients.

Mecanismos celulares y bioquímicos involucrados en la fisiopatogenia de la púrpura trombocitopénica autoinmune / Cellular and biochemical mechanisms involved in physiopathogenesis of autoimmune thrombocytopenic purpura

Autoimmune thrombocytopenic purpura (ATP) is a bleeding disorder caused by excessive destruction of antibody-coated platelets. It is known that platelet destruction takes place in macrophages of reticulo-endothelial system, but immunological mechanisms involved in such destruction are unknown. The objective of this article is to review the literature concerning pathogenesis of ATP: to have controlled experimental conditions some animal laboratory models have been used. The (NZW X BXSB) F1 mice have been studied as autoimmune disease model and Harrington mouse as an immune purpura model. Studies in humans suggest that there are some differences in pathogenesis of acute or chronic ATP, particularly in reactive T cells. For example, in chronic form there are high levels of The (CD4+) activity concomitant with low levels of T suppressor (CD8+) activity, while in acute form there is no dominance of any particular T cell activity or CD4+ is even decreased. Mitogen lymphocyte proliferation is increased in chronic ATP but decreased in acute form.

Treatment of childhood acute immune thrombocytopenic purpura with high-dose methylprednisolone, intravenous immunoglobulin, or the combination of both

OBJECTIVE: To compare the effectiveness of intravenous immunoglobulin (IVIG) alone, high dose methylprednisolone (HDMP) alone and the combination of IVIG and HDMP in the treatment of childhood immune thrombocytopenic purpura (ITP). BACKGROUND: Acute ITP in children is a self-limited disease with a benign course and low mortality rate. Patients with platelet count less than 20,000 x 10(9)/L are at increased risk of bleeding complications, making them candidates for treatment. METHOD: A 4 year retrospective study of 148 patients hospitalized with acute ITP was conducted to compare the effectiveness of HDMP vs IVIG vs the combination of IVIG/HDMP. Statistical methods used were descriptive statistics and variance analysis utilizing F distribution. RESULTS: The IVIG and the HDMP combination demonstrated to be superior to HDMP alone in raising the platelet count within the first 24 hours. The HDMP and IVIG combination was statistically a superior modality of treatment for patients with platelet count greater than 10,000 x 10(9)/L than was IVIG or HDMP alone. Intravenous immunoglobulin had the least effectiveness in patients with platelet count less then 10,000 x 10(9)/L within the first 24 hours. CONCLUSIONS: IVIG followed by the combination of HDMP and IVIG is the most effective therapeutic modality in rapidly increasing the platelet count to safe levels in children with acute ITP when compared to HDMP alone within the first 24 hours. For borderline low platelet count (> 10,000 x 10(9)/L) HDMP and IVIG was superior to IVIG alone.

Childhood thrombocytopenic purpura--review and a case report.

Childhood idiopathic thrombocytopenic purpura is a rare condition seen in children. A case of a 10 year old female child is reported. Bleeding from the gingiva and petechiae on the tongue were observed. The blood picture was normocytic normochromic with neutrophilia and thrombocytopenia. Bone marrow study was suggestive of marrow changes in idiopathic thrombocytopenic purpura. Dental extractions were postponed. Recognition of the disease by the dental profession is stressed.

In-vitro inhibition of antiplatelet autoantibodies by intravenous immunoglobulins and Rh immunoglobulins.

Autoimmune thrombocytopenia (AITP) is caused by autoantibodies to platelet glycoprotein antigens. Intravenous immunoglobulin (i.v.IgG) and Rh immunoglobulin infusions have found great significance in the treatment of AITP patients not responding to corticosteroids and other modes of therapy. In our study, it was observed that immunoglobulins (i.v.IgG & Rh), and their Fab fragments inhibited the binding of antiplatelet autoantibodies to normal platelets, from 15.8 to 90.7% and 25.6 to 90.08% respectively; whereas, their Fc portion did not show any inhibition. The presence of specific anti-idiotypic antibodies to antiplatelet autoantibodies was established by using monoclonal antibodies to Glycoprotein IIb/IIa and Glycoprotein Ib/IX, as the specific idiotype source. The i.v.IgG and Rh immunoglobulin products reacted with the monoclonal antibodies, only through their Fab and not through the Fc portions, thereby confirming its specific anti-idiotype activity.

Púrpura trombocitopênica aloimune neonatal: caracterizaçäo de aloanticorpo plaquetário anti-HPA-1A (Anti-PIA1) por radioimunoprecipitaçäo indireta / Alloimmune neonatal thrombocytopenic purpura: characterization of platelet alloantibody anti-HPA-1A (Anti-PIA1) by indirect radioimmunoprecipitation

A púrpura trombocitopênica alo-imune neonatal (PTAN) é uma doença grave na qual a hemorragia cerebral pode ser fatal ou levar a seqüelas cerebrais permanentes. Similarmente, à doença hemolítica do recém-nascido (RN), a PTAN ocorre devido a aloimunizaçäo materna por um alo-antigeno incompatível presente nas plaquetas fetais. A apresentaçäo clínica é de púrpura generalizada acompanhada de hemorragia gastrointestinal, urinária, e/ou intracranial. O alo-antigeno HPA-1 (PL(A)) é responsável por cerca de 70-80 por cento dos casos de PTAN. Nesse estudo, os autores descrevem o caso de uma gestante que deu a luz a um RN com plaquetopenia acentuada devido a trombocitopenia aloimune. A contagem plaquetária da mae e do RN era 173 x 10(9)/L e 15 x 10(9)/L, respectivamente. O estudo sorológico realizado com a técnica de radioimunoprecipitaçäo indireta demonstrou forte reatividade do soro materno com o complexo glicoprotético GPIIb/IIIa em plaquetas PI(A1)-positivas de doador normal. Assim, o soro materno continha forte atividade anti-HPA-1a (anti-PI(A1)). O RN foi tratado com corticosteróides e gamaglobulina intravenosa. Após o tratamento, o RN teve alta hospitalar com contagem plaquetária elevada para 70 x 10(9)/L.