BACKGROUND: Idiopathic thromboycytopenic purpura(ITP) is one of the most common acquired bleeding disorders in childhood. The entity of chronic ITP is defined as the persistence of thrombocytopenia for more than 6 months from initial presentation. There are the controversies regarding the diagnosis and management of this disease. The aim of this study is to compare the responses to managements and clinical symptoms of children with chronic ITP. METHODS: We retrospectively analyzed patients with chronic ITP diagnosed and managed between Jan., 1992 and June, 1997. Data from 33 cases were collected. The length of follow-up ranged from 6 months to 5 years. Statistical analysis was done using Jandel Sigmastat software(version 2.0). RESULTS: 1) Mean age is 5.4+/-4.3 years. The ratio of female to male is 0.65. 2) Most common clinical symptoms at presentation were petechiae(81.8%) and mucosal bleeding(42.4%). The patients who presented only petechiae and purpurae were 18 cases(54.5%), both petechiae and mucosal bleeding were 9 cases(27.3%). 3) The platelet count was 43,015+/-39,912/mm3. Bone marrow examinations showed normal or increased megakaryocyte, no abnormal cells and normal cellularity. 4) The prolonged complete response lasting more than 6 months without maintenance therapy was attained in 15.2% of the patients. A continuous complete response was observed in 6.1% to prednisone and IV-gamma-globulin. A prolonged complete response to dexamethasone was in 23.8% and continuous complete response was in 14.3%. The splenectomy was underwent in 3 patients. All of them had a prolonged complete response. One of six patients who were treated with combination chemotherapy had a prolonged complete response. CONCLUSION: This analysis of chronic ITP patients suggests that splenectomy remains the most effective treatment and the next one is high dose dexamethasone therapy. But the number of patients was not enough, more attention should be given to the management of chronic idiopathic thrombocytopenic pupura(ITP).
Bone Marrow Examination ; Child ; Dexamethasone ; Diagnosis ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Hemorrhage ; Humans ; Male ; Megakaryocytes ; Platelet Count ; Prednisone ; Purpura ; Purpura, Thrombocytopenic, Idiopathic* ; Retrospective Studies ; Splenectomy ; Thrombocytopenia

Peliosis hepatis is a rare condition that can cause hepatic hemorrhage, rupture, and ultimately liver failure. Several authors have reported that peliosis hepatis develops in association with chronic wasting disease or prolonged use of anabolic steroids or oral contraceptives. In this report we describe a case in which discontinuation of steroid therapy improved the condition of a patient with peliosis hepatis. Our patient was a 64-year-old woman with a history of long-term steroid treatment for idiopathic thrombocytopenic purpura . Her symptoms included abdominal pain and weight loss; the only finding of a physical examination was hepatomegaly. We performed computed tomography (CT) and magnetic resonance imaging (MRI) of the liver and a liver biopsy. Based on these findings plus clinical observations, she was diagnosed with peliosis hepatis and her steroid treatment was terminated. The patient recovered completely 3 months after steroid discontinuation, and remained stable over the following 6 months.
Adrenal Cortex Hormones/therapeutic use ; Female ; Hepatomegaly/complications/*diagnosis/pathology ; Humans ; Liver/pathology ; Magnetic Resonance Imaging ; Middle Aged ; Peliosis Hepatis/complications/*diagnosis/pathology ; Purpura, Thrombocytopenic, Idiopathic/complications/*diagnosis/drug therapy ; Tomography, X-Ray Computed ; Treatment Outcome

Infection of the central nervous system with Nocardia sp. usually manifests as supratentorial abscesses. Supratentorial and cerebellar abscesses from infection with Nocardia sp. following immunosuppression with long-term corticosteroids for idiopathic thrombocytopenia (ITP) have not been reported. An 83 years-old, human immunodeficiency virus (HIV)-negative, polymorbid male with ITP for which he required corticosteroids since age 53 years developed tiredness, dyspnoea, hemoptysis, abdominal pain, and progressive gait disturbance. Imaging studies of the lung revealed an enhancing tumour in the right upper lobe with central and peripheral necrosis, multiple irregularly contoured hyperdensities over both lungs, and right-sided pleural effusions. Sputum culture grew Nocardia sp. Neurological diagnostic work-up revealed dysarthria, dysphagia, ptosis, hypoacusis, tremor, dysdiadochokinesia, proximal weakness of the lower limbs, diffuse wasting, and stocking-type sensory disturbances. The neurological deficits were attributed to an abscess in the upper cerebellar vermis, myopathy from corticosteroids, and polyneuropathy. Meropenem for 37 days and trimethoprime-sulfamethoxazole for 3 months resulted in a reduction of the pulmonary, but not the cerebral lesions. Therefore, sultamicillin was begun, but without success. Long-term therapy with corticosteroids for ITP may induce not only steroid myopathy but also immune-incompetence with the development of pulmonary and cerebral nocardiosis. Cerebral nocardiosis may not sufficiently respond to long-term antibiotic therapy why switching to alternative antibiotics or surgery may be necessary.
Adrenal Cortex Hormones/*adverse effects/*therapeutic use ; Aged, 80 and over ; Cerebellar Diseases/*chemically induced/*diagnosis/pathology ; Humans ; Immunosuppression ; Male ; Muscular Diseases/*chemically induced/pathology ; Nocardia Infections/*diagnosis ; Purpura, Thrombocytopenic, Idiopathic/*drug therapy

PURPOSE: This study examined the outcomes of children with chronic immune thrombocytopenia (ITP). MATERIALS AND METHODS: We retrospectively analyzed the medical records of all patients diagnosed with ITP from January 1992 to December 2011 at our institution. RESULTS: A total of 128 patients (64%) satisfied the criteria for newly diagnosed ITP, 31 (15%) for persistent ITP, and 41 (21%) for chronic ITP. The median age at diagnosis was 4.5 years (range, 1 month to 18 years). The median platelet count at diagnosis was 32x109/L. A comparison of the initial treatment data from 2001 to 2011 with those from 1992 to 2000 showed that the number of bone marrow examinations decreased, whereas observation increased. Chronic ITP presented at an older age than newly diagnosed and persistent ITP (6.6 years vs. 3.8 years vs. 4.1 years, respectively); however, the difference did not reach statistical significance (p=0.17). The probability of complete remission of chronic ITP was 50% and 76% at 2 and 5 years after diagnosis, respectively. Patients aged <1 year at diagnosis had a significantly better prognosis than did older patients (hazard ratio, 3.86; p=0.02). CONCLUSION: Children with chronic ITP showed a high remission rate after long-term follow-up. This study suggests that invasive treatments such as splenectomy in children with chronic ITP can be delayed for 4 to 5 years if thrombocytopenia and therapeutic medication do not affect the quality of life.
Adolescent ; Child ; Child, Preschool ; Chronic Disease ; Female ; Follow-Up Studies ; Humans ; Male ; Platelet Count ; Prognosis ; Purpura, Thrombocytopenic, Idiopathic/*diagnosis/drug therapy ; *Quality of Life ; Remission Induction ; Retrospective Studies ; Treatment Outcome

BACKGROUND: Reticulated platelets (RP) are young platelets with a high mRNA that are newly produced from the bone marrow. Thiazole orange (TO) has been a RNA staining fluorescent dye for reticulocyte, and used for RP recently. The increased percentage of RP (RP%) reflects immaturity and hematopoietic activity of platelets, therefore it may be useful for the diagnosis of idiopathic thrombocytopenic purpura (ITP). METHODS: To assess the usefulness of RP in diagnosing ITP, we compared with RP%s of 50 ITP patients, 35 thrombocytopenic patients due to impaired production and 87 heathly normal controls. Platelets were stained with TO dye, followed by flowcytometric analysis. Platelet associated IgG (PAIgG) was also measured with the same samples. The standard gate was used as a reference with the unstained sample from a normal subject and the RP% was expressed as the percentage of TO positive cells of platelets. RESULTS: The RP% of patients with ITP was significantly higher than those of thrombocytopenia due to impaired platelet production and healthy controls (24.4+/-14.3% vs 8.6+/-5.2% and 8.0+/-5.1%, respectively). There was negative correlation between the platelet count and RP%, and positive correlation between MPV and RP%. In diagnosing for ITP, the sensistivity and specificity of RP% were 81% and 92%, respectively, and more valuable than those of PAIgG test. Using RP% and PAIgG at the same time, the diagnostic efficiency for ITP was not improved. The RP% of an ITP patient was changed to correspond with the disease progression, and that of an AML patient following chemotherapy was increased to precede the rising of the platelet count. CONCLUSION: It suggest that the measurement of reticulated platelets is a very useful test for diagnosis of ITP, furthermore it can be used to estimate the thrombopoietic activity before bone marrow examination.
Blood Platelets ; Bone Marrow ; Bone Marrow Examination ; Citrus sinensis* ; Diagnosis ; Disease Progression ; Drug Therapy ; Flow Cytometry ; Humans ; Immunoglobulin G ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Reticulocytes ; RNA ; RNA, Messenger ; Sensitivity and Specificity ; Thrombocytopenia