Recurrent Idiopathic Thrombocytopenic Purpura Research Articles

Laparoscopic removal of an accessory spleen for recurrent idiopathic thrombocytopenic purpura 5 years after laparoscopic splenectomy using perioperative eltrombopag conditioning

Treatment of idiopathic thrombocytopenic purpura (ITP) refractory to medical management consists of splenectomy with a high success rate. Recurrent ITP may be due to re-growth of a missed accessory spleen. The clinical course of a 59-year-old female who had undergone laparoscopic splenectomy for ITP refractory to medical management 5 years ago developed a sudden drop in her platelet count to as low as 1 K/ml and she developed purpura. On liver/spleen scan and CT scan a 5 × 5 cm mass containing splenic tissue was found close to the tail of the pancreas. Eltrombopag (Promacta®) was used to stabilize her platelet count. During laparoscopy dense adhesion of the omentum to the abdominal wall and stomach from her previous splenectomy had to be divided. The colon was identified, the lesser sack opened through the gastrocolic ligament and the splenic flexure was taken down. The stomach was lifted anteriorly and medially to expose the tail of the pancreas and superior and dorsal to the pancreas at the previous splenic hilum the mass was identified and carefully dissected off the pancreas, kidney and adrenal gland. An endo GI with a white load was used to divide the vascular pedicle, which originated from a side branch of the splenic artery and splenic vein at the tail of the pancreas and secured with an endoloop. The accessory spleen was placed into an endobag, cut in small pieces and removed through the 10–12 mm port. The patient recovered well from the procedure without any complications and her platelet count normalized within a few weeks. Re-growth of splenic tissue causing recurrent ITP is a rare condition but should be considered. Laparoscopic removal of accessory spleens can be safely done. Newly developed agents are helpful in the management of ITP patients.

Presentation and Outcome of Idiopathic Thrombocytopenic Purpura in Children: A Single Institution Experience

Abstract 4683 PURPOSE:To review the presenting features, response to treatment and outcome in children diagnosed with idiopathic thrombocytopenic purpura (ITP) at the University of South Alabama, Children's and Women's Hospital and Specialty Clinic. METHODS:Using ICD code 287.3, data were collected from the specialty clinic's medical records and hospital database for children diagnosed with ITP between January 2005 and September 2010. Recurrent and chronic ITP were defined as thrombocytopenia recurring within or more than 6 months of diagnosis, respectively. Univariate and multivariate logistic regression analyses were performed to evaluate variables associated with chronic ITP. RESULTS:Eight four patients were identified (M,F 1:1) with an average age of 70 months at diagnosis. Mean platelet count at presentation was 14k. Oral or nasal mucosal bleeding occurred in 19(23%) patients but none experienced a serious hemorrhage. Thirty three (39%) patients had an associated illness prior to diagnosis of ITP. Treatment consisted of intravenous immunoglobulin (IVIG) in 38(45%), WinRho in 11(13%), IVIG or WinRho followed by the other in 20(24%), data not available 8(10%) and no therapy in 7 patients (8%). Average platelet count at discharge and within 2 weeks after IVIG and WinRho was 57k, 337k and 57k, 375 respectively. Forty three (51%) were acute, 17(20%) became recurrent, and 24(29%) became chronic ITP. Bone marrow examination was performed in 26(30%) patients upon subsequent relapse but the diagnosis remained unaltered in all cases. Rituximab therapy was provided to five and splenectomy was performed in 7 patients. Four patients failed both modalities, all of whom currently are IVIG dependant.Age <5year (OR 0.12, 95%CI 0.22, 0.67, p=0.01) was protective against development of chronic ITP while platelet count >20k at presentation (OR 6.50, 95%CI 1.35, 31.30, p=0.02) and race other than white (OR 36.63, 95%CI 4.61, 291.09, p=0.001) were found to be significantly associated with the development of chronic ITP. Gender, mean platelet volume, total white cell count, and absolute lymphocyte count had no significant association. CONCLUSION:Our study supports the published data that patients with an initial platelet count >20k, older age and non-white race have an increased risk of progression to chronic ITP. Other published variables had no significant association in our analyses. Response to IVIG and WinRho was no different in our patients while rituximab or splenectomy did not lead to a complete resolution in refractory cases. Since bone marrow examination did not alter the diagnosis in any patient, we suggest that routine performance of this procedure may be omitted when a diagnosis of ITP is consistent with clinical history, physical examination and laboratory data. Disclosures:No relevant conflicts of interest to declare.

Presence of Idiopathic Thrombocytopenic Purpura and autoimmune hemolytic anemia in the patients with common variable immunodeficiency.

Common Variable Immunodeficiency (CVID) is a heterogeneous group of disorders characterized by hypogammaglobulinemia and an increased susceptibility to recurrent infections as well as autoimmunity and malignancies. Idiopathic Thrombocytopenic Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA) are two autoimmune disorders which may be seen in association with CVID. Among 85 CVID patients, seven cases had ITP and/or AIHA (8%). Four of these patients had one or more episodes of ITP, one patient had AIHA, and two patients had both ITP and AIHA (Evans syndrome). Almost, all patients experienced chronic and recurrent infections mostly in respiratory and gastrointestinal systems during the course of the disease. Among the seven patients, five presented their underlying disease with recurrent respiratory and/or gastrointestinal tract infections, while in two remaining patients, CVID was presented with ITP. Three patients died until now; two because of hepatic failure and one due to pulmonary hemorrhage. As CVID is prone to autoimmune disorders, it should be considered as a differential diagnosis of adult-onset ITP and possibly in children. Chronic and recurrent ITP, especially in the presence of propensity to respiratory and gastrointestinal infections mandate the evaluation for an underlying immune dysregulation such as CVID.

Recurrent idiopathic thrombocytopenic purpura in childhood

Idiopathic thrombocytopenic purpura (ITP) is a common haematological disease during childhood, that usually has a benign course; however, literature on the recurrent form of the disease (rITP) is limited. rITP was characterized by intermittent episodes of thrombocytopenia (TP) followed by periods of recovery, unrelated to therapeutic intervention. We retrospectively reviewed features of patients with rITP, diagnosed and systematically followed up at our center, during the period 1975-2004. Forty-eight of 795 children with ITP (6.0 %) presented with rITP. The majority of patients (68.8%) had only one recurrence, whereas only one patient had four. A time interval between two episodes longer than 3 months (up to 96) was identified in 2/3 of episodes and <3 months in 1/3. The initial episode and the first recurrence mostly shared features of acute ITP; however, 22.9% of the episodes appeared with a chronic self-limited course. Bleeding manifestations were rare (18.6% of episodes) and mild, and they tended to occur in severely thrombocytopenic patients, mainly at the onset of the initial episode; intracranial hemorrhage (ICH) occurred in a toddler with short duration thrombocytopenia. Intravenous gamma globulin (IVIG) or corticosteroids were administered in 24.5% of episodes. None of the patients needed splenectomy. rITP is a rare, mild, self-limited type of ITP, although ICH may occur in a profoundly TP child. Recurrence may occur close or far apart to a previous isolated TP episode. The duration of episodes varies considerably from patient to patient and from episode to episode in the same patient. The pathogenesis of rITP still remains unclear.

RECURRENT IDIOPATHIC THROMBOCYTOPENIC PURPURA IN CHILDHOOD

INTRODUCTION: Idiopathic thrombocytopenic purpura (ITP) is usually a benign disease that remits within weeks to years. The literature on recurrent ITP (rITP) is limited. OBJECTIVE: The aim of this study was to retrospectively review patients with rITP who were followed up during the period of 1975–2004. METHODS: We reviewed the outcome of 790 children with rITP RESULTS: Among 790 children with ITP, 47 (5.2%) presented with &amp;gt;1 episode of thrombocytopenia. The median age of the children at onset of the disease was 55.9 ± 35.3 months and at final remission was 94.4 ± 58.9 months. The majority of patients (76.6%) had 1 recurrence, whereas the rest of them had &amp;gt;1 recurrence (up to 4); the total number of recurrences was 63. The interval between 2 episodes was &amp;lt;6 months in 25% of the episodes, 6 to 12 months in 29%, 12 to 24 months in 24%, 24 to 36 months in 8%, and &amp;gt;3 years in 14%. Almost half the patients demonstrated bleeding manifestations at diagnosis, whereas only a minority (5) showed bleeding symptoms during the first recurrence. Hemorrhages occurred at times of severe thrombocytopenia and were, in general, mild; however, 1 patient suffered intracranial hemorrhage. Of the episodes, 28.6% necessitated hospitalization and 17.5% required therapeutic intervention with corticosteroids or/and intravenous immunoglobulin. The long-term outcome of all patients was excellent, and none of them needed splenectomy. CONCLUSIONS: rITP occurs mostly in young children, has a good outcome after &amp;gt;1 to 5 episodes, and a median age that ranges from months to years. The course is more often benign; however, life-threatening hemorrhage may occur in a severely thrombocytopenic patient.

An 18-year review of open and laparoscopic splenectomy for idiopathic thrombocytopenic purpura

Background Laparoscopic splenectomy has become the preferred surgical procedure for the management of idiopathic thrombocytopenic purpura (ITP). However, there studies have directly compared the incidence of recurrent ITP secondary to missed accessory spleens in open versus laparoscopic splenectomy. Methods Open and laparoscopic splenectomies performed for ITP at 4 sites over 18 years were analyzed. The incidence of recurrent disease secondary to missed accessory spleens was compared between the open and laparoscopic splenectomy groups. Results A total of 105 splenectomies (54 open/51 laparoscopic) were performed. Accessory spleens were identified in 6 laparoscopic and 6 open cases ( P = .57). Recurrent disease occurred in 27.6% of open and 14.6% of laparoscopic cases ( P = .222). There were no cases of recurrent ITP secondary to a missed accessory spleen in either group. Conclusions The incidence of missed accessory spleens causing recurrent disease is similar when splenectomy is performed either open or laparoscopically.

Isolated type 5 antimitochondrial autoantibodies are associated with a history of thrombocytopenia and fetal loss

To report an unusual case of clinical antiphospholipid syndrome seen with recurrent fetal loss, chronic thrombocytopenia, and recurrent idiopathic thrombocytopenic purpura, in which the only laboratory marker was M5-type antimitochondrial autoantibodies (AMA). Case report. University general hospital, tertiary level of clinical care. Institutional practice. A 65-year-old woman with antiphospholipid syndrome associated with autoimmune polyglandular syndrome of IIIC type. Clinical history and biochemical and immunologic markers. The presence of M5-type AMA in clinical antiphospholipid syndrome isolated by indirect immunofluorescence. During the 10-year follow-up, immunologic studies detected a persistent positive M5-type AMA at high titer (1/640), antithyroid antibodies, anti-gastric parietal cells, and anti-intrinsic factor. Antinuclear, anti-DNA, and antiphospholipid (anticardiolipin, anti-beta(2)-glycoprotein I) autoantibody tests were all repeatedly negative. Results of coagulation studies and negative lupus anticoagulant were normal on several occasions. Our findings suggest the necessity of determining M5-type AMA in the study panel of antiphospholipid syndrome diagnosis, particularly in the absence of other typical autoantibodies.

High-dose dexamethasone as a first- and second-line treatment of idiopathic thrombocytopenic purpura in adults.

The current first-line choice of treatment of idiopathic thrombocytopenic purpura (ITP) in adults, prednisone, is effective but has many side effects. Furthermore, reduction of the dose leads to a relapse of ITP in a majority of cases. Courses of high-dose dexamethasone (HD) aim to avoid these problems. We treated 36 patients with newly diagnosed or recurrent ITP with an 8-day course of HD, with a peak dose of 40 mg/day. The courses were repeated up to a maximum of six courses, with a 28-day interval. Acute and chronic effects of HD on platelet counts were observed, as well as side effects. HD led to an acute response (rise of platelet count to a level above 50 x 10(9)/l) in 83%. When HD was given as a first-line treatment, 59% of patients were still in remission after 31 months. When HD was given as a second-line treatment, 50% of patients were in remission after 5 months, declining to 25% after 54 months. Side effects were frequent but rarely dangerous. In conclusion, acute effects of HD were excellent. Long-term effects of HD as a first-line therapy of ITP were good, but its long-term effects as a second-line therapy were much poorer.

High P-glycoprotein-mediated export observed in patients with a history of idiopathic thrombocytopenic purpura.

Studies have suggested that high P-glycoprotein expression in lymphocytes from patients with autoimmune disorders may affect disease outcome. Idiopathic thrombocytopenic purpura (ITP) and Evans' syndrome are widely thought to be autoimmune processes, however, the precise mechanisms remain unknown. Peripheral blood mononuclear cells from patients with refractory or recurrent ITP or Evans' syndrome were studied using the rhodamine 123 flow cytometric assay to investigate functional export levels. Lymphocytes from ITP and Evans' syndrome patients showed a significantly decreased ability to retain rhodamine, suggesting increased export protein function. Reverse transcription polymerase chain reaction distinguished P-glycoprotein as the likely export protein.

Laparoscopic accessory splenectomy for recurrent idiopathic thrombocytopenic purpura and hemolytic anemia.

Laparoscopic splenectomy is an effective treatment for idiopathic thrombocytopenic purpura (ITP) and hemolytic anemia that has a remission rate of 80-90%. In some patients in whom the disease persists or recurs, a diagnosis of accessory spleen is made. The long-term efficacy of laparoscopic accessory splenectomy is unknown. Patients who underwent laparoscopic accessory splenectomy were followed in the outpatient clinic. The perioperative course, blood counts, and need for medical therapy to maintain a normal count were recorded. Eight patients underwent laparoscopic accessory splenectomy. All procedures were completed laparoscopically, and all patients were discharged on the 1st postoperative day. Patients were available for a follow-up period of 15 months range, (3-27). None of the ITP patients achieved a complete remission. Two of them had a partial remission, and five ITP patients are now being treated with chronic corticosteroids to maintain a platelet count of >100,000/ml. Laparoscopic accessory splenectomy is associated with a low rate of morbidity and a short hospital stay. Despite its success in removing all residual splenic tissue, most patients will probably not enjoy a complete remission.

Studies on Clinical Characteristics of Recurrent Idiopathic Thrombocytopenic Purpura in Children

Studies on Clinical Characteristics of Recurrent Idiopathic Thrombocytopenic Purpura in Children

Intrahepatic splenic tissue in a patient with recurrent idiopathic thrombocytopenic purpura

Heterotopic splenic tissue (HST) can be classified into two entities: splenosis, resulting from autotransplantation of splenic tissue, and accessory spleens, arising embryologically. HST has been found in numerous locations within the abdominal cavity and beyond. It has particular clinical importance in patients with idiopathic thrombocytopenic purpura (ITP) as a possible cause for relapse after splenectomy.1

Childhood Idiopathic Thrombocytopenic Purpura: Experience at King Khalid University Hospital, Riyadh

Forty-nine children (30 boys and 19 girls) with idiopathic thrombocytopenic purpura (ITP) seen at King Khalid University Hospital (KKUH) Riyadh, during a 7-1/2 year period were retrospectively reviewed with regard to their clinical characteristics, management, and outcome. The age of onset of the disease ranged from 8 months to 11 years with a mean of 4.26 years. Twenty-seven cases presented as acute ITP while 22 cases presented as chronic ITP. The clinicopathological features and response to treatment of the cases were essentially similar to findings in other parts of the world. In a search for a predictor of chronic disease, the platelet counts at four weeks and three months after diagnosis were found to be a significant factor for prediction of chronicity of ITP. If the platelet counts are less than 100x10(9)/L during these periods, the risk of chronic ITP is increased and vice versa. There was, however, no correlation in our patients between duration of symptoms prior to presentation and outcome of the disease as has been reported by investigators in Europe and Australia.

Splenectomy for immune thrombocytopenic purpura.

Of 481 splenectomies performed at the University of Utah, Salt Lake City, 78 (16.2%) were for immune (idiopathic) thrombocytopenic purpura (ITP). The mean platelet count prior to therapy was 13,800/cu mm (range, 500 to 80,000/cu mm). All but two patients were initially treated with corticosteroids, and 58.2% responded with an increase in platelets (mean, 77,900/cu mm). The indications for splenectomy included (1) failure to respond to steroids (33.8%); (2) inability to taper steroids (52.1%); (3) recurrent ITP (5.6%); and (4) miscellaneous (8.5%). There was one death following splenectomy, and the postoperative morbidity was 14.1%. Complete remission occurred in 77.3%, and 84.8% were judged to have benefited from the procedure. The remission rate following splenectomy in patients who responded to preoperative steroids was 93.2%, whereas improvement fell to 68.3% in patients who failed to respond to steroids. This study confirms the benefit of splenectomy for ITP and demonstrates a predictive correlation with response to preoperative preparation with corticosteroids in these patients.

Immunoglobulins in idiopathic thrombocytopenic purpura in childhood.

Levels of immunoglobulins G, A and M were determined in 55 children with idiopathic thrombocytopenic purpura (ITP) (32 acute, 18 chronic and 5 recurrent). Mean IgG levels tended to be low in acute ITP and returned to normal with recovery. In contrast, chronic and recurrent ITP had persistently low IgG and IgA levels. C3 complement levels in 10 cases of acute ITP were normal. Seven patients with chronic ITP had their spleens removed, and IgG and IgA levels tended to rise, while IgM levels decreased in 4 patients after the operation. Low IgA levels observed at the onset of purpura in a child might favor the diagnosis of chronic or recurrent ITP.