scholarly journals Primary Immune Thrombocytopenia and Essential Thrombocythemia: So Different and yet Somehow Similar—Cases Series and a Review of the Literature

2021 ◽  
Vol 22 (20) ◽  
pp. 10918
Author(s):  
Marta Sobas ◽  
Maria Podolak-Dawidziak ◽  
Krzysztof Lewandowski ◽  
Michał Bator ◽  
Tomasz Wróbel
Keyword(s):  
Essential Thrombocythemia ◽  
Immune Thrombocytopenia ◽  
Treatment Modalities ◽  
Review Of The Literature ◽  
Thrombocytopenic Purpura ◽  
Cytokine Modulation ◽  
Primary Immune Thrombocytopenia ◽  
New Treatment ◽  
Sequential Appearance

This article collects several published cases in which immune thrombocytopenic purpura (ITP) is followed by essential thrombocythemia (ET) and vice versa. This surprising clinical condition is possible, but very rare and difficult to diagnose and manage. We have made an attempt to analyse the possible causes of the sequential appearance of ITP and ET taking into consideration the following: alteration of the thrombopoietin (TPO) receptor, the role of autoimmunity and inflammation, and cytokine modulation. A better understanding of these interactions may provide opportunities to determine predisposing factors and aid in finding new treatment modalities both for ITP and ET patients.

scholarly journals Primary Immune Thrombocytopenia: A Translational Research Model for Autoimmune Diseases

2019 ◽  
Vol 8 (11) ◽  
pp. 1971
Author(s):  
Emmanuel Andrès
Keyword(s):  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Translational Research ◽  
Idiopathic Thrombocytopenic Purpura ◽  
Immune Thrombocytopenia ◽  
Research Model ◽  
Thrombocytopenic Purpura ◽  
Primary Immune Thrombocytopenia

Primary immune thrombocytopenia (ITP), formally known as idiopathic thrombocytopenic purpura, is a multifactorial autoimmune disease that is both idiopathic (cause unknown) and rare [...]

The Role of Target Therapy in the Treatment of Gastrointestinal Noncolorectal Cancers: Clinical Impact and Cost Consideration

2018 ◽  
Vol 18 (5) ◽  
pp. 430-441 ◽  
Author(s):  
Massimiliano Berretta ◽  
Carmela Romano ◽  
Raffaele Di Francia ◽  
Chiara De Diviitis ◽  
Vincenzo Canzonieri ◽  
...  
Keyword(s):  
Cancer Treatment ◽  
Multidisciplinary Approach ◽  
Kinase Inhibitors ◽  
Target Therapy ◽  
New Drugs ◽  
Treatment Modalities ◽  
New Treatment ◽  
Remarkable Progress ◽  
Egfr Antibody

Gastrointestinal (GI) tumors are among the leading cause of death in cancer patients worldwide. Particularly, gastric cancer (GC) is the third cause of cancer deaths, whereas esophageal neoplasm is the eighth leading most common cancer worldwide and its incidence, especially adenocarcinoma type, is continuously increasing. Also, Hepatocellular carcinoma, Cholangiocarcinoma and pancreatic cancer represent a very interesting model to multidisciplinary approach and recently new drugs are used in their treatment. Currently, new clinical trials are designed including classic chemotherapy in association with either small molecule inhibitors (i.e. Tyrosine Kinase inhibitors) and/or monoclonal antibody (i.e. anti-EGFR antibody). Moreover, a comprehensive list of new molecules for target therapy is included in this issue. The development of new treatment modalities (multidisciplinary approach) and targeted therapy approaches have contributed to improving the outcome in these cancer diseases. During the past few years, remarkable progress in molecular biology of malignancy, the discovery of specific targets, and the resulting development of systemic drugs that block critical kinases and several molecular pathways have all contributed to progress in cancer treatment, also in GI non-colorectal cancer treatment.

Incidence of Primary Immune Thrombocytopenia (ITP) in Adults in One Region of Russia

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4941-4941
Author(s):  
Anait L. Melikyan ◽  
Elena I Pustovaya ◽  
Elena M. Volodicheva ◽  
Tamara I Kolosheinova ◽  
Marina V Kalinina ◽  
...  
Keyword(s):  
Incidence Rate ◽  
Immune Thrombocytopenic Purpura ◽  
Immune Thrombocytopenia ◽  
Response To Therapy ◽  
Research Database ◽  
Newly Diagnosed ◽  
Thrombocytopenic Purpura ◽  
Target Region ◽  
Primary Immune Thrombocytopenia ◽  
Epidemiological Characteristics

Abstract Introduction. Primary immune thrombocytopenia is a rare disease1. The incidence of ITP is not well estimated in Russia and worldwide. In adults it varies from 1,6 to 3,9/100 000 person-years2-3. The gender and age-associated results are discussed and differ in several investigations4-6. Study objectives: evaluation of the incidence of primary immune thrombocytopenia in adults in one region of Russia Patients and methods. The data source is the Registry of the patients with primary ITP in Russia. 272 adult patients: 77 males (28%) and 195 females (72%), age from 16 to 89 years (median 44 years) with ITP (ICD-10 code D69.3), newly diagnosed cases during the period from 12 Jan 2014 to 24 May 2016. Results. 221 (81%) cases were newly diagnosed in 12 regions of Russia in which registration was performed most actively - more than 5 cases for the duration of the study. But only one region was selected for the first evaluation of epidemiological characteristics because of the number of reasons. There is one hematological central clinic in this region in which diagnosis of ITP can be verified and patients with ITP are treated and monitored most properly. The early started and fully performed registration process can be regarded as covered most part of region population in this target region. 86 cases (27 male, 59 female) were registered in the target region. The gender-age distribution was following: male: age <41 = 10 (37%), age <41-60 = 7 (26%), age >60 = 10 (37%); female: age <29 = 10 (49%), age <41-60 = 15 (25%), age >60 = 15 (25%). The estimated incidence rate in the target region is shown in table 1. The estimated incidence rates in gender-age strata in the target region are demonstrated in table 2. Conclusion. Overall ITP incidence in one region of Russia is 3.20/100 000 person-years. It is compatible to the incidence in other European countries. Our data demonstrate the rise of incidence rate in males with age and its decrease with age in female population. Literature. 1) Rodeghiero F., Stasi R., Gernsheimer T., Michel M., Provan D., Arnold D.M., et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from international working group. Blood. 2009; 113(11): 2386--93. doi: 10.1182/blood-2008-07-162503. 2) Terrell DR, Beebe LA, Vesely SK, Neas BR, Segal JB, George JN. The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports. Am J Hematol. 2010; 85(3): 174-180. 3) Moulis G, Palmaro A, Montastruc J-L, Godeau B, Lapeyre-Mestre M, Sailler L. Epidemiology of incident immune thrombocytopenia: a natiowide population-based study in France. Blood. 2014; 124(22): 3308-3315. 4) Segal JB, Powe NR. Prevalence of immune thrombocytopenia: analyses of administrative data. J Thromb Haemost 2006; 4: 2377-83 5) Schoonen WM, Kucera G, Coelson J, et al. Epidemiology of immune thrombocytopenic purpura in the General Practise Research Database. Br J Haematol 2009; 145(2): 235-244. 6) Lisukov I.A., Maschan A.A., Shamardina A.V., Chagorova T.V., Davydkin I.L., Sycheva T.M., et al. Immune thrombocytopenia: clinical manifestations and response to therapy. Intermediate analysis of data of the Russian register of patients with primary immune thrombocytopenia and review of literature. Oncogematologiya. 2013; 2: 61--9]. Disclosures No relevant conflicts of interest to declare.

scholarly journals Maternal and fetal outcomes of primary immune thrombocytopenia during pregnancy: A retrospective study

2017 ◽  
Vol 11 (1) ◽  
pp. 12-16 ◽  
Author(s):  
KS Gilmore ◽  
C McLintock
Keyword(s):  
Platelet Count ◽  
Immune Thrombocytopenia ◽  
Post Partum ◽  
Bleeding Complications ◽  
Platelet Response ◽  
Thrombocytopenic Purpura ◽  
Primary Immune Thrombocytopenia ◽  
Secondary Outcomes ◽  
Post Partum Haemorrhage ◽  
Auckland Hospital

Objective We reviewed outcomes of 52 pregnancies in 45 women with immune thrombocytopenic purpura who delivered at Auckland Hospital with an antenatal platelet count of <100 × 109/L. Outcome measures Primary outcomes were maternal platelet count at delivery and treatment response. Secondary outcomes included post-partum haemorrhage (PPH). Results Most women had thrombocytopenia at delivery. Treatment with prednisone was given in 14 (27%) pregnancies with responses considered safe for delivery in 11 pregnancies (79%). Women in eight pregnancies also received intravenous immunoglobulin; in five pregnancies (63%) a platelet response acceptable for delivery was achieved. Seventeen pregnancies (33%) were complicated by a PPH ≥500 mL. Ten pregnancies (19%) were complicated by a PPH ≥1000 mL. PPH was reported in all women with a platelet count <50 × 109/L at delivery. Conclusions There were no antenatal bleeding complications but PPH was common among women with platelet counts <50 × 109/L at the time of birth.

Accidental Intra-Arterial Injection: A Case Report, New Treatment Modalities, and a Review of the Literature

2006 ◽  
Vol 64 (6) ◽  
pp. 965-968 ◽  
Author(s):  
John R. Keene ◽  
Karen M. Buckley ◽  
Stephen Small ◽  
Gerald Geldzahler
Keyword(s):  
Case Report ◽  
Treatment Modalities ◽  
Review Of The Literature ◽  
New Treatment

scholarly journals Comparison of the effects of antibody-coated liposomes, IVIG, and anti-RBC immunotherapy in a murine model of passive chronic immune thrombocytopenia

Blood ◽  
2006 ◽  
Vol 109 (6) ◽  
pp. 2470-2476 ◽  
Author(s):  
Rong Deng ◽  
Joseph P. Balthasar
Keyword(s):  
Monoclonal Antibody ◽  
Immune Thrombocytopenia ◽  
Dependent Manner ◽  
Antiplatelet Antibody ◽  
Thrombocytopenic Purpura ◽  
Platelet Counts ◽  
Antibody Immunotherapy ◽  
New Treatment ◽  
Dose Dependent ◽  
Chronic Immune Thrombocytopenia

Abstract The present work evaluated antibody-coated liposomes as a new treatment strategy for immune thrombocytopenic purpura (ITP) through the use of a mouse model of the disease. Effects of antimethotrexate antibody (AMI)–coated liposomes and intravenous immunoglobulin (IVIG)–coated liposomes (15, 30, 60 μmol lipid/kg) were compared with the effects of IVIG (0.4, 1, 2 g/kg) and anti–red blood cell (anti-RBC) monoclonal antibody immunotherapy (TER119, 5, 15, 25, and 50 μg/mouse) on MWReg30-induced thrombocytopenia. Each treatment was found to attenuate thrombocytopenia in a dose-dependent manner and, consistent with previous work, IVIG was found to increase antiplatelet antibody clearance in a dose-dependent manner. TER119 demonstrated greater effects on thrombocytopenia relative to other therapies (peak platelet counts: 224% ± 34% of initial platelet counts for 50 μg TER119/mouse versus 160% ± 34% for 2 g/kg IVIG, 88% ± 36% for 60 μmol lipid/kg AMI-coated liposomes, and 80% ± 25% for 60 μmol lipid/kg IVIG-coated liposomes). However, the effects of TER119 were associated with severe hemolysis, as TER119 decreased RBC counts by approximately 50%. The present work demonstrated that antibody-coated liposomes attenuated thrombocytopenia in this model at a much lower immunoglobulin dose than that required for IVIG effects and, in contrast with TER119, antibody-coated liposomes increased platelet counts without altering RBC counts.

scholarly journals The Frequency of Primary Immune Thrombocytopenia (idiopathic thrombocytopenic purpura) in Patients with Isolated Thrombocytopenia

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4982-4982
Author(s):  
Anait L. Melikyan ◽  
Elena K Egorova ◽  
Elena I Pustovaya ◽  
Tamara I Kolosheynova ◽  
Irina N Subortseva ◽  
...  
Keyword(s):  
Idiopathic Thrombocytopenic Purpura ◽  
Immune Thrombocytopenia ◽  
Thrombocytopenic Purpura ◽  
Female Ratio ◽  
Hematological Diseases ◽  
Viral Hepatitis C ◽  
Number Of Patients ◽  
Primary Immune Thrombocytopenia ◽  
Group 2 ◽  
Group 1

Abstract CONTEXT: Many hematological and non-hematological diseases can be hidden under the mask of isolated thrombocytopenia. The choice of therapeutic tactics is determinated by correct diagnosis. OBJECTIVE: to define the frequency of occurrence of primary immune thrombocytopenia (idiopathic thrombocytopenic purpura-ITP) in the group of patients with isolated thrombocytopenia. Materials and methods: We analysed clinical and laboratory data of 301 patients who applied to the outpatient department of National Research Center for hematology, Russian Federation with thrombocytopenia of unspecified origin. The first group is 183 patients who applied for the first time. The second group is 118 patients with long history of ITP. All patients were examined according to the extended differential diagnostic protocol used in isolated thrombocytopenia and based on international and National clinical recommendations for the diagnosis and treatment of ITP in adults. Results: Median age of patients in both groups was 36 years, male/female ratio in group 1 was 1:2, in group 2 - 1:4. In group 1, the count of platelets in the blood was more than 50*109/l in 87% of cases, while in the second group, in most cases (94%), there was a decrease in the count of platelets <50*109/l. Among the patients of the first group, haemorrhagic syndrome was absent in 50% of cases, even with platelet count less than 50*109/l. In the second group, 88% of patients complained of haemorrhages on the skin and mucosa, in 2% of cases life-threatening bleeding (uterine and gastrointestinal) developed (table 1). The examination carried out according to the protocol allowed to establish the diagnosis of ITP in group 1 in 88 (48%) patients, in group 2 in 100 (85%). Thus, the ratio of primary and secondary thrombocytopenia in group 1 was 1:1, in group 2 - 6:1. (fig. 1). The causes of secondary thrombocytopenia in group 1 were: increased consumption syndrome with thrombogenic complications in 16 (9%) patients, autoimmune diseases, occurring with isolated thrombocytopenia in 13 (7%) cases, virus-associated thrombocytopenia in 12 (7%) patients, drug-induced thrombocytopenia in 8 (4%) patients with diseases of the cardiovascular system, long-taking anticoagulants and disaggregants, in 7 (4%) cases of chronic viral hepatitis C, in 4 (2%) - liver cirrhosis of non-viral etiology, in 4 (2%) HIV infection, in 4 (2%) lymphoproliferative disease, in 2 (1%) acute leukemia, in 3 (2%) cases myelodysplastic syndrome (MDS), 14 (8%) women were diagnosed with gestational thrombocytopenia, EDTA-associated false thrombocytopenia was detected in 8 (4%) patients. Repeated examination of patients of the second group was carried out in the following cases: early relapse, resistance to corticosteroid therapy or loss of response after any line of therapy, incompliance of haemorrhagic syndrome with the count of platelets, the presence of thrombosis in the history, causing doubts in the diagnosis of ITP. The diagnosis of ITP in this group was changed to antiphospholipid syndrome in 4 patients, MDS in 4 cases, in 2 - systemic lupus erythematosus, in 3 - primary immunodeficiency and 2 patients, aged 40 and 44 years were found to have a genetic abnormality - abnormality Meya-Hegglina and thrombasthenia Glanzmann. A comparison of the number of patients diagnosed with ITP and secondary thrombocytopenia by age groups showed that secondary thrombocytopenia are more common at the age of 60 years (38% versus 19%, respectively) (Fig. 2). Conclusion: This study clearly presents a variety of hematological and non-hematological diseases occurring with isolated thrombocytopenia, which indicates the ambiguity of such concepts as the symptom of isolated thrombocytopenia and primary immune thrombocytopenia and requires a complete examination not only in the onset of the disease, but also in the recurrence of ITP. Disclosures No relevant conflicts of interest to declare.

scholarly journals Splenectomy for Primary Immune Thrombocytopenia Revisited at the Era of Thrombopoietin Receptor Agonists: New Insights for an Old Treatment

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 17-17
Author(s):  
Arthur Mageau ◽  
Louis Terriou ◽  
Mikael Ebbo ◽  
Odile Souchaud-Debouverie ◽  
Corentin Orvain ◽  
...  
Keyword(s):  
Board Of Directors ◽  
Research Funding ◽  
Immune Thrombocytopenia ◽  
Sustained Response ◽  
White Pulp ◽  
Advisory Committees ◽  
Thrombocytopenic Purpura ◽  
Thrombopoietin Receptor ◽  
Primary Immune Thrombocytopenia

Abstract Introduction Although splenectomy is still considered as the most effective curative treatment for primary immune thrombocytopenia (ITP), its use has significantly declined in the last decade, especially since the emergence of thrombopoietin receptor agonist (TPO-RAs) and anti-CD20 monoclonal antibodies 1-3. The main objective of our study was to evaluate if splenectomy was still as effective in the modern era, particularly for patients who failed to respond to TPO-RAs and rituximab. One of the secondary objectives was to assess, among patients who did not respond to or relapse after splenectomy, the pattern of response to subsequent intervention with treatments used before splenectomy and particularly TPO-RAs. Methods This multicentre retrospective observational study involved adults who underwent surgical splenectomy for primary ITP in France from 2011 (authorization of TPO-RAs in France) to 2020. To be included in the study, patients had to fulfil the following criteria : age ³18 years, primary ITP diagnosis defined according to the usual international criteria 2. Patients with abnormal spleen histology (other than reactional lymphoid hyperplasia, white-pulp hypoplasia or red pulp hyperplasia) or yet definite secondary ITP were excluded. Response was defined according to international criteria 4. Sustained response was defined as the absence of ITP relapse at last visit. We performed univariable and multivariable logistic regression procedures to calculates the odds ratio associated with a sustained response. Results In total,185 patients, 98 (53 %) women, with median age at splenectomy of 43.3 [interquartile range 27.6-64.3] years, were included in 18 French university and general hospitals from the French reference center network. Most of the patients were splenectomised at the chronic phase of ITP (n=150, 81.1%) and only two patients had undergone surgery within 3 months after ITP onset. Of note, 100 (54.1%) and 135 (73.0%) patients received TPO-RAs and/or rituximab prior to the splenectomy, respectively. The median time of follow-up after splenectomy was 39.2 months [16.5-63.0]. Overall, 144 (77.8%) of patients had an initial response and 23 patients (12.4%) relapsed during follow-up leading to an overall rate of sustained response of 65.4%, similar to the one observed in the pre-TPO-RA's era 1. Characteristics of patients according to the period during which occurred the splenectomy is available in Table 1. Among the 14 patients who failed to respond to both eltrombopag and romiplostim prior to splenectomy a sustained response after splenectomy was observed in 7 (50%). Among the 13 patients who had failed after both TPO-RAs and rituximab, we observed a sustained response in 6 (46%). In the multivariate analysis, an older age (60-75 years: OR 0,39 [0,17-0,86], p=0.02; &gt;75 years: OR 0,28 [0,10-0,75], p=0.013) and a history of more than 4 treatment lines for ITP before splenectomy (OR 0.25 [0.08-0.66], p=0.010) were significantly associated with a lack of sustained response after splenectomy. TPO-RAs were used for 57/64 (89.1%) patients who failed to respond to splenectomy. Among them, 21 were treated with one TPO-RA (i.e. eltrombopag or romiplostim) which was previously used before splenectomy without any efficacy and a response was observed in 13 (62%) of them. Conclusions In conclusion, splenectomy seems to be still a relevant option for treating adult primary ITP not responding to TPO-RAs and rituximab. Patients who fail to respond or relapse after splenectomy should be re-challenged with TPO-RAs. 1. Kojouri, K., Vesely, S. K., Terrell, D. R. & George, J. N. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term 2. Provan, D. et al. Updated international consensus report on the investigation and management of primary immune thrombocytopenia. Blood Adv. 3.Neunert, C. et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 3, 3829-3866 (2019). 4. Rodeghiero, F. et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 113, 2386-2393 (2009). Figure 1 Figure 1. Disclosures Terriou: Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Ebbo: Grifols: Honoraria, Membership on an entity's Board of Directors or advisory committees; Amgen: Honoraria; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; Octapharma: Other: Attendance Grant; Sobi: Other: Attendance Grant. Viallard: Novartis: Consultancy; Amgen: Consultancy; Grifols: Consultancy; LFB: Consultancy. Jeandel: Novartis: Membership on an entity's Board of Directors or advisory committees, Other: Support for congress; Sobi: Membership on an entity's Board of Directors or advisory committees; Amgen: Other: Support for congress; GSK: Other: Support for congress; Pharming: Other: support for congress. Michel: Amgen: Consultancy; Novartis: Consultancy; Rigel: Honoraria; UCB: Honoraria; Alexion: Honoraria; Argenx: Honoraria. Godeau: Grifols: Consultancy; Sobi: Consultancy; Amgen: Consultancy; Novartis: Consultancy. Comont: Takeda: Honoraria, Research Funding; Novartis: Honoraria, Research Funding; Bristol Myers Squibb: Honoraria, Research Funding; AstraZeneca: Honoraria, Research Funding; Abbvie: Honoraria, Research Funding.

scholarly journals RECURRENT POLYP REVEALING A RARE CASE OF CERVICAL RHABDOMYOSARCOMA: ABOUT A CASE AND REVIEW OF THE LITERATURE

2021 ◽  
Vol 9 (02) ◽  
pp. 551-555
Author(s):  
Kamal Aboulfath ◽  
◽  
Mohamed Mahmoud Emed ◽  
K.M. Saoud ◽  
N. Mamouni ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Uterine Cervix ◽  
Rare Case ◽  
Therapeutic Strategy ◽  
Treatment Modalities ◽  
Perioperative Chemotherapy ◽  
Review Of The Literature ◽  
Cervical Mass ◽  
Cervical Polyp

Rhabdomyosarcoma of the uterine cervix is one of the rare histological types of cervical cancer, which occurs mainly in girls and women during genital activity. Given the aggressiveness of the disease, therapeutic strategy is based on the combination of the three treatment modalities (surgery -chemotherapy radiation therapy-).We report a case of cervical rhabdomyosarcoma in a 53 year old woman with no particular pathological history. The first sign reported was postmenopausal metrorrhagia with the presence of a cervical polyp. The bistourning of this polyp was in favour of cervical adenosarcoma. The extension workup showed a cervical mass without metastasis. The patient underwent surgery and underwent a hysterectomy without preservation of adnexae with anatomopathologic results that suggested a cervical rhabdomyosarcoma and a candidate for adjuvant chemo-radiotherapy. Rhabdomyosarcoma of the uterine cervix is a rare tumor that develops most often in young girls. It mainly shows locoregional extension. Treatment is based on surgery including conservative treatment as well as radical treatment associated with perioperative chemotherapy. The role of radiation therapy remains poorly defined.

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