Primary Immune Thrombocytopenic Purpura (ITP) and ITP Associated with Systemic Lupus Erythematosus: A Review of Clinical Characteristics and Treatment Modalities.

This study was conducted to compare the platelet count and the presence of bleeding manifestations at initial diagnosis of immune thrombocytopenic purpura (ITP) between patients with primary and secondary ITP. Medical records for 67 consecutive adult patients with ITP were reviewed retrospectively and the relevant data were abstracted. Thirty-eight (56.7%) patients were diagnosed as having primary ITP and 29 (43.3%) were considered to have secondary ITP. At the time of diagnosis, the median initial platelet count (median: 60 × 10(9)/L) for patients with secondary ITP was significantly (P < .005) higher than that for patients with primary ITP (median: 3.5 × 10(9)/L). Ecchymosis and/or purpura was observed in 4 (13.8%) patients with secondary ITP and in 33 (86.6%) patients with primary ITP (P value <.005). In conclusion, patients with secondary ITP had higher platelet count at diagnosis and were less likely to present with bleeding manifestations than those with primary ITP.

Despite the accepted distinction between primary and secondary immune thrombocytopenic purpura (ITP), a systematic analysis of the incidence of secondary ITP is not available. The present study was aimed at verifying the frequency and, consequently, the approximate rates of prevalence and incidence of secondary ITP and analysing its clinical and laboratory characteristics in patients needing ordinary hospital treatment for ITP. The study was based on 79 consecutive, adult ITP patients admitted to three Italian hospitals in 2000-2002. Using data collected in a previous study on the appropriateness of hospital management of ITP, we evaluated the frequency of secondary ITP, with the diagnosis formulated on the basis of new acquisitions, derived its rates of prevalence and incidence, and examined the available clinical and laboratory parameters. At our case review, a diagnosis of secondary ITP could be formulated in 38% of the 79 patients. This frequency was significantly higher than that determined at the time the patients were discharged from hospital (13.9%) (P = 0.000). The derived rates of prevalence and incidence of secondary ITP in the general population were, respectively, 2.3 and 1.23 per 100 000 inhabitants per year. In comparison with patients with primary ITP, those with a secondary form more frequently had spleen enlargement (P = 0.000), hepatomegaly (P = 0.001) and lower haemoglobin values (P = 0.005). The high frequency of secondary ITP must be mainly attributed to the currently available knowledge about the nature of some forms of ITP. Particular contributors to the high frequency were cases secondary to infections and those observed in patients who had undergone bone marrow or solid organ transplantation. Some clinical and laboratory alterations appear to be more frequent in secondary ITP than in primary ITP. However, the importance that the identification of particular forms of ITP, such as those secondary to Helicobacter pylori or hepatitis C virus infections, has on the choice of treatment suggests that these conditions must be ascertained independently of the presence or absence of clinical and laboratory alterations.

Other Immune Thrombocytopenias

We conducted a retrospective survey to assess prescription practice, response rates to rituximab, and the predictive indicators for a response to rituximab therapy in patients with primary or secondary immune thrombocytopenic purpura (ITP). Data were collected retrospectively from 40 consecutive patients with ITP attending our hospital: 29 (72.5%) had primary ITP and 11 (27.5%) had secondary ITP. Rituximab was given either as four weekly injections (375mg/m(2)) or two injections of 1,000mg given 2weeks apart in 30 and 10 patients, respectively. The primary objective was to evaluate overall (OR) and complete response (CR) to rituximab therapy for ITP. OR was excellent and rapid and similar when secondary ITP was excluded from the analysis: OR was achieved in 28 (71.8%) patients and CR in 22 (56.4%). But, at >6months, of the 28 responders, only 10 of 22 of the evaluable responses (45.5%) were sustained. In addition, except for the nonsignificant occurrence of antinuclear antibodies, no clinical or biological factors were predictive for OR or CR after the rituximab therapy. Twelve patients received a second course of rituximab. Overall, rituximab therapy achieved a response in two thirds of the patients, but the responders exhibited a high rate of early relapses, with no obvious difference according to the regimen of administration or rituximab dose.

Immune thrombocytopenic purpura (ITP) is an acquired thrombocytopenia where autoantibodies are generated against platelet antigens. Primary ITP is often idiopathic, whereas secondary ITP has many potential causes, including drug induced, infection related (human immunodeficiency virus, hepatitis C), leukemias, or autoimmune such as systemic lupus erythematosus. ITP is a common cause of thrombocytopenia in asymptomatic individuals, where evidence of bleeding may be minor or absent. Chronic silent bleeding leading to extreme anemia in patients with ITP is rare, and evidence of multiorgan damage is even rarer; hence the relevance of this case report. Here we describe a case of primary ITP with severe chronic blood loss leading to profound anemia causing renal failure and a type II non–ST elevation myocardial infarction. Our patient underwent extensive workup for the etiology of both thrombocytopenia and anemia and was eventually treated with packed red blood cell and platelet transfusions, along with intravenous steroids and immunoglobulin therapy.

Idiopathic Thrombocytopenic Purpura

Pathobiology of Secondary Immune Thrombocytopenia

Objective: Immune thrombocytopenic purpura (ITP) is the most common cause of thrombocytopenia in children. This retrospective study was designed to Analyze presenting features of ITP cases in Benha, evaluate outcomes among children and Determine the prognostic factors like age, gender, residency, history of preceding vaccination and infection, complaint, initial platelet count and initial treatment modalities as guidelines for new cases at the time of diagnosis.Method: Records of 308 children with ITP in Benha University Hospitals and Benha Children Hospital haematology clinics between May 2014 and January 2021 were retrospectively analyzed. Socio-demographic, clinical, and laboratory data of the studied children were recorded like age, gender, residence, date of diagnosis, complaint at presentation, preceding vaccination or infection, type of bleeding, initial platelet count, LDH(lactate dehydrogenase) level, initial treatment, and follow up of the disease.Results: A total of 308 children diagnosed with ITP were included in our study, clinical courses were determined as acute and chronic in 71.4% and 28.6% respectively. The median age of patients on diagnosis was 5 ± 3.4 years. The male/female ratio was 1.14. Median age at diagnosis was significantly higher in chronic ITP (p0.001), Patients with age ≥10 years developed chronicity more than younger cases (p=0.029). Regarding residency, seasonality, type of bleeding and history of preceding infection or vaccination, the difference was not statistically significant. Platelet count 20 ×109 was significantly higher in chronic ITP(P0.001). LDH level at presentation was significantly higher in chronic cases (p=0.046). Initial lines of treatment of patients admitted were steroids in 88%, IVIG in 5.2%, IVIG with steroids in 2.9% while 3.9% didn't receive any treatment, there was no significant difference in outcome between initial lines of treatment(P=0.105).Conclusion: In our study, age 10 years, females, Initial high platelet count and high LDH level at presentation were found to increase the probability of chronic ITP.Keywords: pObjective: Immune thrombocytopenic purpura (ITP) is the most common cause of thrombocytopenia in children. This retrospective study was designed to Analyze presenting features of ITP cases in Benha, evaluate outcomes among children and Determine the prognostic factors like age, gender, residency, history of preceding vaccination and infection, complaint, initial platelet count and initial treatment modalities as guidelines for new cases at the time of diagnosis.Method: Records of 308 children with ITP in Benha University Hospitals and Benha Children Hospital haematology clinics between May 2014 and January 2021 were retrospectively analyzed. Socio-demographic, clinical, and laboratory data of the studied children were recorded like age, gender, residence, date of diagnosis, complaint at presentation, preceding vaccination or infection, type of bleeding, initial platelet count, LDH(lactate dehydrogenase) level, initial treatment, and follow up of the disease.Results: A total of 308 children diagnosed with ITP were included in our study, clinical courses were determined as acute and chronic in 71.4% and 28.6% respectively. The median age of patients on diagnosis was 5 ± 3.4 years. The male/female ratio was 1.14. Median age at diagnosis was significantly higher in chronic ITP (p0.001), Patients with age ≥10 years developed chronicity more than younger cases (p=0.029). Regarding residency, seasonality, type of bleeding and history of preceding infection or vaccination, the difference was not statistically significant. Platelet count 20 ×109 was significantly higher in chronic ITP(P0.001). LDH level at presentation was significantly higher in chronic cases (p=0.046). Initial lines of treatment of patients admitted were steroids in 88%, IVIG in 5.2%, IVIG with steroids in 2.9% while 3.9% didn't receive any treatment, there was no significant difference in outcome between initial lines of treatment(P=0.105).Conclusion: In our study, age 10 years, females, Initial high platelet count and high LDH level at presentation were found to increase the probability of chronic ITP.Keywords: primary ITP, ITP outcome, ITP prognostic factors.Abbervations: ITP : Immune thrombocytopenic purpura; LDH: lactate dehydrogenaseAbbervations: ITP : Immune thrombocytopenic purpura; LDH: lactate dehydrogenase

Recognizing and Treating Secondary Immune Thrombocytopenic Purpura Associated With Lymphoproliferative Disorders

Secondary immune thrombocytopenic purpura (ITP) with non-Hodgkin lymphoma (NHL) is a rare disease. Although some treatment regimens are available for primary ITP, the treatment strategy for secondary ITP remains unconfirmed. We herein report a 79-year-old man who was diagnosed with secondary ITP with mantle cell lymphoma. Although intravenous immunoglobulin (IVIG) has been considered an effective option for secondary ITP, similar to the treatment of primary ITP, our patient did not benefit from IVIG. A literature review including the current report revealed that IVIG was ineffective in all treated patients. Secondary ITP with NHL should be treated differently from primary ITP.

Background and Aims: Immune thrombocytopenic purpura (ITP) is an acquired bleeding disorder characterized by autoantibodies against platelets. The clinical presentation is variable; the main symptom is bleeding, and many patients are asymptomatic; others have nonspecific symptoms like fatigue. Uncommonly, ITP can present with paradoxical thrombosis. The risk of thrombosis in ITP may be higher than expected, which makes the management of ITP more challenging. This review aims to evaluate patients with ITP who develop thrombosis and identify potential risk factors related to thrombosis in this category of patients.Materials and Methods: English literature was searched using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines for adults above 18 years with primary ITP who had infarctions or thrombotic events. Patients with secondary ITP were excluded. The search included articles published up to 20th October 2021.Results: A total of 73 articles were included. Seventy-seven patients with ITP had developed infarctions and various thrombotic events. Sixty-three patients had arterial events, and 14 patients developed venous thrombotic events.Conclusion: Patients with ITP have low platelets, which predispose them to bleed; despite that, serious thrombotic complications can happen in these patients and are difficult to predict. Therefore, it is critical for physicians to understand that ITP is paradoxically a prothrombotic condition and to address preventive thromboembolic measures whenever possible.

Mortality and Comorbidities of Covid-19 Patients with Immune Thrombocytopenic Purpura - a Review of the National Database 2019-2020

Immune thrombocytopenic purpura (ITP) is characterized by thrombocytopenia and bleeding diathesis. Pancreatitis is a very rare complication but may be fatal. We analyzed data of newly diagnosed ITP patients, excluding those with a history of splenectomy, unknown sex or date of birth, or preexisting pancreatitis at the time of ITP diagnosis, and compared these with selected age-, gender-, and index-year-matched controls, using the Taiwan National Health Insurance Research Database from 1996 to 2013. The study enrolled 100,177 ITP patients and 100,177 controls. We found that pancreatitis risk was higher in secondary ITP patients, regardless of age group, gender, baseline Charlson comorbidity index (CCI) score, history of biliary stone, hyperlipidemia, or alcoholism, than in the control population. Primary ITP patients with CCI score 1 and without biliary tract stone history also showed a higher pancreatitis risk than the controls. The incidence rate and cumulative incidence of pancreatitis were increased in primary, secondary, and unspecified ITP cases. These phenomena may be related to the presence of autoantibodies against glycoprotein IIb/IIIa, or to IgG4, microparticle obstruction, or sclerosis. We noted a direct association between ITP and the development of pancreatitis in Taiwan population.

Patient: Male, 27-year-oldFinal Diagnosis: Immune thrombocytopenic purpura (ITP)Symptoms: Bleeding • purpuraMedication: Azathioprine • eltrombopag • fostamatinib • intravenous immunoglobulin • IVIG • prednisone • rituximab • steroidsClinical Procedure: EGD • PET-CT • plasmapheresis • splenectomySpecialty: Gastroenterology and Hepatology • Hematology • General and Internal MedicineObjective:Unusual clinical courseBackground:Immune thrombocytopenic purpura (ITP) is primarily caused by antibody-mediated destruction of platelets. Alterations in immune homeostasis can induce loss of peripheral tolerance and promote the development of self-reactive antibodies.Primary ITP is the diagnosis of exclusion made after the extensive work-up rules out other possible causes of thrombocytopenia. The association between the ITP and other autoimmune disorders is well-established. In recent years, increasing attention has been directed toward the association between celiac disease (CD) and ITP.Case Report:A 27-year-old man with a history of primary ITP presented with an occasional nosebleed, 1 episode of rectal bleeding, and easy bruising. The patient was later found to have high titers of TTG-IGA and endomysial IGA levels consistent with CD. Our patient not only failed to improve with the gluten-free diet, but also failed multiple lines of treatment including steroids, IVIG, rituximab, eltrombopag, and even a non-traditional treatment for ITP (azathioprine and plasma exchange). The patient’s CD-related antibody titers remained elevated.Conclusions:It is possible that in certain cases the alteration of immune response caused by CD with a concurrent elevation of CD-related antibodies can make ITP refractory to all medical management. Whether or not this refractoriness to treatment is related to the persistently elevated antibody titers of CD or unknown genetic relationship between ITP and CD remains not entirely clear and warrants further molecular, immunologic, and genetic analysis.

Thrombopoietin Levels May Predict Responsiveness to Therapy with Thrombopoietin Agonists Among Patients with Immune Thrombocytopenic Purpura,