Drug-free remission of immune thrombocytopenic purpura following resection of perihilar cholangiocarcinoma: a case report.

Immune thrombocytopenic purpura (ITP) is a rare autoimmune disorder. Although secondary ITP, caused by various underlying diseases, including some malignant tumors, has been reported, instances of ITP resolving after successful treatment of the underlying cause are uncommon and noteworthy. Herein, we present a case of a patient with perihilar cholangiocarcinoma (pCCA) and ITP who achieved drug-free remission of ITP following tumor resection. A 76-year-old man presented with pCCA complicated by ITP. Prednisolone treatment successfully managed his thrombocytopenia, allowing for a left hepatopancreatoduodenectomy. ITP relapse did not occur after discontinuation of prednisolone postoperatively. This case suggests that surgical resection of the underlying malignancy may induce remission of secondary ITP associated with pCCA.

Elective splenectomy for hematological diseases: a vanishing indication.

INTRODUCTION: Splenectomy has been used as a diagnostic and therapeutic tool in the management of hematological diseases for many years. However, the emergence of new medical therapies has modified guidelines for many hematological diseases for which splenectomy was previously considered. We aimed to evaluate the evidence of a decrease in the hematological indications for splenectomy and the reasons and justifications for this change. MATERIAL AND METHODS: We conducted a single-center, retrospective analysis of patients who underwent laparoscopic splenectomy for hematological disease between January 2010 and December 2023. Patients were classified into four groups: 1 autoimmune and hemolytic diseases (HAD), (2) lymphomas, (3) myeloproliferative diseases (MPN), and (4) other splenic diseases. We recorded the annual incidence of splenectomy and the ratio of new medical cases, demographic and clinical data and surgical outcomes. RESULTS: During the study period, 98 patients were referred for splenectomy. There was a significant progressive decrease in this surgical indication, particularly regarding HAD (p < 0.001). The indication for splenectomy for immune thrombocytopenic purpura (ITP) declined to zero despite an increase in the number of patients diagnosed with this disorder (p < 0.001). The pattern of decrease in AHAI and Evans syndrome was similar to that in ITP. The group of splenectomies due to lymphoma persisted consistently during the study period, as did the indication for splenectomy in the context of lymphoma treatment. Splenectomy due to massive splenomegaly secondary to MPN was indicated only in one patient. Splenectomies due to other causes were similarly distributed over the years. CONCLUSIONS: Our findings confirm a significant decrease in the indication for elective surgery for hematological diseases, mainly regarding autoimmune disease. The surgical community and surgical departments should be aware of this situation yet maintain the skills to adopt this technique both safely and efficiently.

Effect of Immune Thrombocytopenic Purpura Medications on Depression Risk: An Analysis of NHANES Data.

BACKGROUND: Immune thrombocytopenic purpura (ITP) in adults typically develops slowly and insidiously. The ITP medications might be linked to psychological disorders, but the connection is not well-understood. OBJECTIVE: This study aimed to examine the association between ITP medication use and the risk of depression among participants in the National Health and Nutrition Examination Survey (NHANES) from 2005 to 2018. METHODS: Using data from 70 190 NHANES participants, we conducted a cross-sectional study, excluding individuals under 18 years, with hypertension, HIV, hepatitis C, and various comorbidities. A total of 17 299 individuals were included in the analysis of this study. We identified 2 populations within this study: those using ITP medications, including prednisone, dexamethasone, and rituximab and those not using ITP drugs. Depression status was assessed using the Patient Health Questionnaire-9 (PHQ-9), and the relationship between ITP medication use and depression was analyzed through multivariate logistic regression. RESULTS: There was no significant association between ITP medication use and an increased risk of depression after adjusting for demographic and health-related variables. Notably, among the study participants, 1.8% of the non-depressed population were on ITP medication compared with 0.3% in the depressed population. The analysis revealed varying depression risks associated with different sociodemographic factors. For instance, the correlation between ITP medication and depression risk was influenced by a combination of age, race, income, and smoking status. CONCLUSION AND RELEVANCE: The study suggests that ITP medication use does not independently increase the risk of depression. This finding is crucial for guiding clinical decisions and managing patient expectations regarding ITP treatment and its psychological impacts.

Intracerebral Hemorrhage in a Patient With Newly Diagnosed Immune Thrombocytopenic Purpura Precipitated by a Viral Illness.

Intracerebral hemorrhage (ICH) is a rare and severe complication of immune thrombocytopenic purpura (ITP) that can be spontaneous. Viral illnesses, other infections, autoimmune disorders, and medications can cause ITP. ITP causes a significant decrease in platelet levels, increasing bleeding risk. ITP can be treated by steroids, intravenous immunoglobulin, plasmapheresis, platelet transfusion, biological agents, and splenectomy. ICH treatment involves the treatment of underlying ITP, as well as any neuro-interventional procedures needed. In this case report, we look at the presenting symptoms and treatment course of an interesting case of ICH in a patient who developed ITP after a viral upper respiratory infection.

Hemostatic mechanism of Jianpi Yiqi Shexue decoction in treatment of immune thrombocytopenia.

OBJECTIVE: To explore the early hemostatic mechanism of Jianpi Yiqi Shexue decoction (, JYSD) in treating immune thrombocytopathy (ITP), based on the functional homeostasis of brain-intestine axis and blood neurotransmitter METHODS: Non-drug treatment cases: Healthy volunteers were selected as normal control group and compared with patients with dysfunctional uterine bleeding, gastrointestinal tumors with bleeding and ITP, to detect the changes of blood 5-hydroxytryptamine (5-HT), ß-endorphin (ß-EP), vasoactive intestinal peptide (VIP) and compare the changes of blood neuro-transmitters in patients with different disease symptoms. Drug treatment cases: According to the randomized controlled multicenter clinical trial, 272 ITP patients were randomly divided into three groups: treatment group (JYSD) combined group (JYSD + Prednisone) control group (Prednisone). The changes of blood neuro-transmitter (5-HT, ß-EP, VIP) before and after treatment were detected on the basis of peripheral blood platelet (PLT) and grade score. RESULTS: Non-drug treatment cases: compared with the normal control group, the 5-HT level was higher, and the VIP and ß-EP levels were both lower in the ITP group (P < 0.001), and the 5-HT, VIP and ß-EP levels in the Gastrointestinal tumors with bleeding group were also lower compared with the normal control group (P < 0.05, 0.001). Drug treatment cases: The PLT grading scores of the combination group and the control group after treatment were lower than that before treatment (P < 0.05, 0.001). The PLT grading score of the 3 groups were compared in pairs after treatment: the combination group was the lowest among the 3 groups, which was better than the treatment group, but no better than the control group (vs the treatment group, P = 0.005, vs the control group, P = 0.709). The statistical results of full analysis set (FAS) and per protocol set (PPS) were consistent. The bleeding symptom scores of the treatment and combination groups began to drop 7 d after treatment, and kept dropping 14 d after treatment until the end of the study (P < 0.05). On the other hand, the control group started to show favorable results 14 d after treatment (P < 0.05). The FAS and PPS analysis results were consistent. In the control group, the 5-HT level was higher and VIP level was lower after treatment, compared with those before treatment (P < 0.05, 0.001). The ß-EP levels were both increased in the treatment and combination group after treatment, compared with those before treatment (P < 0.05). After treatment, the ß-EP levels in the treatment and control groups were significantly lower compared with the combination groups (P < 0.05). After treatment, compared with the control group, the VIP levels in the treatment and combination groups were up-regulated, and the differences were statistically significant by rank sum test (P < 0.01), and by t-test (P = 0.0002, 0.0001). CONCLUSIONS: The prednisone tablet is better than the JYSD in increasing the level of PLT, while prednisone tablet combined with JYSD has more advantages in improving patients' peripheral blood PLT levels. However, in improving the bleeding time of ITP patients, the combination of the two drugs was significantly delayed compared with the single usage, showing the characteristics and advantages of traditional Chinese medicine. JYSD can regulate the neurotransmitter level of ITP patients through the function of the brain-gut axis, mobilize 5-HT in the blood of ITP patients to promote the contraction of blood vessels and smooth muscles, and activate the coagulation mechanism are the early hemostatic mechanisms of JYSD. Up-regulate the levels of ß-EP and balancing VIP levels may be an important part of the immune mechanism of JYSD for regulating ITP patients.

Immune Thrombocytopenic Purpura (ITP) and Chorioretinopathy in Chronic Granulomatous Disease: A Case Report.

BACKGROUND: Chronic Granulomatous Disease (CGD) is a rare immunodeficiency disorder characterized by impaired phagocytic function, leading to recurrent infections and granuloma formation. Genetic mutations in NADPH oxidase complex components, such as CYBB, NCF1, NCF2, and CYBA genes, contribute to the pathogenesis. This case report explores the possible ocular and hematologic complications associated with CGD. CASE PRESENTATION: A 6-year-old girl with a history of vitrectomy, membranotomy, and laser therapy due to congenital blindness (diagnosed with chorioretinopathy) was referred to the hospital with generalized ecchymosis and thrombocytopenia. Diagnostic workup initially suggested chronic immune thrombocytopenic purpura (ITP). Subsequent admissions revealed necrotic wounds, urinary tract infections, and recurrent thrombocytopenia. Suspecting immunodeficiency, tests for CGD, Nitroblue tetrazolium (NBT) and dihydrorhodamine (DHR) were performed. She had a low DHR (6.7), and her NBT test was negative (0.0%). Her whole exome sequencing results confirmed autosomal recessive CGD with a homozygous NCF1 mutation. CONCLUSION: This case underscores the diverse clinical manifestations of CGD, including recurrent thrombocytopenia and possible early-onset ocular involvement. The diagnostic challenges highlight the importance of a multidisciplinary approach involving hematologists, immunologists, and ophthalmologists for accurate diagnosis and management. The rare coexistence of ITP in CGD emphasizes the intricate link between immunodeficiency and autoimmunity, requiring tailored therapeutic strategies.

Chronic Myelomonocytic Leukemia-Associated Immune Thrombocytopenic Purpura: A Report of a Rare Case and a Review of Literature.

Chronic myelomonocytic leukemia (CMML) presents as a complex hematologic malignancy with myelodysplastic and myeloproliferative features. Our case report explores the rare coexistence of CMML with immune thrombocytopenic purpura (ITP) in a 63-year-old female patient. CMML diagnosis followed World Health Organization criteria, and the patient was classified as having high-risk myelodysplastic syndrome (MDS)-CMML stage 2. Initial treatment with subcutaneous azacytidine for CMML proved partially effective, highlighting persistent severe thrombocytopenia. Subsequent investigations revealed secondary ITP associated with Crohn's disease. Conventional ITP therapies, including high-dose steroids and intravenous immunoglobulin, showed limited efficacy. Eltrombopag, a thrombopoietin receptor agonist, was initiated, resulting in the normalization of platelet counts within six weeks. Our case emphasizes the diagnostic challenges and intricate treatment landscape of CMML-associated ITP, suggesting eltrombopag as a potential therapeutic option in refractory cases. The study contributes to the evolving understanding of the complex interplay between myeloid disorders and immune-mediated hematological conditions, calling for personalized and multidisciplinary approaches to enhance patient outcomes.

Autoimmune cytopenia and Kabuki syndrome in paediatrics: Insights in 11 patients.

Kabuki syndrome (KS) is now listed in the Human Inborn Errors of Immunity (IEI) Classification. It is a rare disease caused by KMT2D and KDM6A variants, dominated by intellectual disability and characteristic facial features. Recurrently, pathogenic variants are identified in those genes in patients examined for autoimmune cytopenia (AIC), but interpretation remains challenging. This study aims to describe the genetic diagnosis and the clinical management of patients with paediatric-onset AIC and KS. Among 11 patients with AIC and KS, all had chronic immune thrombocytopenic purpura, and seven had Evans syndrome. All had other associated immunopathological manifestations, mainly symptomatic hypogammaglobinaemia. They had a median of 8 (5-10) KS-associated manifestations. Pathogenic variants were detected in KMT2D gene without clustering, during the immunological work-up of AIC in three cases, and the clinical strategy to validate them is emphasized. Eight patients received second-line treatments, mainly rituximab and mycophenolate mofetil. With a median follow-up of 17 (2-31) years, 8/10 alive patients still needed treatment for AIC. First-line paediatricians should be able to recognize and confirm KS in children with ITP or multiple AIC, to provide early appropriate clinical management and specific long-term follow-up. The epigenetic immune dysregulation in KS opens exciting new perspectives.

Total abdominal hysterectomy in a patient with immune thrombocytopenic Purpura: A case report.

INTRODUCTION: Immune Thrombocytopenic Purpura poses unique challenges in surgical settings due to an increased risk of bleeding. This report details the perioperative management of a patient with Immune Thrombocytopenic Purpura undergoing Total Abdominal Hysterectomy, emphasizing the importance of tailored approaches for such cases. CASE PRESENTATION: A 41-year-old female with Immune Thrombocytopenic Purpura and symptomatic uterine fibroids, despite medical management, opted for Total Abdominal Hysterectomy. Prednisolone therapy and platelet transfusion were used preoperatively to optimize platelet counts. DISCUSSION: Effective management was achieved through meticulous surgery, continued prednisolone therapy, and vigilant postoperative monitoring. This case highlights the value of a multidisciplinary approach in ensuring positive surgical outcomes for Immune Thrombocytopenic Purpura patients. CONCLUSION AND RECOMMENDATION: This case underscores the significance of individualized perioperative care for Immune Thrombocytopenic Purpura patients undergoing major surgery. By optimizing medical therapy and maintaining close monitoring, favorable results can be achieved, enhancing the quality of life for such patients. It is recommended that such comprehensive approaches are considered in similar cases.

Treatment of immune thrombocytopenia during pregnancy with thrombopoietin receptor agonists.

The introduction of thrombopoietin receptor agonists (TPO-RAs) led to a paradigm shift in the management of immune thrombocytopenia (ITP). However, TPO-RAs are not approved for use during pregnancy due to the absence of evidence and concerns for possible effects on the fetus due to their expected transplacental transfer. This comprehensive review examines the safety and efficacy of TPO-RA in 45 pregnancies of women with ITP (romiplostim n = 22; eltrombopag n = 21; both in the same pregnancy n = 2). Mothers experienced failure of the median of three treatment lines during pregnancy prior to TPO-RA administration. A platelet response (>30 × 109 /L) was seen in 86.7% of cases (including a complete response >100 × 109 /L in 66.7%) and was similar between eltrombopag and romiplostim (87.0% and 83.3%, p = 0.99). The maternal safety profile was favourable, with no thromboembolic events encountered. Neonatal thrombocytopenia was noted in one third of cases, with one case of ICH grade 3, and neonatal thrombocytosis was observed in three cases. No other neonatal adverse events attributable to TPO-RAs were seen. This review suggests that the use of TPO-RA during pregnancy is associated with a high response rate and appears safe. Nevertheless, TPO-RA should not be routinely used in pregnancy and should be avoided in the first trimester until further evidence is accumulated.

Evans syndrome during pembrolizumab therapy for upper urinary tract cancer.

Introduction: Immune checkpoint inhibitors are available for the treatment of advanced urothelial carcinoma; however, serious adverse events occasionally occur. Here, we report a rare case of Evans syndrome attributed to the use of an immune checkpoint inhibitor. Case presentation: A 56-year-old man was diagnosed with left renal pelvic cancer and underwent left laparoscopic radical nephroureterectomy. Eight months postoperatively, computed tomography revealed para-aortic lymph node metastasis. Despite receiving chemotherapy, the disease progressed, and pembrolizumab was initiated. After 26 months of pembrolizumab treatment, the patient developed fever and anemia. Hematologic examination confirmed the diagnosis of Evans syndrome. He was treated with blood transfusions and corticosteroids, and gradual symptom improvement was observed. Conclusion: This report highlights the potential risk of Evans syndrome associated with immune checkpoint inhibitor treatment. Clinicians should be aware of this possibility and consider early intervention with corticosteroids.

Refractory immune thrombocytopenic purpura (ITP) after accessory splenectomy: A case report and literature review.

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a platelet count of less than 100 x 109 /L, resulting from antibody-mediated platelet destruction. Treatment for ITP typically involves steroids, and intravenous immunoglobulins (IVIG) can be added. Splenectomy is performed in cases with refractory ITP. Rituximab can suppress immunity but has limited efficacy in ITP cases. Herein, we present a rare case of a 30-year-old female who was first diagnosed with ITP and underwent a splenectomy two years later. However, seven years after surgery, she was presented with symptoms of ITP. A splenic scan showed an accessory spleen in the spleen bed, for which she underwent accessory spleen removal surgery. Her laboratory tests three days post-operation showed a rise in platelet count and hence was discharged a few days later. The patient had recurrent attacks of ITP even after the removal of the normal and accessory spleen, suggesting that accessory spleen removal may not always be an effective treatment for ITP. The patient eventually died. While splenectomy is a common treatment for ITP, it may not always be effective in all cases, and other treatments such as bone marrow transplantation may be necessary.

A cost-utility analysis of thrombopoietin receptor agonists for treating pediatric immune thrombocytopenia purpura after failure of first-line therapies.

BACKGROUND: Thrombopoietin receptor agonists (TPO-RAs) have emerged as a recommended treatment for children with persistent and/or chronic immune thrombocytopenic purpura (ITP). The purpose of this study was to evaluate the cost-effectiveness of TPO-RAs relative to treatment without TPO-RAs (non-TPO-RAs/usual care) for ITP in children who do not respond to first-line therapy and in whom splenectomy is not recommended in Ontario, Canada, from a hospital payer perspective. PROCEDURE: A 2-year Markov model with an embedded decision tree was used. Data on medications used, dose, response rate, bleeding, and emergency treatment events were collected from the Hospital for Sick Children in Toronto. The health outcomes were described in quality-adjusted life-years (QALYs). Health-state utilities were derived from the peer-reviewed literature. Scenario analyses, deterministic, and probabilistic sensitivity analyses were conducted. Economic costs were measured in 2021 Canadian dollars ($1.00 = US$0.80) RESULTS: TPO-RAs are estimated to result in an increased cost of $27,118 and a QALY gain of 0.21 compared to non-TPO-RAs over a 2-year horizon, resulting in an incremental cost-effectiveness ratio (ICER) of $129,133. In a 5-year scenario analysis, the ICER fell to $76,403. In the probabilistic sensitivity analysis, TPO-RAs exhibit a 40.0% probability of being cost-effective at a conventional ($100,000) willingness-to-pay threshold per QALY gained. CONCLUSIONS: Further assessment of the long-term efficacy of TPO-RAs is warranted to obtain more precise long-term estimates. As the costs of TPO-RAs decline with the introduction of generic formulations, TPO-RAs may be increasingly cost-effective.

Insidious onset of spontaneous spinal epidural hematoma in immune thrombocytopenic purpura: a case-based review.

INTRODUCTION: Spontaneous spinal epidural hematoma (SSEH) can result from various etiologies with a variable degree of neurological deficits. Here, we describe a rare case of SEH secondary to immune thrombocytopenic purpura (ITP) in a child and review the literature of SSEH caused by ITP. CASE REPORT: A 9-year-old female who presented with rapid neurological decline, including bowel and bladder incontinence and paraparesis. A SSEH was observed extending from C2 to T6, causing a mass effect on the spinal cord. Her platelet count was only 7000/µL. Multidisciplinary care was established with neurosurgery, pediatric hematology, and pediatric surgery. The patient was managed emergently with splenectomy and surgical evacuation, with multilevel laminectomy and laminoplasty for evacuation of the hematoma. After a short course of rehabilitation, the patient regained all neurological function. CONCLUSION: We report the first case of cervicothoracic SSEH secondary to ITP in a child managed with emergent splenectomy and surgical evacuation with multilevel lamoplasty. We also described the methods of timely diagnosis, urgent management, and overall prognosis of patients with this condition.

Diffuse large B­cell lymphoma and monoclonal gammopathy secondary to immune thrombocytopenic purpura: A case report.

The present study reports the case of a patient with diffuse large B-cell lymphoma (DLBCL) and monoclonal gammopathy (MG) secondary to immune thrombocytopenia purpura (ITP). The clinical diagnoses and investigations of this case are reported. To the best of our knowledge, this is the first study to report DLBCL and MG secondary to ITP. The patient presented with a rare constellation of diseases, which made the diagnosis and treatment difficult for the physicians. The patient was followed up for 10 years using the morphological examination of bone marrow cells after chemotherapy, and currently continues with follow-up examinations. Treatments and prognoses for ITP, DLBCL and MG are common. However, treatments and prognoses are unclear for patients with all three conditions. The different clinical manifestations and disease processes of DLBCL and MG secondary to ITP cause difficulties for physicians in terms of treatment and prognosis. The present case report describes the comprehensive evaluation, diagnosis and treatment of a patient with DLBCL and MG secondary to, and concurrent with, ITP.

Immune Thrombocytopenic Purpura Presenting with Pituitary Apoplexy: A Case Report and Literature Review.

Background Pituitary apoplexy is a rare condition that usually occurs in the setting of a pituitary adenoma. It can present with symptoms of visual disturbances, vertigo, headache, and neurological impairments. Computed tomography (CT) scans can aid in identifying pituitary apoplexy and ruling out other diseases. We present a unique case of pituitary apoplexy in the setting of immune thrombocytopenic purpura (ITP). Case Description A 61-year-old man with a past medical history significant for myocardial infarction presented to the emergency department with symptoms of diplopia and headache 36 hours after onset. The patient was found to have severe thrombocytopenia with a platelet count below 20,000. A CT of the head revealed a possible pituitary adenoma with compression of the optic chiasm. The patient's platelet count continued to decrease throughout his admission and dropped below 7,000 on day 2 of admission. The patient was given platelet transfusion along with intravenous immunoglobulins. The patient underwent endoscopic transsphenoidal resection of the pituitary mass. Pathology of the mass revealed immature platelets characteristic of immune ITP in the setting of pituitary apoplexy. Conclusion While ITP in the setting of pituitary apoplexy is a rare entity, we believe that clinicians should have pituitary apoplexy on their differential diagnosis in patients with ITP.

Naproxen-Induced Evans Syndrome.

Evans syndrome is an autoimmune disorder characterized by the simultaneous occurrence of autoimmune hemolytic anemia and immune thrombocytopenic purpura. It can further be classified as primary Evans syndrome when it occurs by itself, or secondary Evans syndrome when it is associated with other autoimmune and lymphoproliferative disorders. Corticosteroids and immunoglobulins are the first-line treatments for primary Evans syndrome, and subsequent options include other immunosuppressive medications. Medical literature provides little information about the triggers of primary Evans syndrome. Knowing such information, however, is essential to recognize, treat and prevent the recurrence of the disease effectively.  We report a 68-year-old female who presented with shortness of breath, cough, bruises, scleral icterus, and dark urine after several days of naproxen therapy for pain. Further workup noted direct antiglobulin test positive for IgG, anemia, and thrombocytopenia. Imaging studies showed deep venous thrombosis. She was diagnosed with Evans syndrome and improved following prompt treatment with corticosteroids, anticoagulants, blood transfusion therapies, and discontinuation of naproxen. The prognosis of Evans syndrome is poor, variable, and characterized by relapses. Early diagnosis and treatment are therefore associated with better prognosis.  This case is critical because it shines a light on one of the major causes of Evans syndrome, reports a practical approach to treating the condition, and paves the way for future research on Evans syndrome. This case is also the first reported naproxen-induced Evans syndrome in the world's literature.

Impact of restrictive measures due to the Covid-19 pandemic on the incidence of immune thrombocytopenia in children: an Italian single center experience.

The COVID-19 pandemic has had a huge effect all over the world and its impact has been even more profound in the world of Healthcare. In this brief report we'd like to report about our experience in pediatric newly diagnosed thrombocytopenia and how we have seen the landscape of this disease change in the last 2 years. In particular, we believe that the use of personal protective equipment and lockdown measures have reduced the incidence of viral triggers that are supposed to be responsible for the vast majority of ITP cases. Along with these data, we found some other significant differences in the period taken into account.