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

Immune thrombocytopenic purpura (ITP) is an immune-mediated disorder characterized by the destruction of platelets and megakaryocytes due to autoantibodies against the platelet surface proteins. ITP without any apparent cause of thrombocytopenia is defined as primary ITP, and ITP in the setting of SLE is secondary ITP, which can be diagnosed after excluding other causes of thrombocytopenia by history, physical examination, and laboratory testing. Patients with ITP associated with SLE have higher median platelet count and less bleeding manifestations compared to the patients with primary ITP. It can be very challenging to diagnose primary ITP in SLE patients as other causes of thrombocytopenia including drug-induced thrombocytopenia, antiphospholipid syndrome, and thrombotic microangiopathic process should be ruled out. Corticosteroids are the main modality of treatment. IVIG can be used in severe cases. Splenectomy was found to be less effective in ITP associated with SLE compared to primary ITP. Control of disease activity with immunosuppressive therapy can be helpful in some cases associated with active disease flares in SLE patients.

Novel Approach in Cardiac Sarcoidosis: A Case Report Highlighting Abatacept as a Promising Treatment Option.

Cardiac sarcoidosis (CS) is a rare auto-immune disorder where immune cells form granulomas in the heart that may lead to potential arrhythmias and heart failure. Due to the low prevalence of CS, the management remains challenging, requiring a multidisciplinary approach. In addition to the management of the resulting arrhythmias and heart failure, corticosteroids and immunosuppressants are used as anti-inflammatories to prevent disease progression. Immunosuppressive regimens for the treatment of CS are not yet well established. Abatacept has been approved for rheumatoid arthritis and psoriatic arthritis and both are mainly Th1-driven autoimmune diseases. Even though there are several different drugs used to treat corticosteroid-dependent sarcoidosis, abatacept may represent a unique option as its side effects differ from other drugs like methotrexate, azathioprine, or mycophenolate, especially bone marrow and liver toxicity. We present the case of a 52-year-old CS patient treated with abatacept after the failure of methotrexate and mycophenolate mofetil. Our patient had a history of stage D heart failure with reduced ejection fraction (HFrEF) with ejection fraction (EF) of 15-20%, nonischemic cardiomyopathy (NICM) s/p left heart catheterization (LHC), CS diagnosed by positron emission tomography (PET), status post implantable cardioverter-defibrillator (ICD) implantation, lung sarcoid, paroxysmal atrial fibrillation, and aflutter, who followed with cardiology, rheumatology, and pulmonology. He had multiple admissions for heart failure exacerbations. The patient was initially diagnosed with pulmonary sarcoidosis after which he completed a small course of steroids. CT chest showed lymphadenopathy; however, endobronchial ultrasound (EBUS) did not show evidence of pulmonary sarcoidosis. During an admission for heart failure about four years later, cardiac PET CT showed CS, and rheumatology was brought on board. The patient initially refused steroids and steroid-sparing agents. At subsequent visits, the patient was amenable to medication and was started on methotrexate 10mg weekly. However, given worsening chronic kidney disorder, methotrexate was discontinued and mycophenolate 200mg daily was started. A couple of weeks after mycophenolate was started, the patient felt like "his throat was closing up" and his stomach was cramping, which was thought to be an allergic response to the mycophenolate so it was discontinued. He then received an abatacept infusion which he tolerated well. Currently, our patient has been referred for heart transplantation.

Adalimumab-associated Philadelphia chromosome positive acute lymphoblastic leukaemia in a patient with Crohn's disease.

The US Food and Drug Administration has approved TNF(Tumor necrosis factor) alpha inhibitors to manage a range of inflammatory conditions, including Crohn's disease, rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis and other inflammatory disorders. However, these inhibitors can potentially increase the risk of secondary blood malignancies due to TNF alpha's role in various cellular processes such as angiogenesis, cell cycle proliferation, apoptosis and differentiation. In this article, we present a unique case study of a patient who developed Philadelphia-positive acute lymphoblastic leukaemia (ALL) while receiving adalimumab, a potent monoclonal antibody that specifically binds to TNF-alpha. We describe the patient's successful treatment using standard-of-care chemotherapy and tyrosine kinase inhibitors, resulting in complete remission with no measurable residual disease. Furthermore, we conducted a literature review on this subject and identified five similar cases of ALL associated with TNF alpha inhibitors.

Osteitis Condensans Illi - An overlooked cause of low back pain mimicking sacroiliitis.

Osteitis Condensans Illi (OCI) is an underrecognized cause of low back pain involving iliac bones with relative sparing of sacroiliac joint. We present a case of 48-year-old female who was diagnosed as a case of OCI after having back symptoms for 4 years.

Complete Remission of Advanced Hepatocellular Carcinoma in a Patient With Ulcerative Colitis Treated With Atezolizumab and Bevacizumab: A Case Report.

In this case study, a 73-year-old man who had previously undergone colectomy had a history of ulcerative colitis and alcohol abuse and presented with fatigue, weight loss, and a liver lesion. After a biopsy, he was diagnosed with stage IV-A hepatocellular carcinoma with poor differentiation and cirrhotic architecture, and molecular testing revealed positivity for multiple genes. A combination of atezolizumab and bevacizumab was administered, resulting in complete remission lasting beyond 16 months, demonstrating the potential of these drugs as a treatment option for advanced hepatocellular carcinoma (HCC). The patient's history of autoimmune conditions could have contributed to his robust response to the treatment. The report highlights the sustained survival benefits of this treatment beyond month 16.

COVID-19 Positive Status is Associated with Increased In-hospital Mortality in Patients with Acute Myocardial Infarction: A Systematic Review and Meta-analysis.

Patients hospitalized for acute myocardial infarction (AMI) may have concomitant positive coronavirus disease 2019 (COVID-19). We aimed to compare the risk of in-hospital mortality in patients primarily hospitalized for AMI with or without concomitant COVID-19 positive status. Using the random-effects model, we conducted a systematic review and meta-analysis of published articles from December 1, 2019, to April 1, 2022. There were eight studies with 10,128 patients, including 612 patients with COVID and 9516 patients without COVID. A total of 261 patients (42.64%) with COVID-19 positive and 612 patients (6.43%) with negative COVID-19 status died in the hospital. Pooled data showed that patients with a primary diagnosis of AMI with COVID-19 infection had more than five times increased risk of in-hospital mortality compared to patients without COVID-19 (OR: 5.06, 95% CI: 3.61, 7.09; I2 = 35%, P < 0.001). However, pooled data from five studies with adjustment of baseline differences in patient demographics and characteristics, comorbidities, and in-hospital pharmacology revealed more than three times increased risk of in-hospital mortality compared to patients who had primary AMI without COVID-19 infection (aOR: 3.47, 95% CI: 2.21, 5.45; I2 = 0%, P < 0.001). In subgroup analysis, ST-elevation myocardial infarction (STEMI) had lower in-hospital mortality (OR 4.23, 95% CI: 3.31, 5.40; I2 = 0%, P < 0.001) compared to non-ST-segment elevation myocardial infarction (NSTEMI) (OR 9.97, 95% CI: 5.71, 17.41; I2 = 0%, P < 0.001) (p-value = 0.006). Our study shows that COVID-19 infection is associated with increased in-hospital mortality in patients with index hospitalization for AMI.

A Rare Case of Hashimoto's Encephalopathy With Mosaic Turner Syndrome.

Mosaicism in Turner syndrome (TS) is a 20%-30% occurrence, with 45, X plus at least another cell line. The haploinsufficiency of the X chromosome is usually responsible for the higher risk of autoimmunity in TS, exhibiting mainly as thyroiditis, type 1 diabetes, etc. Though Hashimoto's thyroiditis is commonly seen in patients with TS, the concurrence of encephalopathy in these patients is significantly rare and has not been reported. We present a case of a young female with mosaic TS who presented with altered mental status. The initial workup was negative for stroke and pulmonary embolism and cerebrospinal fluid (CSF) analysis did not show any infectious etiology. Thyroid peroxidase (TPO) antibodies (Abs) and thyroglobulin Abs were elevated. As the patient's mental status deteriorated, there was a concern for Hashimoto's encephalopathy (HE), hence the patient was started on high-dose IV steroids. Within 24 hours, the patient responded to the IV steroids and an improvement in mentation was noted. HE is a rare immune-mediated disorder, characterized by impaired brain function. The onset of which can be rapid or slowly developing over the course of many years but responds effectively to steroids. Turner syndrome is associated with a high incidence of autoimmune disorders, thus in the setting of a negative workup for more obvious causes, HE should be a consideration when encountered in a clinical scenario.