Hoarseness as the initial presentation of multiple myeloma.

Key Clinical Message: Myeloma of the thyroid cartilage is a rare but important differential diagnosis of a laryngeal mass. Although hoarseness as the initial presenting symptom in multiple myeloma is extremely rare, a clinician should always consider it. Abstract: Multiple myeloma (MM) is a malignant plasma cell disorder characterized by an uncontrolled proliferation of monoclonal plasma cells. Although the clinical presentation at diagnosis can be quite variable, thyroid cartilage infiltration in MM is rare. Here we discuss a 65-year-old Caucasian male presenting to the ENT doctor with continuous hoarseness for 3 months. The initial clinical examination showed a tangible mass at the left lymph node level II-III. Further examination with fiber-optic laryngoscopy showed a bulging of the aryepiglottic and ventricular fold. Neck and chest CT scan revealed multiple osteolytic bone lesions in addition to the large lesion in the left thyroid cartilage. Laboratory work-up, PET-CT scan and biopsy of the thyroid cartilage were performed and eventually all confirmed the presence of a new diagnosis of IgA kappa MM. The patient was referred to the department of hematology to start with chemotherapy.

First report of a de novo iTTP episode associated with an mRNA-based anti-COVID-19 vaccination.

Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening thrombotic microangiopathy, characterized by disseminated thrombus formation in the microvasculature, causing severe organ failure. Immune-mediated TTP (iTTP) is occasionally described after vaccination, especially against viral agents. We report a case of a 38-year-old woman with a de novo iTTP after exposure to the mRNA-based anti-coronavirus disease 2019 (COVID-19) vaccine produced by Pfizer-BioNTech. She presented with increased bruising and petechiae starting 2 weeks after receiving the first dose of the anti-COVID-19 vaccine. Laboratory data revealed a severe ADAMTS13-deficiency in combination with a very high autoantibody titer against ADAMTS13. She was successfully treated with plasma exchange, corticosteroids, rituximab, and caplacizumab. To our knowledge, this is the first case report of iTTP after mRNA-based COVID-19 vaccination in a previously TTP-naïve patient.

A special case of hypertrophic cardiomyopathy with a differential diagnosis of isolated cardiac amyloidosis or junctophilin type 2 associated cardiomyopathy.

Differential diagnosis between hypertrophic cardiomyopathy (HCM) and cardiac amyloidosis (CA) is mandatory since the prognosis is very different, but not always possible as both diseases present with increased myocardial thickness and mass. Despite better knowledge of the pathophysiology of both HCM and CA, and new developments in diagnosis, many patients with cardiac involvement in systemic amyloidosis are still only diagnosed in an advanced stage. Improvements in non-invasive diagnostic methods such as ultrasound techniques and cardiac magnetic resonance imaging will eventually obviate the need for invasive studies in order to prove amyloid cardiomyopathy. Nevertheless, today, an endomyocardial biopsy still remains the golden standard. We present an 86-year-old man, diagnosed with hypertrophic cardiomyopathy, in whom echocardiography and cardiac magnetic resonance imaging strongly suggested amyloidosis to be the underlying cause. Interestingly, a new variant of the junctophilin 2 (JPH2) gene, related to hypertrophic cardiomyopathies, was found in our patient.

Dendritic Cell-Based and Other Vaccination Strategies for Pediatric Cancer.

Dendritic cell-based and other vaccination strategies that use the patient's own immune system for the treatment of cancer are gaining momentum. Most studies of therapeutic cancer vaccination have been performed in adults. However, since cancer is one of the leading causes of death among children past infancy in the Western world, the hope is that this form of active specific immunotherapy can play an important role in the pediatric population as well. Since children have more vigorous and adaptable immune systems than adults, therapeutic cancer vaccines are expected to have a better chance of creating protective immunity and preventing cancer recurrence in pediatric patients. Moreover, in contrast to conventional cancer treatments such as chemotherapy, therapeutic cancer vaccines are designed to specifically target tumor cells and not healthy cells or tissues. This reduces the likelihood of side effects, which is an important asset in this vulnerable patient population. In this review, we present an overview of the different therapeutic cancer vaccines that have been studied in the pediatric population, with a main focus on dendritic cell-based strategies. In addition, new approaches that are currently being investigated in clinical trials are discussed to provide guidance for further improvement and optimization of pediatric cancer vaccines.

Passenger Lymphocyte Syndrome (PLS): A Single-center Retrospective Analysis of Minor ABO-incompatible Liver Transplants.

Background and Aims: Due to the shortage of donor livers, minor ABO-incompatible liver transplantations are commonly performed. Together with the allograft, immunocompetent B-lymphocytes, called passenger lymphocytes, are transplanted. In case of minor ABO-incompatibility, these passenger lymphocytes produce antibodies directed towards the recipient's red blood cells, which causes immune-mediated hemolysis, also known as the passenger lymphocyte syndrome (PLS). Although this is a self-limiting disorder, serious complications can occur, including graft failure. Retrospectively, we evaluated the role of PLS in minor ABO-incompatible liver transplantations performed at our center. Methods: A retrospective analysis was conducted for all minor ABO-incompatible liver transplantations performed at the Antwerp University Hospital between 2003 and 2015. All patient files were inspected for clinical and laboratory findings. In cases of PLS diagnosis, the applied treatment was also studied. Results: In total, 10 patients underwent a minor ABO-incompatible liver transplantation and 4 showed signs of PLS. All 4 PLS patients were treated with different therapeutic strategy, corresponding to the severity of hemolysis. In all 4 cases, PLS resolved following treatment. Conclusion: When performing minor ABO-incompatible liver transplantations, knowledge of PLS is elemental. Next to a high index of clinical suspicion, we suggest routine screening for markers of hemolysis, with emphasis on haptoglobin level and direct antiglobulin test, weekly in the first 4 weeks post-transplantation as well as in case of a sudden hemoglobin drop within the first 3 months after transplantation. Peri- and postoperative transfusion support using donor-compatible blood has been suggested to prevent the occurrence or limit the extent of hemolysis.

The big imitator strikes again: a case report of neurosyphilis in a patient with newly diagnosed HIV.

BACKGROUND: Neurosyphilis is the result of an infection of the central nervous system caused by the spirochete Treponema pallidum. Its clinical presentation includes meningovascular syphilis, tabes dorsalis, and dementia paralytica, resulting in a wide range of symptoms such as psychosis, Parkinsonism, and depression. CASE REPORT: A 49-year-old male was admitted to a psychiatric hospital because of social withdrawal and self-neglect, indicative of a major depression. A routine HIV-test was positive and resulted in an admission to the Antwerp University Hospital. Clinical examination showed Argyll Robertson pupils, a wide-based gait, absence of vibration sense in the lower limbs, and a MMSE-score of 25/30. Blood analysis revealed a CD4+ count of 99 cells/µL and a HIV viral load of 2,13 × 105 copies/mL plasma. A serum TPHA (T. pallidum hemagglutination assay) titre of 1/20480 and RPR (rapid plasma reagin) titre of 1/128 were detected. TPHA and RPR titre in the cerebrospinal fluid were, respectively, 1/10240 and 1/4. A brain MRI showed diffuse cortical atrophy and lesions in the white matter compatible with HIV-encephalopathy. The diagnoses of advanced HIV-infection and late neurosyphilis were made. HAART (highly active antiretroviral therapy) and high-dose IV penicillin G were started. CONCLUSION: In all patients with new-onset dementia or untreatable psychosis, neurosyphilis should always be considered. Argyll Robertson pupils are regarded as pathognomonic of neurosyphilis. The management of neurosyphilis includes high-dose IV benzyl penicillin for 10 to 14 days. Close follow-up including a lumbar puncture after 6 months is warranted to ensure treatment recovery.