A Case of Epistaxis as the First Sign of Acute Idiopathic Thrombocytopenic Purpura

Idiopathic thrombocytopenic purpura (ITP) is an acquired thrombocytopenia caused by the action of autoantibodies against platelet antigens. It is traditionally defined by a platelet count of less than 10 × 104/μL. Most patients with ITP are asymptomatic; however, symptoms have been confirmed in some cases. Conversely, it is very rare to find epistaxis as the first sign of ITP. We report the case of an 84-year-old man who came to the ear, nose, and throat department with severe and repeated epistaxis. We decided to keep him hospitalized as it was very difficult to stop the nasal bleeding. A full blood count showed a platelet level of only 1000/μL. Hematologic results confirmed the diagnosis of ITP. The patient underwent treatment with intravenous gamma-globulin, platelet transfusions, and romiplostim with a favorable response.

COVID-19: Imbalanced Immune Responses and Potential Immunotherapies

The ongoing pandemic coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is rapidly spreading and has resulted in grievous morbidity and mortality worldwide. Despite the high infectiousness of SARS-CoV-2, the majority of infected individuals are asymptomatic or have mild symptoms and could eventually recover as a result of their balanced immune function. On the contrary, immuno-compromised patients are prone to progress into severe or critical types underpinned by the entanglement of an overexuberant proinflammatory response and injured immune function. Therefore, well-coordinated innate and adaptive immune systems are pivotal to viral eradication and tissue repair. An in-depth understanding of the immunological processes underlying COVID-19 could facilitate rapidly identifying and choosing optimal immunotherapy for patients with severe SARS-CoV-2 infection. In this review, based on current immunological evidence, we describe potential immune mechanisms and discuss promising immunotherapies for COVID-19, including IL-6R blockades, convalescent plasma, intravenous gamma globulin, thymosin alpha1, corticosteroids, and type-I interferon, and recent advances in the development of COVID-19 vaccines.

Treatment of Corticosteroid Non-responsive Relapse of Neuromyelitis Optica with Intravenous Gamma Globulin – a Case Report

We report a case of a patient with a relapse of neuromyelitis optica. The relapse was initially treated with intravenous corticosteroids. A therapy with intravenous gamma globulin was started as there was no symptomatic improvement. The patient responded well to the treatment with no significant side effects. Worldwide experience with gamma globulin treatment of neuromyelitis optica is limited and randomised control trials are lacking, therefore accumulation of data from case reports is of paramount importance.

1531. A Twenty 3-Year Retrospective Study of Secondary Hemophagocytic Syndrome in a Pediatric Third-Level Referral Center in Mexico City

Background Secondary hemophagocytic syndrome (SHS) may develop as a result of excessive immune activation, with proliferation of T cells and macrophages. It is often fatal and remains ill-recognized in children, leading to false or delayed diagnosis and suboptimal management. This condition has been described more often in association with virus, but it can be secondary to bacteria, fungi, and parasites. Methods Retrospective, descriptive, and observational study. We systematically reviewed the clinical files of patients diagnosed with SHS at the Instituto Nacional de Pediatría between January 1995 and December 2018; age, gender, clinical features, laboratory results, management, and final outcome were registered. Results 49 cases of SHS were diagnosed, 26 (53%) were male, mean age at diagnosis was 67.5 months (newborn–15.5 years), 47 (96%) presented with fever, 46 (94%) hepatomegaly, 37 (76%) splenomegaly, 28 (57%) lymphadenopathy, 18 (37%) jaundice, and 4 (8%) bleeding. The most common laboratory findings were anemia in 29 (59%) patients, thrombocytopenia in 39 (80%), hypertriglyceridemia in 39 (80%), with isolation of Epstein Barr Virus in 18 (35%), followed by Cytomegalovirus in 2 (4%), Escherichia coli in 2 (4%), Salmonella enterica in 2 (4%), and Leptospira, Brucella abortus, Shigella sonnei, Enterococcus faecium, Enterovirus and congenital Rubella in 1 case each (2%). Intravenous Gamma-globulin therapy was administered to 38 (78%) patients, antimicrobials to 26 (53%), systemic steroids to 46 (94%) and VP16 to 32 (65%). 22 (45%) had full recovery, 6 (12%) patients presented with a recurrent episode of SHS, and 21 (43%) died. Conclusion Due to the rarity of this syndrome, variable clinical presentation, and lack of specificity of the clinical and laboratory findings, the diagnosis of SHS is often delayed. EBV remains an important etiologic agent of this syndrome, identified in 35% of our cases. The mortality rate around the world is reported approximately around 45%, confirmed in our study in 43% of the cases, remarking the importance of prompt diagnosis and treatment to increase the survival in our population. Disclosures All authors: No reported disclosures.

Parvovirus B19-triggered acute hemolytic anemia and thrombocytopenia in a child with Evans syndrome

Background: Human parvovirus B19 (HPV-B19) is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals.Case report. We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thromocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg.Conclusion: Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present with reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.

Congenital nephrotic syndrome may respond to cyclosporine A: A case report and review of literature

Introduction. Congenital nephrotic syndrome (CNF) is manifested at birth or within the first three months of life. The Finnish-type of CNF is caused by the mutation of the NPHS1 gene, which encodes nephrin in the podocyte slit diaphragm. It is a very severe disease, for which immunosuppressive therapy is not advised. Here we describe a patient with CNF who responded to CsA by partial remission. Case outline. A girl aged 2.5 months presented with severe non-syndromic steroid-resistant nephrotic syndrome. She needed aggressive support including daily albumin infusions and diuretics. Substitution of vitamin D, thyroxin, and anticoagulants were regularly administered. She was also treated with angiotensin converting enzyme inhibitor, without clear benefits regarding proteinuria. In addition, she received intravenous gamma-globulin replacement therapy and antibiotics during frequent infections. While waiting for the results of genetic analyses and faced with many problems related to daily albumin infusions, infections, and thromboembolic complications, cyclosporine A (CsA) was introduced as an alternative to early nephrectomy and consequent renal failure. The patient responded by partial remission and CsA treatment continued at home without the albumin infusions. After almost five years since the beginning of the treatment, the patient?s renal function remains unreduced. Conclusion. Our case demonstrates that CsA can induce partial remission in patients with genetic forms of steroid-resistant nephrotic syndrome without influencing the glomerular filtration rate. However, its long-term effect and safety should carefully be monitored.