Intravenous Infusion Of Gamma Globulin Research Articles

A single-centre study on abnormal antinuclear antibodies in children caused by intravenous infusion of gamma globulin.

To clarify the impact of intravenous infusion of gamma globulin (IVIg) on antinuclear antibodies (ANAs) in children. A retrospective analysis was performed on the data of children with nonspecific autoantibody-related diseases whose antinuclear antibody (ANA) and autoantibody profiles were detected in our hospital from January to March 2022. A total of 108 patients with a clear history of IVIg infusion within 28 days composed the IVIg group, and 1201 patients without a history of IVIg infusion composed the non-IVIg group. All patients in the IVIg group had either positive ANAs or positive autoantibodies. Anti-SSA, anti-Ro52 and anti-AMA Mi2 were the top three autoantibodies in the IVIg group. The proportions of patients who were positive for either of these three autoantibodies in the IVIg group were significantly greater than those in the non-IVIg group (all P<0.5). Spearman correlation analysis revealed that the signal intensities of anti-SSA and anti-Ro52 were negatively correlated with the number of days of ANA detection after IVIg infusion (P<0.05). Multiple logistic analyses revealed that a greater total dosage of IVIg, greater IVIg per kilogram of body weight, and fewer ANA detection days after IVIg infusion were independent risk factors for positive anti-SSA and anti-Ro52 results. It is recommended that if rheumatic diseases are suspected, ANA detection should be carried out beforeIVIg infusion. But for patients who are positive for at least one of these three autoantibodies after IVIg infusion, doctors should first consider adoptive antibodies.

Common variable immune deficiency in adult patients: analysis of 13 cases and literature review

To investigate the clinical and immunological characteristics, treatment and prognosis of common variable immune deficiency (CVID) in adult patients. We retrospectively analyzed the clinical data of 13 adult patients hospitalized in our hospital for CVID diagnosed according to the criteria in International Consensus Document (2016), and analyzed their clinical manifestations, laboratory test results, imaging findings, pathological examinations and treatments. The mean age of onset was 24.46±16.82 years in these patients, who had a mean age of 32.54±14.86 years at diagnosis with a median diagnostic delay of 5 years (IQR: 2-15 years). The main manifestation of the patients was repeated infections, including repeated respiratory tract infection (10 cases; 76.9%) and repeated diarrhea (3 cases; 23.1%). Three (23.1%) of the patients had autoimmune disease and 10 (76.9%) had chronic pulmonary disease. IgG, IgA and IgM were decreased in all the patients. The proportion of CD4+T cells decreased in 10 patients (76.9%), CD8+T cells increased in 11 patients (84.6%), and CD4/ CD8 decreased in 10 patients (76.9%). Complement C3 decreased in 58.3% (7/12) and C4 decreased in 33.3% (4/12) of the patients. Twelve patients (92.3%) were treated with intravenous infusion of gamma globulin with symptomatic treatments. One patient died due to massive gastrointestinal hemorrhage, and the other patients showed improve ments after the treatments and were discharged. The clinical manifestations of CVID are diverse, and recurrent respiratory tract infection is the most common manifestation. Decreased IgG often accompanied by lowered IgA and IgM levels is a common finding in laboratory tests. The treatment of CVID currently relies on gamma globulin with symptomatic treatments for the complications.

Diagnosis and treatment progress of Guillain-Barré syndrome

Guillain-Barre syndrome(GBS)is a most common nervous system disease that may cause acute delayed paralysis. The findings of latest researches showed that GBS includes a group of autoimmunity related peripheral nervous disease presenting varied clinical phenotype and pathophysiological changes. The clinical top priority to choose the treatments were intravenous infusion of gamma globulin and plasma exchange. With the rapidly deepening of pathological mechanisms of GBS, new treatment approaches will appearto serve in clinical care.

Stiff limb syndrome with lower limb myoclonus

Rationale:stiff limb syndrome (SLS) is a variant of stiff-man syndrome, primarily affecting a specific limb. Its diagnosis has always been challenging due to the lack of a specific confirmation test. We present a rare case of a patient with lower limb myoclonus and rigidity.Patient concerns:A 53-year-old male presented with a sudden onset of progressive left lower extremity myoclonus and muscle rigidity for 3 days. He rapidly showed signs of right lower limb involvement with severe joint stiffness and inability to walk.Diagnosis:The symptoms nature, physical examination, careful elimination of differential diagnosis suggested a diagnosis of stiff limb syndrome.Interventions:Intravenous infusion of gamma globulin 0.4 mg/kg coupled with baclofen and clonazepam were given after admission. He also received an injection of botulinum toxin A to relieve his muscle stiffness.Outcomes:The patients’ condition improved after the initial treatment with complete disappearance of muscle twitching. Further improvements were seen later on after the local administration of botulinum toxin A.Lessons:Stiff limb syndrome shares the same complex symptoms with many other conditions. Its diagnosis relies heavily on clinical presentations and on ruling out other conditions. However, unusual symptoms such as myoclonus can occur in few cases and together with the rarity of the condition, the prevalence of misdiagnosis is high. Therefore, being aware and recognizing the signs and symptoms is crucial for proper management. Additionally, EMG is a very important test if the present condition is suspected. However, a negative EMG result or a negative anti-glutamic acid decarboxylase antibody test should not exclude SLS diagnosis.

Recurrent gastrointestinal bleeding with ANCA Associated Glomerulonephritis successfully treated by transarterial embolization

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of autoimmune diseases that normally affects multiple organs. Recurrent gastrointestinal (GI) bleeding, a critical complication of AAV, remains a challenge. Here, we report a case of AAV complicated by pulmonary hemorrhage, severe recurrent gastrointestinal bleeding, and rapid progressive renal insufficiency that was treated successfully with selective transarterial embolization, continuous veno-venous hemofiltration, plasma exchange, intravenous gamma globulin infusion, followed by steroids and cytotoxic drug therapy. We report this case considering that selective transarterial embolization may be a safe and effective alternative method in recurrent AAV associated GI bleeding caused by AAV refractory to medical therapy.

Ectopic Cervical Thymoma Accompanied by Good&amp;apos;s Syndrome

Ectopic cervical thymoma (ECT) is a rare tumor that is frequently misdiagnosed as a thyroid tumor or other malignancy. A 34-year-old male with a right palpable neck mass had been mistakenly diagnosed with T-cell lymphoblastic lymphoma even after an open biopsy. The atypical clinical course, including hypogammaglobulinemia, led us to the correct diagnosis; ECT accompanied by Good's syndrome (GS). After the intravenous infusion of gammaglobulin, tumor resection and a subsequent video-assisted thoracoscopic extended thymectomy were performed. The final diagnosis was type AB thymoma, Masaoka stage I. This report is, to the best of our knowledge, the first description of this extremely rare combination.

Rituximab Maintenance Therapy Until Progression After Rituximab and Chemotherapy Induction in Patients With Follicular Lymphoma

The PRIMA (Primary Rituximab and Maintenance) study established 2 years of maintenance rituximab (MR) as a standard of care for follicular lymphoma (FL) patients who achieve an objective response after induction chemotherapy. A 17% improvement in progression-free survival (PFS) compared with those who did not receive MR was observed. However, the decision to stop MR after 2 years was arbitrary, and the PRIMA study reports only short-term follow-up of 3 years. Longer series on FL outcomes describe ubiquitous relapse and death following recurrence. The optimal duration of MR is under investigation. Herein, we report our experience with prolonged MR in FL. In this retrospective analysis, the outcome of 25 consecutive, unselected, previously untreated patients with low-grade high tumor burden FL in need for treatment is described. All patients achieved a partial or complete response to induction immunochemotherapy and received ongoing MR therapy. With a median follow-up of 5.0 years and 5.2 years mean duration of MR, there are no relapses. Five deaths have occurred, unrelated to lymphoma or therapy. Prolonged MR treatment has been well-tolerated. Hypogammaglobulinemia is the only observed adverse event, with only one patient requiring monthly intravenous gamma globulin infusions due to recurrent pneumonia. There has been no discontinuation of MR or refusal to remain on MR. These provocative long-term results suggest that continuous MR beyond 2 years is safe and may be associated with prolonged PFS in patients with FL who achieve an objective response after immunochemotherapy.

Limbic encephalitis associated with anti‐GAD antibody and common variable immune deficiency

A variety of autoantibodies have been identified with complex neurological disorders including limbic encephalitis. The underlying trigger for the immune-mediated process and the role of autoantibodies in the pathogenesis of limbic encephalitis remain to be clarified. Here, we report a 16-year-old female who was diagnosed with acute-onset non-neoplastic limbic encephalitis. The initial treatment with pulse doses of i.v. methylprednisolone improved the neurological symptoms. During the next 12 months, progressive decline was reported in her academic functioning and seizure control. Additional diagnostic evaluation revealed no evidence of malignancy or central nervous system infection but circulating anti-GAD antibodies were present in the serum and cerebrospinal fluid. Intravenous gammaglobulin infusion was initiated and continued monthly. Intravenous and oral steroids were added to the intravenous immunoglobulin treatment because of the worsening course and seizures, despite treatment with antiepileptic medications. Screening for quantitative immunoglobulins demonstrated hypogammaglobulinaemia with low immunoglobulin M and G in addition to low immunoglobulin A levels. There was a lack of protective pneumococcal antibody titers before and after immunization. Therefore, common variable immunodeficiency was suspected despite there being no history of recurrent infections. To our knowledge, this is the first report describing a possible link between immune-mediated limbic encephalitis and immune deficiency.

Guillain–Barre in a patient with ankylosing spondylitis secondary to ulcerative colitis on infliximab therapy

We describe a 47-year-old woman with severe spondylarthropathy secondary to ulcerative colitis who developed a Guillain-Barre after the use of anti-TNF-alpha. She first developed ulcerative colitis in November 1997. In 2003, she developed uveitis and, in 2005, axial and enthesitis form of spondylarthropathy. In May 2007, her condition was exacerbated. Therapy with infliximab has been initiated. The patient received 5-mg/kg infusions of infliximab. She had significant improvement in her arthritis and was in remission for her ulcerative colitis. She was admitted to the hospital 2 weeks after her third dose of infliximab for having developed paraesthesia of her hands and lower limbs. Neurophysiology studies demonstrated an acquired segmental demyelinating polyneuropathy consistent with Guillain-Barre syndrome (GBS). Laboratory investigations were unremarkable. She was treated with intravenous corticosteroids with no improvement. After this, she received infusions of intravenous gammaglobulin (IVIg) with complete recovery of the muscle strength within a few weeks. A follow-up electromyographic study 3 months later showed normal finding. The development of GBS in our patient may be secondary to her anti-TNF-alpha treatment. At present, she remains off anti-TNF-alpha therapy.

Thrombotic Microangiopathy After ABO-Incompatible Living Donor Liver Transplantation: A Case Report

Thrombotic microangiopathy (TMA) has rarely been reported in the setting of liver transplantation. Herein we have reported a successful case of TMA after ABO-incompatible living donor liver transplantation (LDLT) treated with plasma exchange and high-dose intravenous gamma-globulin infusion. A 50-year-old woman was diagnosed with hepatitis C virus-related cirrhosis. We performed an ABO-incompatible LDLT (group B to O) with preoperative plasma exchange to reduce the anti-B hemagglutinin titers to 1:8. The immunosuppressants consisted of tacrolimus, mycophenolate mofetil, and steroid. On postoperative day (POD) 8, her anti-B hemagglutinin titer suddenly increased to 1:64. The serum lactate dehydrogenase (LDH) level was grossly elevated (1518 IU/L). On POD 13, we suspected infection of an intra-abdominal hematoma ( Serratia marcescens) which was drained surgically. On day 5 after the reoperation, thrombocytopenia developed with a platelet count of 3×10 4/mm 3. A peripheral blood film showed severe red blood cell (RBC) fragmentation. Thus, we made a clinical diagnosis of TMA and reduced the tacrolimus dose. We started intensive daily plasma exchange (4 L/d) with fresh frozen plasma and high-dose intravenous gamma-globulin infusions. One week thereafter, thrombocytopenia improved with reduced transfusion requirements. The peripheral blood film showed normal RBC morphology. The serum LDH returned to baseline levels. Four factors were considered to have caused TMA in this case: the prescription of tacrolimus, ABO-incompatible liver transplantation, bacterial infection, and surgical stress. These factors may have all contributed by causing significant endothelial injury and TMA.

Hemorheological changes after intravenous gammaglobulin administration in patients with neurological disorders

Hemorheological changes due to intravenous gammaglobulin (IVIg) administration have been considered to influence blood viscosity and this way the blood flow in microcirculation. The study was carried out in the group of 10 patients with various neurological disorders (seven with polyradiculoneuropathy, two suffering from myasthenia and one with multiple sclerosis). Patients were treated routinely with intravenous gammaglobulin infusion (Sandoglobulin, Sandoz, 24 g a day in the course of 5 days therapy). The following hemorheological factors were estimated: relative blood viscosity, plasma viscosity, red cell deformability and erythrocytes aggregation. For rheological examination the microviscosimeter Low Shear 40 (Contraves) was used. Each patient was examined two times: before treatment initiation and at the end of therapy after five days. At the comparison of first and last measurements a significant increase of plasma viscosity (p<0.04) was found, whereas erythrocyte deformability was significantly improved (p<0.05). The value of relative blood viscosity at shear rate of 0.1 s(-1) was significantly decreased (p<0.05) and statistically unchanged at other studied shear rates. The results suggest an existence of a protective feedback mechanism in the studied group of patients which has been exemplified by the red cell elasticity improvement.

Potential Application of Gene Therapy to X-Linked Agammaglobulinemia

X-linked agammaglobulinemia (XLA), or Bruton's disease, is the most common human primary humoral immunodeficiency. XLA is caused by mutations of the Bruton's tyrosine kinase (BTK), a key regulator of B-cell physiology. Since the mid 80's, substitutive therapy by intravenous gammaglobulin infusions has significantly improved XLA patient survival and quality of life. Nevertheless, some frequent affections persist despite treatment, and lead to handicapping and further to morbid clinical complications for XLA individuals. Development of gene therapy by transfer of the BTK gene into hematopoietic progenitors could represent an alternative strategy for the treatment of Bruton's disease, with the advantage of a definitive cure for XLA patients. Gene therapy of XLA could be considered as a paradigm for future expansion of gene therapy approaches for many other diseases, since future utilization may be strictly dependent on a marked improvement of risk-benefit ratio compared to pre-existing treatments.

Management of Bleeding Complications of Hematologic Malignancies

Persons with hematologic malignancies bleed for a variety of reasons, including alterations in platelet function and numbers, clotting factor deficiencies, circulating anticoagulants, and defects in vascular integrity. The management of bleeding begins with a full characterization of the hemostatic defect. Vitamin K deficiency always should be considered and excluded by clinical history and laboratory tests. Localized bleeding is treated by packing, topical hemostatic agents, dressings, vessel ligation, laser beam coagulation, or embolization. Platelet transfusions are administered for hemorrhage secondary to severe platelet dysfunction or thrombocytopenia, but usually are not indicated if there is no bleeding, even though platelets may be as low as 10,000/microL. Bleeding due to thrombocytopenia that is refractory to random-donor platelets may respond to cross-matched compatible platelets, or to recombinant factor VIIa (rFVIIa). Fresh frozen plasma is indicated infrequently; bleeding due to coagulopathies is better managed with cryoprecipitate if fibrinogen is low, or with clotting factor concentrates appropriate for the specific clotting factors found to be deficient. rFVIIa or activated prothrombin complex concentrate usually controls hemorrhage due to autoantibodies directed against factor VIII, and acquired von Willebrand's disease may be responsive to desmopressin or intravenous gamma globulin infusion. Antifibrinolytic agents often enhance other hemostatic therapies, but should be withheld if there is genitourinary bleeding or evidence of disseminated intravascular coagulation. Finally, plasmapheresis and immunoadsorption to remove paraproteins may be helpful when other measures fail to curb bleeding.

The use of vinca alkaloids in preparation for splenectomy of corticosteroid refractory chronic immune thrombocytopenic purpura patients

Administration of vinca alkaloids (VA) to chronic corticosteroid refractory immune thrombocytopenia (ITP) patients results in a temporary increase of platelet count. The aim of the study was to evaluate the efficacy of vinca alkaloids in preparing adult corticosteroid refractory chronic ITP patients for splenectomy as well as to compare the costs of this method with costs of applying intravenous immunoglobulins. The study included 12 chronic ITP patients refractory to corticosteroids applied for 3-144 months. The patients were prepared for splenectomy with average 3.0 (from 1 to 4) 2-h intravenous infusions of vinca alkaloids at 7 day intervals. In eight patients, vincristin was used in a total dose of 6 mg (2 mg per infusion), in two patients, vinblastin was used in total dose of 30 mg (10 mg per infusion), and in two patients, vincristin and vinblastin infusions were administered alternatively. In nine of the 12 treated patients (75%) the platelet count increased to > or = 80 x 10(9)/l, which allowed safe splenectomy. Three patients unreactive to VA treatment were prepared for splenectomy with intravenous gammaglobulin infusions. Splenectomy was performed in 12 patients, in eight with laparoscopic method, in four with classic method. No complications during surgical intervention were observed. In none of the VA treated patients was myelosupression or liver or/and kidney dysfunction observed. Splenectomy resulted in normalization of platelet count in all patients after operation and in six of nine patients followed up for 10 months (on the average). Matching of VA costs with treatment efficacy and comparison with similar costs for intravenous immunoglobulin treatment revealed many fold lower costs of the former method.

Kongenitale intestinale Lymphangiektasie: Eine seltene Differerenzialdiagnose hypoproteinämischer Ödeme im Säuglingsalter

Congenital intestinal lymphangiectasia is a rare disease in childhood, which may already cause protein-losing enteropathy in newborns. This is a case report of an infant with generalized edema and protein-losing enteropathy, in whom intestinal lymphangiectasia was diagnosed at the age of two months. Following repetitive intravenous albumin und gamma globulin infusions, the elimination of long-chain fats from the diet and the substitution with medium-chain triglycerides (MCT) led to an improvement of the protein-losing enteropathy. In newborns with low level of serum protein and edema protein-losing enteropathy caused by congenital lymphangiectasia might be considered as a differential diagnosis.

Coronary risk factors in Kawasaki disease treated with additional gammaglobulin

Aims: To assess the hypothesis that an additional intravenous gammaglobulin (IVGG) infusion, if administered early, may prevent coronary artery lesions (CAL) in patients with Kawasaki disease (KD) who do not...

Pregnancy with systemic lupus erythematosus and four IVF failures treated with intravenous gamma globulin infusions

Pregnancy with systemic lupus erythematosus and four IVF failures treated with intravenous gamma globulin infusions

Pregnancy with systemic lupus erythematosus and four IVF failures treated with intravenous gamma globulin infusions

Pregnancy with systemic lupus erythematosus and four IVF failures treated with intravenous gamma globulin infusions

Guillain-Barré syndrome and hyponatraemia.

Hyponatraemia is a recognized association of the Guillain-Barré syndrome (GBS) and is also known to occur after the administration of intravenous infusion of gamma globulin (IVIG), a treatment often used in management of GBS. To document serum sodium concentration in GBS patients before, during and after management procedures (including IVIG) positive pressure ventilation (PPV) and the use of medications capable of causing a low serum sodium. To consider whether pseudohyponatraemia might be a contributory factor. To assess whether hyponatraemia is a predictor of poor outcome. Clinical record audit of 84 patients with GBS admitted to Christchurch Hospital, New Zealand, over a 10-year period. Serum sodium concentration was significantly low (<133 mmol/L) in 26 of 84 (31%) patients. In 12 of these cases the hyponatraemia developed during or after IVIG, suggesting that pseudo-hyponatraemia was a contributing factor. Six (7%) significantly hyponatraemic patients died, but no eunatraemic or slightly hyponatraemic patient died (P = 0.001). Six of 38 patients aged > or =50 years died, whereas none of the 46 patients aged <50 years died (P = 0.007). Five of 19 ventilated patients died but only one of 65 non-ventilated died (P = 0.002). Significant hyponatraemia occurred in 26 (31%) of the patients, but in 12 of these it appeared likely that pseudohyponatraemia caused by IVIG was a contributing factor. Hyponatraemic patients have a poorer prognosis than eunatraemic patients, however it is difficult to separate this factor from other poor prognostic factors (older age and PPV).

Fc receptor blockade and immune thrombocytopenic purpura

Inhibition of antibody-coated platelet destruction in patients with immune thrombocytopenic purpura (ITP) is a well-known mechanism of treatment effect. A number of interventions that would ameliorate the thrombocytopenic effect of ITP patient plasma when infused into normal recipients were demonstrated in 1965. Subsequently, the antibody-coated chromium-labeled red blood cell clearance study was developed to allow direct in vivo assessment of Fc receptor (FcR) blockade. This was first demonstrated for corticosteroids but more extensive investigation began with the study of intravenous infusions of gammaglobulin (IVIg). The unequivocal demonstration of FcR blockade following IVIg initiated novel approaches. One involved the infusion of a monoclonal anti-FcRIII ligand-blocking antibody into patients with refractory ITP. The efficacy of this treatment demonstrated that FcR blockade was not an epiphenomenon but rather an important mechanism of the increase in the platelet count in patients with ITP. Confirmation of its importance was obtained from the infusion of intravenous (IV) anti-D and the use of the isolated Fc piece of IgG. Recent studies have begun to explore the possibility of a monoclonal anti-FcRI and monoclonal anti-Ds. In summary, FcR blockade is an important mechanism of treatment effect in patients with ITP. Cytokine release as a consequence of this interaction and other immunomodulatory effects have only begun to be studied.