High-dose Intravenous Gammaglobulin Therapy Research Articles

Six patients with Kawasaki disease showing retropharyngeal low‐density areas on computed tomography

Six patients with Kawasaki disease showing retropharyngeal low‐density areas on computed tomography

Hemolytic disease of the newborn due to anti‐Dib: a case study and review of the literature

The severity of hemolytic disease of the newborn (HDN) due to Diego(b) (Di(b)) mismatch ranges from no symptoms to severe jaundice that requires exchange transfusion (ET). The clinical significance of anti-Di(b) is incompletely recognized. A male newborn, referred with jaundice, was revealed to have HDN due to Di(b) mismatch and was treated successfully with phototherapy and high-dose intravenous gamma globulin (IVGG). The literature of HDN caused by Di(b) mismatch was reviewed. The cases were classified into three groups according to their severity: the mildest needed no therapy (NO), the moderate group received phototherapy alone (PHOTO), and the most severe was treated with ET and/or high-dose IVGG therapy plus phototherapy (ET/IVGG). Among 27 cases of HDN due to Di(b) reported to date, 10, 6, and 11 cases required NO, PHOTO, and ET/IVGG, respectively. A significant correlation (p < 0.01) was found between the maternal anti-Di(b) titer and the severity of the disease when the ET/IVGG group was compared with the NO group. All mothers of the group that needed ET/IVGG had an anti-Di(b) titer of 64 or greater. A maternal high titer (> or =64) of anti-Di(b) is associated with a higher risk of severe hyperbilirubinemia for mismatched newborns.

Effect of Plasma Exchange in Kawasaki Disease Refractory to High Dose Intravenous Gammaglobulin Therapy

Background: In Kawasaki disease (KD), administration of intravenous gammma-globulin (IVGG) reduced frequency of coronary artery lesion (CAL) from 25% to 8%. However, approximately 500 children still develop CAL every year in Japan. We evaluated the efficacy and safety of plasma exchange (PE) for children with KD intractable to IVGG therapy. Patients and Methods: Among 255 children with KD treated with initial IVGG, 119 children were identified at high risk for CAL according to the increases in fractional changes (FC) of white blood cell count, neutrophils, and CRP between baseline and 1 to 2 days after IVGG treatment. Seventy-five were administered additional IVGG, and finally 50 of them were underwent the PE therapy in 3 consecutive days. All the children were serially monitored for the development of CAL by echocardiography during the course of the disease until days 28-30. The incidence of CAL was compared among the children treated and those untreated with PE. Results: The demographic differences between the treated and untreated children were not significant on admission. The PE therapy was safely performed without any adverse reactions, and has completed in each child. Out of 50 children treated PE, only 10 (20.0%) had CAL, including temporary dilatation in 7, persistent CAL in 1, and giant aneurysms in 2 children. In contrast, 24 children (40.7%) among 69 without PE were affected with CAL, including giant aneurysm in 5 (P < 0.0004). Conclusions: We suggested that PE therapy performed within 10 days after the onset of the disease should be effective and safe for the children with KD intractable to the IVGG therapy. The PE therapy may be applied as soon as possible when the fractional increases in inflammatory markers after the initial IVGG are determined.

Role of Plasmapheresis in Thrombocytopenic Purpura Associated With Waldenström's Macroglobulinemia

A 67-year-old man with Waldenström's macroglobulinemia had a relapse of chronic idiopathic thrombocytopenic purpura (ITP), which had been refractory to corticosteroids, splenectomy, vinca alkaloids, and high-dose intravenous gamma-globulin therapy. A biclonal gammopathy (IgM kappa and IgG lambda) was detected in his serum and was likely responsible for his refractory thrombocytopenia. He was treated with chlorambucil and prednisone. Plasmapheresis was effective in temporarily maintaining platelet counts and in decreasing morbidity until immunosuppression was completely effective against the production of the monoclonal protein. The previously reported experiences with use of plasmapheresis in patients with chronic ITP are discussed. Plasmapheresis may be of value in the treatment of selected patients with severe ITP and monoclonal gammopathy.

Experience of high-dose intravenous gammaglobulin therapy for neonatal immune hemolytic jaundice due to blood type incompatibility

Experience of high-dose intravenous gammaglobulin therapy for neonatal immune hemolytic jaundice due to blood type incompatibility

High-Dose Intravenous Gammaglobulin Therapy for Children with Newly Diagnosed Idiopathic Thrombocytopenic Purpura

High-Dose Intravenous Gammaglobulin Therapy for Children with Newly Diagnosed Idiopathic Thrombocytopenic Purpura

Lupus nephritis in children ? a review of 167 patients

Relatively few studies have been made of children with lupus nephritis. The prognosis of children with lupus nephritis is ominous for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. Up to now few studies have been made on this subject. To evaluate the clinical course, histopathology, and prognosis of lupus nephritis in children, to identify the risk factors for renal failure and mortality, and to share our experience in treating lupus nephritis in children. Retrospectively, 167 children under 18 years of age with lupus nephritis at Veterans General Hospital-Taipei, Taiwan from 1979 to 1991 were studied. All patients received renal biopsy and follow-up biopsies were performed in 36 children. The clinical and serologic parameters at the time of renal biopsy were recorded. There were 55 (33%) patients with class II, 30 (18%) with class III, 69 (41.3%) with class IV, and 13 (7.8%) with class V nephritis based on initial biopsy. The mean follow-up time was 59 months. Follow-up biopsies were histologically stationary in 29 patients, progressive in five, and regressive in two. The results revealed that those with persistent hypertension, anemia, increased serum creatinine concentration, and decreased creatinine clearance rate at initial biopsy were more prone to develop renal failure. Low titer of CH50 hemolytic assay appeared to be a poor prognostic indicator. The overall renal and patient 5-year survival rates were 93.1% (135/145) and 91.08% (143/157), respectively. They were 87.7% (50/57) and 82% (55/67), respectively, of patients with class IV proliferative glomerulonephritis. The prognosis of children with class IV nephritis in this study was better than that reported previously. All children surviving without renal failure were maintaining their normal lives with little organ dysfunction. The improved results may be due to earlier renal biopsy for precise histopathologic definition, better supportive care, and selective use of aggressive therapy, including methylprednisolone pulse therapy, intravenous cyclophosphamide, intravenous prostaglandin E1 therapy, high-dose intravenous gammaglobulin therapy, and cyclosporin A for those with high risk factors.

Southwestern Internal Medicine Conference: High-Dose Intravenous Gamma Globulin Therapy

Southwestern Internal Medicine Conference: High-Dose Intravenous Gamma Globulin Therapy

High-dose intravenous gammaglobulin therapy for acquired von Willebrand disease

Patients with acquired von Willebrand disease may present with severe bleeding, which is usually difficult to manage. Adequate haemostasis in acquired von Willebrand disease may be achieved with the infusion of factor VIII/von Willebrand factor concentrates or with the administration of desmopressin. We report a case of acquired von Willebrand disease with severe postoperative bleeding, responding poorly to classical von Willebrand factor replacement therapy but successfully treated with high-dose intravenous gammaglobulins. This new treatment mode of acquired von Willebrand disease is discussed in the light of a critical analysis of the literature.

Lupus Nephritis in Children—A Review of 167 Patients

Background. Relatively few studies have been made of children with lupus nephritis. The prognosis of children with lupus nephritis is ominous for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. Up to now few studies have been made on this subject. Objectives. To evaluate the clinical course, histopathology, and prognosis of lupus nephritis in children, to identify the risk factors for renal failure and mortality, and to share our experience in treating lupus nephritis in children. Methods. Retrospectively, 167 children under 18 years of age with lupus nephritis at Veterans General Hospital-Taipei, Taiwan from 1979 to 1991 were studied. All patients received renal biopsy and follow-up biopsies were performed in 36 children. The clinical and serologic parameters at the time of renal biopsy were recorded. Results. There were 55 (33%) patients with class II, 30 (18%) with class III, 69 (41.3%) with class IV, and 13 (7.8%) with class V nephritis based on initial biopsy. The mean follow-up time was 59 months. Follow-up biopsies were histologically stationary in 29 patients, progressive in five, and regressive in two. The results revealed that those with persistent hypertension, anemia, increased serum creatinine concentration, and decreased creatinine clearance rate at initial biopsy were more prone to develop renal failure. Low titer of CH50 hemolytic assay appeared to be a poor prognostic indicator. The overall renal and patient 5-year survival rates were 93.1% (135/145) and 91.08% (143/157), respectively. They were 87.7% (50/57) and 82% (55/67), respectively, of patients with class IV proliferative glomerulonephritis. Conclusions. The prognosis of children with class IV nephritis in this study was better than that reported previously. All children surviving without renal failure were maintaining their normal lives with little organ dysfunction. The improved results may be due to earlier renal biopsy for precise histopathologic definition, better supportive care, and selective use of aggressive therapy, including methylprednisolone pulse therapy, intravenous cyclophosphamide, intravenous prostaglandin E1 therapy, high-dose intravenous gammaglobulin therapy, and cyclosporin A for those with high risk factors.

Effectiveness of High-Dose Intravenous Gamma Globulin Therapy in Acquired von Willebrand's Disease

An 82-year-old patient with acquired von Willebrand’s disease in association with a non-Hodgkin lymphoma and a benign IgG(λ) monoclonal paraproteinaemia is described with severe recurring nasopharyngeal bleedings, who responded poorly to desmopressin (DDAVP) and factor VIII/von Willebrand factor concentrates but was successfully treated with high-dose intravenous γ-globulin therapy.

Infection-induced Transient Increase in the Platelet Count, and a Transient Remission of Thrombocytopenia with High-dose Intravenous Gammaglobulin Therapy during Intercurrent Pneumonia in Chronic Idiopathic Thrombocytopenic Purpura

Infection-induced Transient Increase in the Platelet Count, and a Transient Remission of Thrombocytopenia with High-dose Intravenous Gammaglobulin Therapy during Intercurrent Pneumonia in Chronic Idiopathic Thrombocytopenic Purpura

Immunotherapy and other novel therapies

Although no true breakthroughs occurred, publications during the 12-month period of this review added substantial definition to certain novel immunotherapies potentially applicable to the treatment of rheumatoid arthritis. Overall, this period witnessed maturation in the field of biologic interventions. Clinical trials provided further data needed to assess the efficacy of high-dose intravenous gamma-globulin therapy in patients with systemic juvenile rheumatoid arthritis, and extended uncontrolled experience with interferon-gamma in adult rheumatoid arthritis was obtained. An intriguing immunostimulant and antiviral drug, isoprinosine (inosine pranobex), failed in a scientifically rigorous trial in rheumatoid arthritis. Provocative insights into totally new approaches surfaced in additional reports from a variety of immunologic areas. Although seemingly distal to rheumatoid arthritis, these papers are cited because their further development or adaptations could reach a stage where clinical trials in rheumatoid arthritis are warranted.

Images Before and After Splenectomy Showing the Distribution of Homologous ln-111 Platelets in a Case of Immune Thrombocytopenic Purpura

ln-111 donor platelet studies were performed before and after splenectomy on a 63-year-old man with immune thrombocytopenic purpura (ITP). Donor platelets supplied by the Australian Blood Transfusion Service were used. The patient's platelet count ranged from 1 to 20 x 109/1–1. Bone marrow examination showed plentiful megakaryocytes. In this case, the ITP did not respond to any remedial measures, including corticosteroids, highdose intravenous gamma globulin therapy, and treatment with danazol, cyclophosphamide, azathioprine, and vincristine-labeled platelets. The patient eventually suffered a left parietal haemorrhage and died. Death was attributed to ischemic heart disease.

Autoreactive platelet antibody in post transfusion purpura

Post transfusion purpura in a DR3, DRw52 positive, PlA1 negative woman rapidly responded to high-dose intravenous gammaglobulin therapy. Using the platelet immunofluorescence test (PIFT) high titre IgG and IgM alloantibodies were detected in the patient's acute serum. These alloantibodies had PlA1 specificity and the patient's serum produced a broad, 100 kDa band on Western Blot with PlA1 positive platelets. Acute phase serum also contained an IgG antibody which reacted in the PIFT with the patient's own platelets, and PlA1 negative platelets. When tested by Western Blot with PlA1 negative and Glanzmann's Thrombasthenia platelets, the acute serum produced bands at 180 and 200 kDa. The autoreactive antibody was not detected once the platelet count returned to normal. These findings provide evidence for the hypothesis that the mechanism of autologous platelet destruction in PTP is autoimmune.

Effective treatment with double filtration plasmapheresis (DEPP) and high dose intravenous gammaglobulin therapy in a pregnant systemic lupus erythematosus(SLE) patient.

症例は24才,女性. SLEの診断にて,昭和61年よりステロイド薬投与を行っていた.同年12月,妊娠2カ月を確認.本人の希望強く, SLEの経過も良好であったため,妊娠を継続した.妊娠36週, SLE増悪を認め入院.直ちにdouble filtration plasmapheresis (DFPP)および大量γ-globulin療法を行った.治療により症状改善を認め,妊娠38週めに健康な女児を無事娩出した.上記治療法は妊娠中のSLE悪に対し有効と考えられた.

A Patient with Graves' Disease who Developed Hypothyroidism Associated with Thyroid Stimulation Blocking Immunoglobulin during Anti-thyroid Drug Therapy.

A girl, 12 years of age, developed Graves' disease compounded with rheumatic fever and idiopathic thrombocytopenic purpura. Thrombocytopenia improved under short-term treatment with steroids and her mitral valvular insufficiency, due to the rheumatic fever, disappeared 4 years later. Initially, she had been treated with propylthiouracil (PTU) for 28 months. She suffered a relapse 9 months after stopping PTU and so she was given further PTU therapy. However, hypothyroidism developed 11 months after the initiation of therapy and continued, though further PTU treatment was discontinued. She now receives 1-thyroxine and maintains a euthyroid state. At the onset of the patient's hyperthyroidism, the TSH-binding inhibitor immunoglobulin (TBII) and the thyroid stimulating antibodies (TSAb) were found to be positive. During the remission period, only the thyroid stimulation blocking immunoglobulin (TSBI) was weakly positive. At relapse, only TBII was mildly positive. When hypothyroidism developed, both TBII and TSBI were positive, and TSAb was negative in all testings of her diluted IgGs. The patient's TBII and thyroid dysfunction were unaffected by high-dose intravenous gammaglobulin therapy or by treatment with prednisolone 0.5 mg/kg/day for 2 weeks. In conclusion, the emergence of TSBI during or after anti-thyroid drug therapy might possibly lead to hypothyroidism in patients with Graves' disease.

Intravenous Treatment with Gammaglobulin in Adults with Immune Thrombocytopenic Purpura: Review of the Literature1

The results of high-dose intravenous gammaglobulin therapy (IVGG) of adults with immune thrombocytopenic purpura (ITP) were reviewed in 28 published reports which included 282 patients. Overall, 64% of the patients responded to IVGG with a peak platelet count greater than 100,000/mm3; 83% had peak platelet counts greater than 50,000/mm3. Unmodified immunoglobulin was superior to modified immunoglobulin; 70% of the patients treated with the former having platelet increases to greater than 100,000/mm3 compared to only 49% of those treated with the latter. More patients were refractory among those who had had ITP for at least 3 years. A higher peak platelet count immediately after IVGG administration was correlated with a longer duration of the platelet response. Patients above the age of 60 tended to have a weaker response to IVGG than did younger patients, and females tended to respond better than males. A pre-IVGG platelet count of less than 10,000/mm3 was not associated with a poor response to IVGG. While there is still no way to reliably predict response prior to therapy in an individual patient, the above information may help in the decision of whether or not treatment with IVGG is likely to be successful. It also suggests that inhibition of antiplatelet antibody production is an important mechanism of IVGG effect at least in some patients.

Effective high-dose intravenous gammaglobulin therapy for passive immune thrombocytopenia in the neonate

The second child of a mother with idiopathic thrombocytopenic purpura was given 400 mg/kg per day polyethylenglycol-treated gammaglobulins during the first 5 days of life. Thrombocytes increased from 15 X 10(9)/l to 200 X 10(9)/l within 10 days and remained at this level afterwards.

Echovirus polymyositis in patients with hypogammaglobulinemia: Failure of high-dose intravenous gammaglobulin therapy and review of the literature

A 29-year-old man with X-linked hypogammaglobulinemia was treated with prednisone and methotrexate for polymyositis. Subsequently, it was established that disseminated echovirus 11 infection was causing the polymyositis. Treatment with large doses of intravenous gamma-globulin did not result in improvement. Viral cultures of blood, urine, and cerebrospinal fluid gave positive results throughout treatment and at postmortem examination. Multiple cultures of other tissues, including muscle, also gave positive results at postmortem examination. Severity of infection and treatment with prednisone and methotrexate prior to referral, diagnosis, and gammaglobulin treatment may explain the lack of response. A review of 23 cases of echovirus infection in patients with hypogammaglobulinemia revealed that the infection in these patients may cause meningoencephalitis or a polymyositis-like syndrome or both. Treatment with immunosuppressive agents, the standard therapy for polymyositis, is contraindicated, and intravenous or intraventricular gammaglobulin or both may be helpful.