Inflammatory myopathy with anti-Golgi antibody and anti-SS-A/Ro antibody

We report a 74-year-old woman with anit-Golgi antibody and anti-SS-A/Ro antibody who contracted inflammatory myopahy presenting 'ALS-like' symptoms. We identified anti-Golgi antibody directly using confocal microscopy and successfully treated her with steroid. This report suggests that there is a new categorized subgroup of inflammatory myopathy with these specific antibodies and the pattern of autoantibody in these patients indicates the specific clinical course and treatment strategy.     

Autoimmune Haemolytic Anaemia in Infancy with Anti-Kpb Specificity

Autoimmune haemolytic anaemia (AIHA) in infancy is rare. We report a case of AIHA in a male infant whose serum contained an antibody with apparent anti-Kp^b specificity. Autoantibody with anti-Kp^b specificity has been described in adults; to our knowledge, this is the first case of this kind described in infancy with AIHA. Clinical course and response to red cell transfusion are described.     

Glycoprotein IIb/IIIa complex is the target in mirtazapine-induced immune thrombocytopenia

Mirtazapine (MW 265.36), a tetracyclic antidepressant of the piperazine-azapine group which augments central noradrenergic and serotonergic activity, is currently used as an oral antidepressant. We report a case of severe thrombocytopenia in a 66-year-old patient occurring after mirtazapine administration, suggesting an immune mechanism. This report documents the first case of mirtazapine-induced immune thrombocytopenia. The patient's serum was screened for drug-induced anti-platelet antibody with the chromium(51) (Cr(51)) platelet lysis technique. The drug-dependent antibody was characterized using flow cytometry, the monoclonal antibody immobilization of platelet antigens assay (MAIPA assay), and immunoprecipitation. By the Cr(51) platelet lysis technique, we obtained an equivocal result for the detection of mirtazapine-induced antibody. However, the patient's serum tested positive for mirtazapine-induced antibody by flow cytometry. The results showed that the binding ratio of 5.7 (mean fluorescence intensity) in the presence of the patient's serum and mirtazapine in a final concentration of 1.0 mmol/L was strongly positive. The antibody was found to bind the glycoprotein (GP) IIb/IIIa complex by MAIPA assay by using five different monoclonal antibodies against GP complexes Ib/IX, GPIIb/IIIa, or GPIa/IIa. Immunoprecipitation studies showed that the GPIIb/IIIa complex was precipitated by antibody in the presence, but not in the absence, of mirtazapine. These findings provide evidence that immune thrombocytopenia can be caused by sensitivity to the antidepressant mirtazapine. This is the first well-documented case of mirtazapine-induced immune thrombocytopenia.     

Lambda Cold Agglutinin with Anti-A1 Specificity in a Patient with Reticulosarcoma

Abstract. Erythrocyte auto-antibodies may be associated with lymphomas. We report a case of reticulosarcoma in a patient of A₁ blood group phenotype whose direct Coombs test was positive with anti-IgM and anti-complement antiglobulins. In serum, a cold antibody of IgM type was detected, which exhibited a monoclonal pattern with Λ-light chains. Moreover, it was shown to display an unusual auto-anti-A₁ specificity.     

Immune thrombocytopenia more than a year after allogeneic marrow transplantation due to antibodies against donor platelets with anti-PIA1 specificity: evidence for a host-derived immune reaction

We report on a male patient transplanted from his HLA-matched sister for Ph1-chromosome positive chronic myelogenous leukaemia who developed immune thrombocytopenia more than 1 year after transplantation. The platelet antibody reacted with the platelet specific antigen PlA1 on donor platelets, and also on recipient platelets after engraftment. A presumed host-versus-donor induced thrombocytopenia was supported by Southern blot analysis using a Y-chromosome specific probe demonstrating residual host-origin cells in the patient's excised spleen.     

Takayasu Arteritis With Antiphosphatidylserine/Prothrombin Antibody-Positive Antiphospholipid Syndrome: Case Report and Literature Review

A relationship between Takayasu arteritis (TA) and positive antiphospholipid antibody states has been pointed out, but patients with TA complicated with antiphospholipid antibody syndrome (APS) are rare. Here we report the case of a 17-year-old Japanese man diagnosed with TA based on pulselessness of the left brachial artery, discrepancy of blood pressure between the upper extremities, and arterial wall thickening and narrowing of artery in contrast computed tomography. He was also diagnosed with provisional APS based on a pulmonary infarction without narrowing of the pulmonary artery and positive antiphosphatidylserine/prothrombin antibody. The patient also had concurrent Crohn''s disease (CD) based on histopathological findings, which may have been associated with TA. We started high-dose corticosteroid therapy and anticoagulation therapy, and his symptoms including fever, dizziness, chest pain, and lower-right uncomfortable abdomen improved.We reviewed 9 cases of TA with APS including our patient by conducting a PubMed search. Based on past reports, we considered the relationship among TA, APS, and CD.Clinicians should bear in mind that many etiologies can exist in 1 patient, and differential diagnoses are essential.     

Phase separation in solutions of monoclonal antibodies and the effect of human serum albumin

We report the observation of liquid-liquid phase separation in a solution of human monoclonal antibody, IgG2, and the effects of human serum albumin, a major blood protein, on this phase separation. We find a significant reduction of phase separation temperature in the presence of albumin, and a preferential partitioning of the albumin into the antibody-rich phase. We provide a general thermodynamic analysis of the antibody-albumin mixture phase diagram and relate its features to the magnitude of the effective interprotein interactions. Our analysis suggests that additives (HSA in this report), which have moderate attraction with antibody molecules, may be used to forestall undesirable protein condensation in antibody solutions. Our findings are relevant to understanding the stability of pharmaceutical solutions of antibodies and the mechanisms of cryoglobulinemia.     

Molecular remission of Philadelphia/bcr-abl-positive acute myeloid leukaemia after treatment with anti-CD33 calicheamicin conjugate (gemtuzumab ozogamicin, CMA-676)

The anti-CD33 monoclonal antibody linked to NAc-gamma calicheamicin gemtuzumab ozogamicin (CMA-676) was used to treat a patient with Philadelphia/bcr-abl-positive acute myeloid leukaemia. We report a morphological and cytogenetic complete remission after treatment with two doses of gemtuzumab ozogamicin as a single agent. Using real-time polymerase chain reaction (PCR), gemtuzumab ozogamicin treatment resulted in a 3-log tumour mass reduction in bone marrow.     

A case of primary antiphospholipid antibody syndrome presenting with dysfunctional uterine bleeding and cerebral infarction

We report a 34-year-old woman who developed primary antiphospholipid antibody syndrome (APS) presenting with dysfunctional uterine bleeding and cerebral infarction. Antiphospholipid antibody syndrome presenting with bleeding manifestations is rare. We should recognize that APS may be associated with not only thrombosis but also bleeding.     

Antiretroviral therapy for treatment-naïve chronic HIV-1 infection with an axonal variant of Guillain-Barré syndrome positive for anti-ganglioside antibody: a case report

Guillain-Barré syndrome sometimes manifests as immune reconstitution inflammatory syndrome. We report a treatment-na?ve male with chronic HIV-1 infection who presented with an axonal variant of Guillain-Barré syndrome. Antiretroviral therapy commenced one month later and no rapid improvement or deterioration of tetraparesis was noted. This is the first report that describes the detection and serial measurements of anti-ganglioside antibody in a patient with HIV infection. This case suggests a limited role for T-cell immunity in the production of anti-ganglioside antibody and the pathogenesis of axonal variants, since the antiretroviral therapy-induced improvement in T-cell immunity neither re-elevated anti-ganglioside antibody titer nor worsened tetraparesis.     

K12 Is Located on the Kell Blood Group Protein in Proximity to K/k and Jsa/Jsb

K12 is a high-prevalence antigen, with no known antithetical partner, that is associated with the Kell blood group system. We report studies on the 5th propositus (MS) with the K:-12 phenotype and the second case to show that the K12 is inherited. The anti-K12 in his serum did not destroy antigen-positive incompatible red cells transfused on at least 3 occasions over 6 years. Red cell membranes from MS possessed the Kell protein that was indistinguishable from control membranes. K12 antigen was shown by immunoprecipitation to be on a protein with an apparent molecular mass of 93,000 and by monoclonal antibody immobilization of erythrocyte antigens (MAIEA) assay to be on the Kell protein in proximity to K/k and Js^a/Js^bantigens. These data remove the K:-12 phenotype from its current Kell-related (or para Kell) status and elevates the K12 antigen to a bona fide member of the Kell blood group system.     

Successful treatment of life-threatening Evans syndrome due to antiphospholipid antibody syndrome by rituximab-based regimen: a case with long-term follow-up

An association of antiphospholipid antibody syndrome with antibodies directed against either phospholipids or plasma proteins strongly suggest that B-cell dysfunction may be involved in its pathogenesis. Antiphospholipid antibody syndrome with autoimmune cytopenias shows a poor response rate to conventional treatment with anticoagulants, glucocorticosteroids, immunosuppressive agents, intravenous immunoglobulin or plasmapheresis. We report a case of life-threatening antiphospholipid antibody syndrome with Evans syndrome receiving successful multimodal treatment including anti-CD20 monoclonal antibody rituximab with long-term follow-up.     

First-time use of bevacizumab for aggressive, metastatic hepatocellular carcinoma in an HIV/hepatitis B virus coinfected patient: a case report

We present a case of an HIV-1 infected patient with history of chronic hepatitis B and chronic alcohol use without cirrhosis, who presented with aggressive hepatocellular carcinoma with multiple metastases. Systemic chemotherapy combined with use of bevacizumab (anti-vascular endothelium growth factor monoclonal antibody) was without effect and the patient succumbed to his disease within few weeks. To our knowledge, this is the first report in the English literature of bevacizumab use for metastatic hepatocellular carcinoma in HIV-infected patients.     

Coumarin-induced skin necrosis associated with acquired protein s deficiency and antiphospholipid antibody syndrome

We report a case of skin necrosis occurring during coumarin treatment in a patient with acquired protein S deficiency and the primary antiphospholipid antibody syndrome.     

A case of primary antiphospholipid antibody syndrome presenting with dysfunctional uterine bleeding and cerebral infarction

Abstract

We report a 34-year-old woman who developed primary antiphospholipid antibody syndrome (APS) presenting with dysfunctional uterine bleeding and cerebral infarction. Antiphospholipid antibody syndrome presenting with bleeding manifestations is rare. We should recognize that APS may be associated with not only thrombosis but also bleeding.     

Tubulo-interstitial nephritis (TIN) with no glomerular lesions, distal renal tubular acidosis and asteatosis cutis in a patient with systemic lupus erythematosus (SLE): a case report]

We report a 28-year-old woman with systemic lupus erythematosus (SLE) who showed tubulo-interstitial nephritis (TIN) without any glomerular changes. In 1990, she was admitted to our hospital, complaining of anorexia, vomiting and persistent high fever. Laboratory findings showed proteinuria, pancytopenia, hypocomplementemia and positive for antinuclear antibody, anti-DNA antibody, anti-Sm antibody, anti-SSA antibody and anti-SSB antibody. We made a diagnosis of SLE. Furthermore, distal renal tubular acidosis and asteatosis cutis were revealed. The diagnosis of Sj?gren's syndrome was not made. We treated with high-dose prednisolone (60mg/day) and achieved improvement of symptoms and laboratory data. Open renal biopsy showed TIN without any glomerular changes. Predominant TIN is very rare in SLE. We discussed its pathogenesis and relation to the renal lesions of Sj?gren's syndrome.     

Fulminant Crohn's colitis: when only an antibody will do

BACKGROUND: Fulminant Crohn's colitis complicated by toxic dilatation of the large bowel usually requires surgery, which carries a substantial morbidity and mortality rate. METHODS: We report the case of a female with steroid refractory fulminant Crohn's colitis complicated by toxic megacolon who was successfully treated with the antitumor necrosis factor monoclonal antibody adalimumab. RESULTS: Adalimumab induced rapid clinical response and remission, thereby avoiding emergency colectomy. CONCLUSIONS: This is the first report of treatment of fulminant Crohn's colitis with an antitumor necrosis factor antibody and the first report of the use of adalimumab for fulminant colitis of any cause.     

Acute acalculous cholecystitis with a decrease in CD4/CD8 ratio

Acute acalculous cholecystitis (AAC) usually occurs in the elderly and in those with severe preexisting pathological conditions. However, there have recently been reports of AAC in relatively young immunosuppressed patients, such as those with acquired immunodeficiency syndrome (AIDS). We report here a 27-year-old woman with AAC who received an emergent cholecystectomy. Although anti-human immunodeficiency virus antibody (anti-HIV) was not detected, a decrease in the CD4/CD8 ratio in sera was found. This rare case of AAC in a patient with decreased CD4/CD8 ratio who showed no other related diseases suggests that surgeons should keep in mind the possible presence of immunosuppression in this condition.     

A case of adult T cell leukemia/lymphoma presenting as severe tracheal stenosis

A 57-year-old woman was referred to our hospital for further examination of a tracheal stenosis shown on computed tomography findings. Bronchoscopy revealed multiple protruding tumors in the lumen of the trachea. Cytological findings of the cell block material from pleural effusion indicated that the T-cell lymphoma was composed of pleomorphic lymphoid cells. Serum human T-cell leukemia virus type 1 antibody was positive and supported the clinical diagnosis of ATLL. Systemic chemotherapy induced the remarkable improvement of the lesions, the infiltrative lung shadow and the soft tissue neoplasm. We report a rare case of adult T cell leukemia/lymphoma (ATLL) with endobronchial involvement.     

Herpes simplex virus type-1 meningoencephalitis showing disseminated cortical lesions

We report a 41-year-old man with meningoencephalitis associated with herpes simplex virus type 1 (HSV-1). The patient developed fever, headache and dysuria followed by generalized convulsion and neck stiffness, and the CSF showed pleocytosis. The titers of enzyme-linked immunosorbent assay against HSV measured 6 days after onset showed a significant rise; IgG antibody 4.89 (<0.2) and IgM antibody 1.45 (<0.8) in CSF, IgG antibody 46.1 (<2.0) and IgM antibody 1.76 (<0.8) in the serum. The antibody index for IgG was 0.50, and that for IgM was 4.2. CFS neutralization test showed HSV-1 antibody of x16 and HSV-2 antibody of 相似文献