Fulminant hepatic failure and autoimmune hemolytic anemia associated with Epstein-Barr virus infection

Neisseria weaveri (formerly CDC [Centers for Disease Control and Prevention] group M-5 is part of the normal canine oral flora. Infections in humans are usually associated with dog bite wounds. Very rarely the organism has been isolated from sites other than wounds, or from deep seated infections. A 60-year-old man was admitted to our hospital because of an acute exacerbation of his bronchiectasis. Gram stain of bronchial washings and expectorated sputum showed numerous polymorphs and Gram-negative bacilli. Routine bacterial culture yielded a heavy pure growth of a Gram-negative rod-shaped organism that was strongly oxidase and catalase positive, indole negative, non-motile and did not ferment carbohydrates. The organism was identified as N. weaveri by using 16S rRNA sequencing. The patient was treated with a 3 weeks course of ofloxacin and had a good response. Sputum culture after treatment yielded normal respiratory flora only. To our knowledge, this is the first reported case of lower respiratory tract infection caused by N. weaveri.     

Fulminant course of leptospirosis complicated by multiple organ failure

We describe a case of a 39-year-old male, who initially presented with severe muscle pain, fever, shortness of breath and tachycardia. He was admitted to hospital with suspected myocarditis. The next days he developed a generalized icterus and acute renal failure. Suspecting leptospirosis an intravenous therapy with penicillin was started. Due to pulmonary and circulatory insufficiency intensive care was necessary. In course the patient developed all known manifestations of leptospirosis including, cardiac arrhythmia and asystolia due to AV-block III degrees, recurrent atelectases of the lungs, hyperbilirubinemia, thrombocytopenia, hepatitis, pancreatitis, very severe rhabdomyolysis and polyradiculitis with areflexia and tetraplegia. Additionally, the patient had a transient hyperthyreosis, which has not been described in the literature so far. After 33 days the patient left the intensive care unit and was discharged out of hospital a fortnight later. 4 weeks later he was able to return to work. The only residuum of this illness is a partial paresis of his right quadriceps muscle.     

Reticulocytopenia in autoimmune hemolytic anemia

1. In a series of 57 cases of autoimmune hemolytic anemia it was found that25 or 44 per cent had a relative reticulocytopenia at times of hemolytic crisis.The mortality rate in this group was significantly higher than in those whoshowed a reticulocyte response consistent with the severity of anemia. Specialsignificance is given to the high mortality rate among the patients with idiopathic AHA.

2. Four illustrative cases are presented.

3. There is a discussion of mechanisms that may be responsible for reticulocytopenia in the presence of severe anemia and erythroid hyperplasia of the marrow.

Submitted on January 23, 1956 Accepted on March 7, 1956     

Treatment of autoimmune hemolytic anemia

The appropriate therapy of autoimmune hemolytic anemia (AIHA) is dependent on the correct diagnosis and classification of this family of hemolytic disorders. Although the majority of cases are warm AIHA, there are several distinct types of cold AIHA and a number of drug-induced etiologies of AIHA, which must be investigated to determine if stopping a drug will induce a remission. In warm AIHA, corticosteroids are standard, followed by consideration of splenectomy in recalcitrant cases. If steroids and splenectomy are insufficient, other forms of immunosuppressive therapy are typically initiated. In cold AIHA, keeping the patient warm in often sufficient, but therapy directed at an underlying lympholiferative disorder may be helpful. Brisk hemolysis, inadequate responses to therapy, and worsening anemia require transfusion therapy. Although the pretransfusion workup is made difficult by the presence of the autoantibody, transfusion services can usually provide blood safe for transfusion by excluding underlying alloantibodies. When transfusion is urgently required and compatible blood cannot be located, incompatible blood may be provided as a life-saving measure. Communication between the transfusion service and the hematologist is critical to assess the risks in these settings. Hemoglobin-based oxygen carriers may provide an important bridging therapy in the future. Requests for "least incompatible" blood do not enhance transfusion safety and often result in unnecessary delays.     

自身免疫性溶血性贫血127例临床分析

目的：研究自身免疫性溶血性贫血的临床特征。方法：对127例自身免疫性溶血性贫血的临床资料进行回顾性分析。结果：①127例中病因明确者41例（32．3％），感染性疾病16例（39．0％），且感染是疾病复发或病情加重最常见的诱因，与药物有关病例5例（12．2％）。②抗体分型与临床及预后相关：IgG＋C3型溶血较重，C3型溶血较轻，IgG型介于两者之间；C3型完全缓解率较高。③误诊病例15例（11．8％）。④糖皮质激素加用免疫抑制剂治疗或糖皮质激素加用大剂量静脉免疫球蛋白治疗疗效优于单用糖皮质激素治疗（P〈0．01）。结论：AIHA抗体分型与临床及预后相关；基础疾病的追查及治疗有重要意义；应重视临床表现及实验室特征以防止误诊病例的发生；糖皮质激素加用免疫抑制剂或加用大剂量静脉免疫球蛋白的治疗方法疗效较好。     

Acquired von Willebrand syndrome with autoimmune hemolytic anemia

A 17-year-old female underwent examination due to the chief complaints of epistaxis and bleeding from the gums. In addition to hemolytic anemia (Coombs positivity), she was found to have a marked reduction in the von Willebrand factor antigen (vWF:Ag) and ristocetin cofactor activity (vWF:RCo). Anti vWF antibodies mainly comprised of IgG1 fractions were also detected, so acquired von Willebrand syndrome (AvWS) with autoimmune hemolytic anemia (AIHA) was diagnosed. The recovery of vWF:Ag and vWF:RCo following administration of a factor VIII concentrate containing vWF was good, but the half life was short at about two hours and the collagen binding activity and vWF:RCo in mixing tests with control plasma were only mildly inhibited. Therefore, the anti vWF antibody in the present case was thought not to inhibit vWF function but to be involved in clearance promotion. Hemostatic control during hemorrhage was performed using a factor VIII concentrate containing vWF. With the administration of prednisolone thereafter, anti vWF antibodies disappeared at the same time as there was improvement in the AIHA. Throughout the course the patient contracted acute hepatitis A, became pregnant and gave birth, but there were no clear effects on the clinical phenotype of AvWS.     

Idiopathic autoimmune hemolytic anemia successfully treated with danazol

A 50 year old female suffered from idiopathic autoimmune hemolytic anemia. She was initially treated with prednisolone, and anemia was improved moderately. However, maintenance of Hb value was difficult with the decreased dosage of prednisolone. She was subsequently administered danazol, and anemia was completely improved. It is worth to use danazol for the treatment of the patient of autoimmune hemolytic anemia who is refractory to prednisolone therapy.     

Acquired von Willebrand syndrome with autoimmune hemolytic anemia

Acquired von Willebrand syndrome is reported in a 31-year-old woman with autoimmune hemolytic anemia (AIHA). The patient, who had no family history of bleeding, presented with a hemorrhagic diathesis of recent origin. Routine coagulation studies showed a normal platelet count, prolonged bleeding time and abnormal glass bead retention. Plasma levels of factor VIII-von Willebrand factor (F VIII-vWF) were low and ristocetin-induced platelet aggregation (RIPA) was decreased. In vitro, the patient's plasma exhibited inhibitory activity against vWF: ristocetin cofactor activity (vWF: Rco) but had no effect on RIPA. The multimeric pattern of the patient's vWF resembled that of patients with inherited type II von Willebrand disease, in that the largest multimers were missing. Clinical improvement resulted after treatment of AIHA with prednisolone (PSL), and F VIII-vWF returned to normal levels with normalization of vWF multimers. However, following tapering the dose of PSL, plasma levels of F VIII-vWF again decreased.     

Infantile giant cell hepatitis with autoimmune hemolytic anemia

Giant cell hepatitis (GCH) is characterized by large and multinucleated (syncytial) hepatocytes in the context of liver inflammation. Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity. The etiology is unknown; concomitant viral infections (as potential trigger factors) have been identified in a few patients. The pathogenesis reportedly relies upon immune-mediated/ autoimmune mechanisms. This condition should be considered in any infant developing Coombs-positive anemia; indeed, anemia usually precedes the development of hepatitis. The clinical course is usually aggressive without the appropriate immunosuppressive therapy, which may include steroids, conventional immunosuppressors (e.g., azathioprine and cyclophosphamide as first-line treatments), intravenous immunoglobulin, and biologics (rituximab). Improvements in medical management (including the availability of rituximab) have significantly reduced the mortality of this condition in the last decade.     

Complete remission of multiple myeloma after autoimmune hemolytic anemia: possible association with interferon-alpha

A patient with multiple myeloma (MM) was being maintained on human recombinant interferon-alpha (INF-alpha) after VAD and autologous bone marrow transplantation (pretreated with melphalan). An episode of immune thrombocytopenia and (Coombs positive) autoimmune hemolytic anemia (AIHA) was noted while on maintenance INF-alpha, which remitted when it was withdrawn. Following this event, he achieved a state of stable disease that persists (more than 3 years) with no specific myeloma treatment. This sequence of events suggests a relationship between an immunological reaction induced by INF-alpha and the prolonged phase of stable disease.