特发性Evans综合征复发后确诊为小B淋巴细胞淋巴瘤一例

Evans综合征是指同时或相继发生自身免疫性溶血性贫血和免疫性血小板减少症,依据病因可分为原发性和继发性两种,其中血液系统恶性肿瘤是较为常见的继发因素.现报道1例特发性Evans综合征病例,首次起病时排除了可能的继发因素,经利妥昔单克隆抗体联合硼替佐米治疗后病情改善,1年后再次发病并最终确诊为小B淋巴细胞淋巴瘤,提示对...     

Application of therapeutic plasma exchange in hematology

Therapeutic plasma exchange (TPE) was used in 146 patients with hematologic disorders: hyperviscosity syndrome, 74; cryoglobulinemia, 53; porphyria, 9; immune complex disease, 3; cold agglutinin disease, 1; hemolytic uremic syndrome, 1; autoimmune hemolytic anemia, 1; autoimmune thrombocytopenia, 1; autoimmune neutropenia, 1; Clq deficiency, 1; and secondary immunodeficiency, 1. It was shown that TPE applied in patients with hyperviscosity syndrome resulted in rapid reduction of paraprotein concentrations, and normalization or significant decrease of serum viscosity associated with marked clinical improvement (regression of neurologic, renal, hematologic, visual and other disturbances). Application of TPE in patients with cryoglobulinemia resulted in plasma cryoglobulin reduction and clear clinical effects (blood flow improvement, skin ulcer healing, reversal of impaired renal function and disappearance of purpura and other abnormalities). Very good results were obtained in patients with porphyria (decreased sensitivity to sunlight) and also in patients with Clq deficiency. Satisfactory clinical improvement and better laboratory findings were also seen in patients with immune complex disease, autoimmune hemolytic anemia, autoimmune thrombocytopenia and hemolytic uremic syndrome.     

Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome

OBJECTIVE: To evaluate the efficacy of rituximab for the treatment of adult patients with immune cytopenia, including idiopathic thrombocytopenic purpura (ITP), autoimmune hemolytic anemia, and Evans syndrome. PATIENTS AND METHODS: We retrospectively reviewed the medical charts of all patients treated with rituximab for immune cytopenia at the Mayo Clinic in Rochester, Minn, through January 1, 2003. Fourteen patients (median age at first diagnosis, 51 years; range, 21-79 years) were identified who received 1 or more treatment courses of rituximab for treatment of refractory ITP (12 patients), autoimmune hemolytic anemia (AIHA) (5 patients), or both ITP and AIHA (classified as Evans syndrome) (4 patients). Data regarding age, diagnosis, date of diagnosis, previous treatments, comorbid conditions, blood cell counts before taking rituximab, number of rituximab treatments, and response to treatment were extracted and analyzed. RESULTS: Of 12 patients treated for ITP, 6 were receiving corticosteroid-based treatment either alone or combined with other immunosuppressive therapy at the time they received rituximab. Complete remission occurred in 5 (42%) of 12 patients with ITP and in 2 (40%) of 5 patients with AIHA. Response to rituximab in patients with Evans syndrome was seen in either ITP or AIHA, but not both. Complete response was often durable in ITP. Responses were seen in both splenectomized and nonsplenectomized patients. CONCLUSIONS: Our findings, considered with the results of other studies, suggest that rituximab deserves early consideration as salvage therapy for immune cytopenias that are refractory to both corticosteroid treatment and splenectomy. This series represents the largest series of adult patients with AIHA and Evans syndrome.     

Evans' syndrome: possible benefit from plasma exchange

Because of previous reports of benefit of plasma exchange in immunologic disorders, we plasmapheresed a 45-year-old woman with immune thrombocytopenia and autoimmune hemolytic anemia (Evans' syndrome) who appeared to be dying despite splenectomy, prednisone, immunosuppressives and transfusions. Nine liters of plasma were removed over a 12-day period. Prior to plasma exchange, the patient's hematocrit remained below 16 per cent despite six units of red blood cells over a three-day period. Following plasma exchange, the hematocrit rose to 29 per cent; the platelet count gradually rose from 14,500/microliter to 272,000/microliter; and the transfusion requirements declined to only two units of red blood cells over the next 37 days. We conclude that plasmapheresis should be considered in the management of patients with refractory immune thrombocytopenia and autoimmune hemolytic anemia.     

Acute thrombotic thrombocytopenic purpura following abdominal surgeries: a report of three cases

Acute thrombotic thrombocytopenic purpura (TTP) occurred in three patients following abdominal surgeries. One patient underwent extensive lysis for intestinal adhesions with bowel resection, another cholecystectomy for acute cholecystitis, and the third right colectomy and partial intestinal resection for colon cancer. The diagnosis of acute TTP was established on the basis of absent hematologic features of TTP prior to surgery and development of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and unexplained mental changes after surgery. Hematologic evidence of TTP developed 3 to 9 days after surgery. Other clinical features were acute respiratory distress syndrome (ARDS) in two patients and peripheral digit ischemic syndrome (PDIS) also in two patients. In all three patients, establishing the diagnosis of TTP was delayed. Exchange plasmapheresis in one patient was ineffective due to associated ARDS and two others died soon after the diagnosis was established. In view of our experience, postoperative TTP should be considered in the differential diagnosis of the patient who develops unexplained anemia and thrombocytopenia following an abdominal surgery. Presence of hemolytic anemia, schistocytosis, and unexplained thrombocytopenia should alert the possibility of TTP.     

A Rare Presentation of Epstein–Barr Virus Infection

BackgroundEpstein–Barr virus (EBV) is a herpesvirus spread by intimate contact. It is known to cause infectious mononucleosis. Complications, including hematologic pathology and splenic rupture, are uncommon. This report is a case of EBV-induced autoimmune hemolytic anemia and biliary stasis.Case ReportAn 18-year-old man presented to the emergency department with abdominal pain, nausea, vomiting, and jaundice. He did not have risk factors for liver injury or hepatitis. His vital signs were notable for a fever. On examination, he was obviously jaundiced, but not in distress. Laboratory evaluation showed hemolytic anemia and biliary stasis. Ultimately, his inpatient workup yielded positive EBV serology and a positive direct agglutinin test with cold agglutinins. He made a full recovery with supportive care.Why Should an Emergency Physician Be Aware of This?EBV is a widely disseminated herpesvirus. Infectious mononucleosis is a common presentation of acute infection, and treatment of EBV-related diseases are largely supportive. Complications, such as splenic rupture and hematologic pathology, are uncommon. Biliary stasis and autoimmune hemolytic anemia in the form of cold agglutinin disease secondary to EBV is rare, and typically resolves with supportive care and cold avoidance. More advanced treatment methods are available in the setting of severe hemolysis. Elevated transaminases, direct hyperbilirubinemia, or evidence of hemolytic anemia in the setting of a nonspecific viral syndrome should raise suspicion for EBV infection. Rapid recognition can lead to more prompt prevention and treatment of other EBV-related complications.     

Drug-associated disease: hematologic dysfunction

Hematologic dysfunction, including thrombocytopenia, anemia, neutropenia, thromboses, and coagulopathy, occur commonly during critical illnesses. A major challenge is to identify drug-induced causes of hematologic dysfunction. Given the wide variety of drug-induced hematologic effects, clinicians always should consider any concomitant drugs in the differential diagnosis of acquired hematologic dysfunction. The most severe effects include drug-induced aplastic anemia, heparin-induced thrombocytopenia, and drug-induced thrombotic microangiopathy. Certain drugs are associated with multiple hematologic effects. For example, cisplatin can cause hemolytic uremia syndrome and erythropoietin deficiency, and quinine can precipitate immune-mediated thrombocytopenia, immune-mediated thrombocytopenia, and thrombotic microangiopathy.     

Successful treatment of acute spleno-porto-mesenteric vein thrombosis after ChAdOx1 nCoV-19 vaccine. A case report

Several cases of deep venous thrombosis in people who had recently received Vaxzevria (previously known as COVID-19 Vaccine AstraZeneca) have recently been reported, mainly presenting as cerebral vein/cerebral venous sinus thrombosis. This syndrome has been termed “vaccine-induced immune thrombotic thrombocytopenia (VITT)”. Acute spleno-porto-mesenteric vein thrombosis is an uncommon but serious condition with potential sequelae, such as small-bowel gangrene and end-stage liver failure. We describe a case of concomitant thrombosis of portal, superior mesenteric and splenic veins in a young female patient with no other risk factors who received Vaxzevria (previously ChAdOx1 nCoV-19 vaccine, AstraZeneca) 17 days before. The diagnostic workup and the successful endovascular treatment and systemic anticoagulation management is reported.     

Refractory autoimmune hemolytic anemia in a systemic lupus erythematosus patient: A clinical case report

Warm autoimmune hemolytic anemia (AIHA) is a hematologic disorder with an incidence of 1–3 per 10⁵ individuals/year. Patients with systemic lupus erythematosus (SLE) develop AIHA in 3% of adult cases and 14% of pediatric cases. We report a case of AIHA refractory to multiple lines of treatment in a patient with SLE, who eventually responded to a proteasome inhibitor‐based combination. A patient with systemic lupus erythematous was diagnosed with symptomatic autoimmune hemolytic anemia. The patient was refractory to multiple lines of treatment including prednisone, intravenous immune globulin, methylprednisolone, rituximab, cyclophosphamide, mycophenolate mofetil, and splenectomy. She eventually had a beneficial response to a proteasome inhibitor‐based combination with bortezomib plus mycophenolate mofetil. The treatment of refractory autoimmune hemolytic anemia can be challenging. Patients with AIHA refractory to primary or secondary treatments must resort to receiving novel therapeutic modalities including combinations targeting plasma cell, T‐ and B‐cell proliferation.     

Fatal pulmonary embolism following splenectomy in a patient with Evan’s syndrome: case report and review of the literature

Background

Evans syndrome (ES) is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Splenectomy is one of the treatment options for disease refractory to medical therapy. Venous thromboembolism (VTE) following splenectomy for hematological diseases has an incidence of 10%.

Case presentation

Here we describe a case report of a young patient hospitalized with severe hemolytic anemia with Hgb 4.8 g/dl. He developed thrombocytopenia with platelet nadir of 52,000/mm³, thus formally diagnosed with ES. He failed standard medical therapy. He underwent splenectomy and had a fatal outcome. Autopsy confirmed the cause of death as pulmonary embolism (PE).

Conclusions

This case report and review of the literature highlight important aspects of the association between VTE, splenectomy, and hemolytic syndromes including the presence of thrombocytopenia. The burden of the disease is reviewed as well as various pathophysiologic mechanisms contributing to thromboembolic events in these patients and current perioperative prophylactic anticoagulation strategies. Despite an advancing body of literature increasing awareness of VTE following splenectomy, morbidity and mortality remains high. Identifying high risk individuals for thromboembolic complications from splenectomy remains a challenge. There are no consensus guidelines for proper perioperative and post-operative anti-coagulation. We encourage future research to determine which factors might be playing a role in increasing the risk for VTE in real time with hope of forming a consensus to guide management.

     

Exacerbation of autoimmune hemolytic anemia associated with pure red cell aplasia after COVID-19: A case report

Autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) are rare complications of coronavirus disease 2019 (COVID-19). Herein, we report the case of a 28-year-old Japanese man who showed severe AIHA exacerbation associated with PRCA after COVID-19. AIHA was diagnosed and maintained for 5 years. Approximately 4 weeks after COVID-19, the patient developed severe anemia (hemoglobin level, 3.4 g/dL). Laboratory test results confirmed hemolytic exacerbation of IgG-mediated warm-type AIHA. Despite the hemolysis phase, the bone marrow revealed extreme hypoplasia of erythroblasts with a decreased reticulocyte count, similar to that observed in patients with PRCA. During oral prednisolone treatment, the patient recovered from anemia and showed increased reticulocyte count and reduced hypoplasia of marrow erythroblasts. Exacerbation of AIHA and PRCA was triggered by COVID-19 because other causes were ruled out. Although this case report highlights that COVID-19 could lead to hematological complications such as AIHA and PRCA, the exact mechanisms remain unclear.     

Description of serologic features in autoimmune lymphoproliferative syndrome

BACKGROUND: Autoimmune lymphoproliferative syndrome (ALPS) is a recently recognized and rare disorder associated with inherited defects in the FAS: gene or other regulators of lymphocyte apoptosis. It is characterized by massive lymphadenopathy; splenomegaly; autoimmunity including episodes of immune hemolytic anemia, thrombocytopenia, and neutropenia.(1) The serologic basis for immune cytopenias associated with ALPS has not been previously characterized. STUDY DESIGN AND METHODS: RBC, granulocyte, and platelet serologies for ALPS patients and hepatitis C patients were assessed. Medical records were reviewed for clinical, immunologic, serologic, and transfusion history. Testing included: DAT; serum screening for antibodies to RBCs, granulocytes, platelets, cardiolipin, penicillin-coated RBCs, and human leukocyte antigens; antibody identification and IgG subclass; RBC phenotype. RESULTS: In a cohort of 11 patients with apoptosis defects (eight with heterozygous FAS: gene mutations); many had histories of hemolytic anemia (7), thrombocytopenia (4), and/or leukopenia (11); nine received steroid therapy, seven underwent splenectomy; five had been remotely transfused. On the basis of serologic testing even when they were clinically stable, nine had positive DATs; two had alloantibodies; 6 had IgG and/or IgM antibodies to cardiolipin; seven had platelet-directed antibodies; three had granulocyte-directed antibodies; none had HLA antibodies. CONCLUSIONS: Nearly all ALPS patients have antibodies directed against one or more hematopoietic cell lineages. Serologic testing is critical in the evaluation of these individuals and when transfusion is indicated, red cells that are matched for clinically significant C, E, and K antigens should be considered.     

Hemolytic uremic syndrome: an emerging health risk

Hemolytic uremic syndrome is caused primarily by Shiga toxin-producing Escherichia coli O157:H7. The most common cause of acute renal failure in children, hemolytic uremic syndrome also can occur in adults. Characteristic features of the syndrome are microangiopathic anemia, thrombotic thrombocytopenia, and renal failure. Although the presentation of this syndrome is diverse, the classic prodromal illness is bloody diarrhea following ingestion of hamburger meat contaminated with E. coli O157:H7, the most common mode of infection in the United States. Children with hemolytic uremic syndrome generally present with gastroenteritis complaints (e.g., abdominal pain or tenderness, nausea or vomiting, fever, anemia); affected adults may be asymptomatic. Complications from hemolytic uremic syndrome can include intussusception, chronic renal failure, and seizures in severe cases. Because an incubation period of approximately one week occurs between the start of diarrhea and the onset of hemolytic uremic syndrome, physicians should maintain a high index of suspicion; early laboratory testing is important to diagnose and manage this syndrome. Obtaining a complete blood count and stool culture and performing Shiga toxin testing are the first of a series of tests that may help diagnose hemolytic uremic syndrome.     

Intra-abdominal abscess caused by Listeria monocytogenes in a patient with acquired hemolytic anemia and thrombocytopenia

We report a case of an intra-abdominal abscess caused by Listeria monocytogenes in a postoperative patient with Evans syndrome (acquired hemolytic anemia and thrombocytopenia). Focal infections with L. monocytogenes are uncommon but have been reported in immunocompromised patients. A few cases of liver abscess in diabetic patients have also been reported. The present case is significant because of the paucity of previously described focal intra-abdominal infections caused by L. monocytogenes, particularly as a postsplenectomy pathogen.     

Life-threatening autoimmune hemolytic anemia in a patient with the acquired immune deficiency syndrome

Increasing numbers of patients are being seen with the acquired immune deficiency syndrome (AIDS). An abnormal serology with a positive direct antiglobulin test has been observed in these patients and is usually not thought to contribute to significant clinical morbidity. We describe a patient with AIDS who presented with a severe hemolytic anemia which was not distinguishable clinically and serologically from the idiopathic form of autoimmune hemolytic anemia.     

Autoimmune hemolytic anemia associated with a hypernephroma

This report describes a case of warm-antibody-mediated hemolytic anemia associated with a hypernephroma. Only four patients with renal cell carcinoma and Coombs'-positive hemolytic anemia have been reported previously. Although rare, the occurrence of autoimmune hemolytic anemia in association with a hypernephroma should be considered one of the possible hematologic complications of this tumor.     

Haematological Manifestations of Primary Sjogren's Syndrome: A Clinicopathological Study

Clinically significant cytopenias are thought to be uncommonin primary Sjögren's syndrome: only a few cases have beenreported in the literature. Over a 3-year period we identifiedhaematological abnormalities in 11 of 27 patients with Sjögren'ssyndrome. Six patients had a positive direct antiglobulin test,including one patient with all the features of autoimmune haemolyticanaemia and two others with some features of this condition.Four patients had immune thrombocytopenia and two patients hadmyelodysplastic syndrome. Neutropenia was noted in two patients,one patient had aplastic anaemia and one had pure red cell aplasia.Haematological disorders were found to be common in patientswith Sjögren's syndrome (40 per cent). Accordingly, wesuggest that patients with immune cytopenia(s) should be screenedfor Sjögren's syndrome using sensitive assays for anti-SS.Aand anti-SS.B antibodies, and that patients with Sjögren'ssyndrome should be periodically monitored, with a full bloodcount to rule out any haematological abnormality.     

Management of thrombocytopenia in bone marrow failure: a review

The clinical course of many neoplastic and primary bone marrow diseases will result in cytopenias secondary to bone marrow failure or infiltration. Acute and chronic leukemias, the myelodysplastic syndromes (MDS), aplastic anemia, breast and prostate cancer, as well as other hematologic and solid tumors, all may lead to chronic, severe cytopenias. Management of anemia and neutropenia are well described in the medical literature. Less well detailed are management approaches for patients with chronic thrombocytopenia, with or without active bleeding. Severe thrombocytopenia presents many difficult management choices for caregivers, patients and their families, especially near the end of life. The use of platelet transfusions in this patient population presents complex issues; platelets are logistically more difficult to transfuse than red cells and carry risks including acute febrile episodes, alloimmunization, and infection. In this review, we discuss the association of chronic thrombocytopenia to serious bleeding and the role of various prophylactic and therapeutic interventions available to palliative care and hospice providers. Specifically, this review examines the following issues: What is the morbidity and mortality from chronic thrombocytopenia in the setting of cancer or other bone marrow failure states? Is there a role for prophylactic platelet transfusions in the palliative care setting, and if so, with what frequency of monitoring, and at what transfusion threshold? What is the impact of alloimmunization and how can it be minimized? What treatments are available besides, or in addition to, platelet transfusions for acute bleeding episodes?     

Hematologic adverse effects of clopidogrel

Clopidogrel is used as a frontline antiplatelet agent in patients with coronary artery disease, cerebrovascular disease, and peripheral vascular disease. Hematologic complications and bleeding have been the most feared outcome of antithrombotic and antiplatelet agents. Among the thienopyridines, clopidogrel is considered to be a safer alternative to ticlopidine due to its decreased incidence of hematologic adverse effects. Although thrombotic thrombocytopenia purpura is the most reported hematologic adverse effect of clopidogrel; neutropenia, acquired hemophilia, isolated thrombocytopenia or idiopathic immune thrombocytopenia, and thrombotic thrombocytopenia purpura with hemolytic uremic syndrome are other rare yet recognized hematologic adverse effects of clopidogrel. Patients treated with clopidogrel should be carefully monitored for hematologic adverse effects especially in the first 2 to 3 months after initiation of therapy. Early recognition and prompt initiation of treatment can be life saving in patients who have hematologic adverse effects to clopidogrel. We have drafted this review by performing literature search using Medline, Pubmed, and EMBASE search engine with relevant search words for all reported hematologic adverse effects and manifestations of clopidogrel and their management.     

Immune cytopenias as the presenting finding in primary Sjögren's syndrome

A diagnostic delay of several years in primary Sj?gren's syndrome is common, even in patients who present with sicca symptoms. It is much more likely in cases with prominent symptomatic extraglandular involvement. We report on three such patients who presented as Coomb's positive haemolytic anaemia, systemic symptoms with agranulocytosis and gingival bleeding due to immune thrombocytopenia, to alert clinicians to the fact that primary Sj?gren's syndrome may present as clinically significant immune-mediated cytopenia in the absence of sicca symptoms. Sj?gren's syndrome, a common autoimmune disorder, should be considered in the differential diagnosis of apparently 'idiopathic' cytopenias and actively sought by directed history, Schirmer test and autoantibody screening.