Immunoregulatory abnormalities in evans syndrome

Wang, Winfred C.; Herrod, Henry G.; Pui, Ching‐Hon; Presbury, Gerald; Wilimas, Judith A.

doi:10.1002/ajh.2830150409

Cited by 59 publications

(56 citation statements)

References 30 publications

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“…7 Immune abnormalities, intrinsic to the disease and/or to chronic immune suppression, are associated with Evans syndrome. 8 This patient's course demonstrated numerous opportunistic infections mandating pre-, peri-and posttransplant anti-microbial therapy.…”

Section: Discussionmentioning

confidence: 90%

Allogeneic stem cell transplantation for Evans syndrome

Oyama

Papadopoulos

E.C.M.³

et al. 2001

Bone Marrow Transplant

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Summary:Evans syndrome is a rare disorder characterized by combined autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Standard treatments consist of transfusions, corticosteroids, splenectomy, IVIG, anabolic steroids, vincristine, alkylating agents, or cyclosporine. In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome but may also be complicated by significant toxicities. Bone Marrow Transplantation (2001) 28, 903-905. Keywords: Evans syndrome; allogeneic stem cell transplantationIn December 1998, a 28-year-old male patient with refractory Evans syndrome (Hb of 9.1g/dl and platelets of 27 × 10 9 /l) was referred to Northwestern University for an allogeneic matched sibling HSCT. Symptoms first occurred in February 1996 when epistaxis and ecchymosis developed following flu-like symptoms of headache and diarrhea. Thrombocytopenia and anemia were present with platelets of 2 × 10 9 /l, hemoglobin of 8.8 g/dl, and WBC of 6.3 × 10 9 /l. Evidence of hemolysis included hyperbilirubinemia (3.8 mg/dl), an increased LDH (424 U/l), decreased haptoglobin (7 mg/dl), reticulocytosis (corrected reticulocyte count 5.5%), bone marrow erythroid hyperplasia, and a direct Coombs test (DAT) positive for warm reactive antiIgG and anti-C3D. Evidence for ITP included anti-platelet antibodies and increased megakaryocytes within the marrow. Cytomegalovirus (CMV) antibody was positive for IgM but negative for IgG indicating recent infection. There was no other evidence of coincidental or precipitating infections. Serology was negative for HIV, hepatitis A, B, and C, and indicated past Epstein-Barr virus infection. Anti-nuclear and anti-double stranded DNA antibodies were negative. An initial response was obtained with intravenous immunoglobulin (IVIG) and corticosteroids. Due to inability to wean high-dose corticosteroid therapy, or transfusion-dependent cytopenias, the patient was treated and subsequently failed multiple interventions including splenectomy (August 1996), IVIG, danazol, and vincristine (April 1997), azathioprine 100 mg/day (August 1997), IVIG (March 1998), and cyclosporine, IVIG, and vincristine (April 1998). Upon referral for transplant, the patient was platelet and red blood cell (RBC) transfusion-dependent despite prednisone, cyclosporine, and IVIG.Pre-transplant, the disease and treatment-related complications included hemoglobinuria, malaise, steroid-induced diabetes, vincristine-induced peripheral neuropathy, cyclosporine-induced tremor and nephropathy necessitating daily intravenous hydration, anemia-related syncope, retinal hemorrhage, and respiratory and renal failure that necessitated ICU transfer and respiratory support. Pre-transplant opportunistic infectious included cryptococcal fungemia, persistent CMV viremia, and mycobacterium avium positive blood cultures. Maintenance antimicrobial therapy ...

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Section: Discussionmentioning

confidence: 90%

Allogeneic stem cell transplantation for Evans syndrome

Oyama

Papadopoulos

E.C.M.³

et al. 2001

Bone Marrow Transplant

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“…More recently, the spectrum of the disease has broadened, especially in children, and there is increasing evidence to suggest that ES reflects a state of profound immune dysregulation as opposed to a coincidental combination of immune cytopenias. 16 To better define the clinical spectrum of adult ES, we have analyzed retrospectively the data from 68 adults with a well-defined ES seen in the departments of internal medicine or hematology of 8 major institutions in France and Italy. Despite the possible bias because of the retrospective design of the study and because the number of patients remains too small to point out many statistically significant differences between subgroups, this analysis gives a new insight into adult ES.…”

Section: Discussionmentioning

confidence: 99%

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Michel

Chanet²,

Dechartres

et al. 2009

Blood

239

257

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Evans syndrome (ES) is a rare disease characterized by the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) and/or immune neutropenia. To better describe the characteristics and outcome of ES in adults, a survey was initiated in 2005. The data from 68 patients (60% of them women) fulfilling strict inclusion criteria for ES are reported. The mean age at time of ITP and/or AIHA onset was 52 plus or minus 33 years, both cytopenias occurred simultaneously in 37 cases (54.5%). ES was considered as "primary" in 34 patients (50%) but was associated with an underlying disorder in half of the cases, including mainly systemic lupus, lymphoproliferative disorders, and common variable immunodeficiency. All patients were given corticosteroids, but 50 of them (73%) required at least one "second-line" treatment, including splenectomy(n ‫؍‬ 19) and rituximab (n ‫؍‬ 11). At time of analysis, after a mean follow-up of 4.8 years, only 22 patients (32%) were in remission off treatment; 16 (24%) had died. In elderly patients, the risk of cardiovascular manifestations related to AIHA seems to be higher than the ITP-related risk of severe bleeding. In conclusion, ES is a potentially life-threatening condition that may be associated with other underlying autoimmune or lymphoproliferative disorders. (Blood. 2009;114:3167-3172)

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“…Interestingly, Wang et al [17]demonstrated decreased CD4 and increased CD8 circulating lymphocyte populations in Evans syndrome [17]. Also, the BAL pattern of BOOP is characterized by a decreased CD4/CD8 ratio with predominant CD8 lymphocytes [18].…”

Section: Discussionmentioning

confidence: 99%

Bronchiolitis obliterans Organizing Pneumonia Associated with Evans Syndrome

et al. 2001

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The association of bronchiolitis obliterans organizing pneumonia (BOOP) with insulin-dependent diabetes mellitus (IDDM) and Evans syndrome (autoimmune pancytopenia) has not been reported previously. We describe the case of a 4-year-old child diagnosed with IDDM and Evans syndrome who presented malaise, fever and nonproductive cough for several months. The chest radiograph revealed several patchy alveolar opacities with peripheral and bilateral distribution and multiple hilar and mediastinal adenopathies. An open lung biopsy established the diagnosis of BOOP. During the follow-up over the next 7 years, the patient had chronic relapses in spite of corticosteroid treatment and developed restrictive lung disease.

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Immunoregulatory abnormalities in evans syndrome

Cited by 59 publications

References 30 publications

Allogeneic stem cell transplantation for Evans syndrome

Allogeneic stem cell transplantation for Evans syndrome

The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Bronchiolitis obliterans Organizing Pneumonia Associated with Evans Syndrome

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