Evans syndrome

Evans syndrome is one of the autoimmune diseases. The body's defense depends on it against its own red Blutkörpchern and against the platelets. The disease may be secondary as a separate entity or in approximately 50% under other conditions, such as Chronic lymphocytic leukemia or systemic lupus erythematosus occur. The underlying cause of the disease is not known. The incidence is about 1:1,000,000. The disease was first described by RS Evans and RT Duane in 1949 and then again shown by Evans and another in 1951 in more detail.

Symptoms

In the patients, the autoimmune hemolytic anemia alone or in combination with autoimmune thrombocytopenia. Because of the thrombocytopenia bleeding may occur.

Secondary Evans Syndrome

In about half the cases the Evans syndrome is based on a different disease:

• Autoimmune diseases are the most common cause, especially systemic lupus erythematosus, Sjogren's syndrome as well as the or the antiphospholipid syndrome.
• Infectious diseases caused by cytomegalovirus, influenza A, parvovirus, hepatitis virus, varicella, Nocardia, Leishmania or Epstein -Barr virus
• Tumor diseases such as chronic lymphocytic leukemia, B- cell and T - Tell- non-Hodgkin's lymphoma, multiple myeloma, monoclonal gammopathy of undetermined significance, amyloidosis, chronic myelomonocytic leukemia, Kaposi's sarcoma, or pancreatic adenocarcinoma
• Others, such as Graves' disease, dermatomyositis, Guillain -Barre syndrome, ulcerative colitis, bronchiolitis obliterans ( BOOP ), Castleman's disease, celiac disease or acute inflammatory demyelinating polyradiculoneuropathy.
• Pregnancy

Diagnosis

As with autoimmune hemolytic anemia of by signs of hemolysis as a decreased hemoglobin content and a debased haptoglobin. In addition, the platelets are low.

Laboratory

In most cases the erythrocyte sedimentation rate is greatly increased. In addition, one sees a poor settling of erythrocytes in the reduction tube. The hematocrit is lowered and the lactate dehydrogenase is almost always slightly increased. The direct Coombs' test is positive. In rare cases, neutropenia is observed instead of thrombocytopenia

Therapy

The treatment recommendations are based solely on case series and case reports, there are no randomized controlled trials.

The therapy consists of the administration of glucocorticoids since the first description by Dameshek 1950. The goal is a more than 30% hematocrit or hemoglobin level above 10 g / dL. When setting of glucocorticoids, no improvement, or to relapse, can be used immunoglobulins or immunosuppressive agents (eg, danazol, cyclosporine, or vincristine ). The off- label use of rituximab was successful in some cases. As with the ITP can be achieved by removal of the spleen ( splenectomy ) treat the bleeding symptomatic remission is observed in 20-40%. In recurrent and otherwise uncontrollable cases can be a bone marrow transplant.

Swell

• Autoimmune