Evans Syndrome

The Evans syndrome is one of the autoimmune diseases . The body's own defense is directed against its own red blood cells and against the platelets . The disease can be an independent entity or, in about 50%, secondary to other diseases such as B. chronic lymphocytic leukemia or systemic lupus erythematosus occur. The triggering cause of the disease is not known. The frequency is around 1: 1,000,000. The disease was first described by RS Evans and RT Duane in 1949 and then re-elaborated by Evans and others in 1951.

Symptoms

The patients are found to have autoimmune hemolytic anemia alone or in combination with autoimmune thrombocytopenia . Bleeding may occur due to the thrombocytopenia.

Secondary Evans Syndrome

In about half of the cases, Evans syndrome is caused by another disease:

• Autoimmune diseases are the most common cause, especially systemic lupus erythematosus , but also Sjogren's syndrome or antiphospholipid syndrome .
• Infectious diseases caused by cytomegalovirus , influenza A , parvovirus , hepatitis viruses, varicella , nocardia , leishmania or Epstein-Barr virus
• Tumor diseases such as chronic lymphocytic leukemia , B-cell and T-cell non-Hodgkin lymphomas , multiple myeloma , monoclonal gammopathy of unclear significance , amyloidosis , chronic myelomonocytic leukemia , Kaposi's sarcoma or pancreatic adenocarcinoma
• Immunodeficiencies such as selective immunoglobulin A deficiency or variable immunodeficiency syndrome (CVID)
• others such as Graves disease , dermatomyositis , Guillain-Barré syndrome , ulcerative colitis , bronchiolitis obliterans (BOOP), Castleman's disease , celiac disease, or acute inflammatory demyelinating polyradiculoneuropathy.
• pregnancy

diagnosis

As in autoimmune hemolytic anemia, the signs of hemolysis are found such as decreased hemoglobin and decreased haptoglobin . In addition, the blood platelets are low.

laboratory

Usually the sedimentation rate is greatly increased. In addition, you can see poor settling of the erythrocytes in the sedimentation tube. The hematocrit is decreased and the lactate dehydrogenase is almost always slightly increased. The direct Coombs test is positive. In rare cases, instead of thrombocytopenia , a neutropenia observed.

therapy

The therapy recommendations are based solely on case series and individual case reports; there are no randomized controlled studies .

Since it was first described by Dameshek in 1950, the therapy has consisted of the administration of glucocorticoids . The goal is a hematocrit over 30% or a hemoglobin level over 10 g / dl. If there is no improvement under glucocorticoids, or if relapses occur, immunoglobulins , immunosuppressants (e.g. ciclosporin , vincristine ) or the androgen preparation danazol can be used. The off-label use of rituximab has also been successful in some cases. As with ITP , the tendency to bleeding can be treated symptomatically by removing the spleen ( splenectomy ) ; remission is observed in 20–40%. In recurrent cases that cannot be controlled otherwise, a bone marrow transplant can be performed.

Individual evidence

1. ^ Evans syndrome. In: Orphanet (Rare Disease Database). , last accessed on October 2, 2013.
2. ↑ RS Evans, RT Duane: Acquired hemolytic anemia; the relation of erythrocyte antibody production to the activity of the disease; the significance of thrombocytopenia and leukopenia. In: Blood . 1949; Volume 4, pp. 1196-1213.
3. ↑ RS Evans, K. Takahashi, RT Duane, R. Payne, C. Liu: Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. In: Archives of Internal Medicine , 1951, 87 (1), pp. 48-65.
4. ^ Douglas E. Wright, Rachel P. Rosovsky, Mia Y. Platt: Case 36-2013 - A 38-Year-Old Woman with Anemia and Thrombocytopenia. In: New England Journal of Medicine , 2013, Volume 369, Issue 21, Nov. 21, 2013, pp. 2032-2043; doi: 10.1056 / NEJMcpc1215972 .
5. ↑ G. Emilia, C. Messora, G. Longo, M. Bertesi: Long-term salvage treatment by cyclosporin in refractory autoimmune haematological disorders. In: Br J Haematol . 1996; 93 (2), pp. 341-344. doi: 10.1046 / j.1365-2141 .
6. ↑ M. Koike, T. Ishiyama, K. Saito et al. a .: Effective danazol therapy for a patient with Evans syndrome. In: Rinsho Ketsueki. (Japanese) 1993; 34 (2), pp. 143-146.
7. ↑ TD Shanafelt, HL Madueme, RC Wolf, A. Tefferi: Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome. In: Mayo Clin. Proc. 2003 Nov; 78 (11), pp. 1340-1346. doi: 10.4065 / 78.11.1340 .
8. ↑ M. Zecca, B. Nobili, U. Ramenghi and a .: Rituximab for the treatment of refractory autoimmune hemolytic anemia in children. In: Blood , 15 May 2003, 101 (10), pp. 3857-3861. doi: 10.1182 / blood-2002-11-3547 .
9. ^ R. Martino, A. Sureda, S. Brunet: Peripheral blood stem cell mobilization in refractory autoimmune Evans syndrome: a cautionary case report. In: Bone Marrow Transplant . 1997, 20 (6), p. 521. doi: 10.1038 / sj.bmt.1700924 .

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !