Evans' syndrome (thrombocytopenic purpura) 287.32 Evans' (thrombocytopenic purpura) 287.32 ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 287.32 is one of thousands of ICD-9-CM codes used in healthcare.
The diagnosis of primary Evans syndrome is made upon blood tests to confirm not only hemolytic anemia and immune thrombocytopenic purpura, but also a positive direct antiglobulin test (DAT) and an absence of any known underlying cause.
Signs and symptoms may include purpura, paleness, fatigue, and light-headedness. The exact cause of this condition is unknown. The best treatment options for Evans syndrome depend on many factors, including the severity of the condition; the signs and symptoms present; and each person's response to certain therapies. [1] [2]
It has been observed that there is a risk of developing other autoimmune problems and hypogammaglobulinemia, in one cohort 58% of children with Evans syndrome had CD4-/CD8- T cells which is a strong predictor for having autoimmune lymphoproliferative syndrome. Evans syndrome is considered a very rare autoimmune disease.
ICD-10-CM Code for Evans syndrome D69. 41.
Evans syndrome is an autoimmune condition that presents with two or more cytopenias, which commonly includes autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP), with or without immune neutropenia (only in 15% of cases, according to a report) [1].
Evans Syndrome as First Manifestation of Primary Immunodeficiency in Clinical Practice. J Pediatr Hematol Oncol. 2017 Oct;39(7):490-494. doi: 10.1097/MPH.
There is no specific test that will diagnose Evans syndrome. Instead, a doctor will run a variety of tests to rule out other health issues. Some of the tests that doctors may use to confirm a diagnosis include: a complete blood count (CBC), which measures a person's number of blood cells.
Evans syndrome occurs in individuals of all ages. In a 1997 survey of North American pediatric hematologists, the median reported age at diagnosis was 7.7 years (range, 0.2-26.6 years).
The annual Evans syndrome incidence and prevalence rose significantly during the study period, to 1.8 per million person-years and 21.3 per million persons, respectively, in 2016. The median survival with Evans syndrome was 7.2 years (primary Evans syndrome: 10.9 years; secondary Evans syndrome: 1.7 years).
First-line therapy is usually corticosteroids and/or intravenous immunoglobulin, to which most patients respond; however, relapse is frequent. Options for second-line therapy include immunosuppressive drugs, especially ciclosporin or mycophenolate mofetil; vincristine; danazol or a combination of these agents.
An autoimmune disease is the result of the immune system accidentally attacking your body instead of protecting it. It's unclear why your immune system does this. There are over 100 known autoimmune diseases. Common ones include lupus, rheumatoid arthritis, Crohn's disease and ulcerative colitis.
Immune thrombocytopenic purpura (ITP) is a blood disorder characterized by a decrease in the number of platelets in the blood. Platelets are cells in the blood that help stop bleeding. A decrease in platelets can cause easy bruising, bleeding gums, and internal bleeding.
Anemia is a lower-than-normal number of red blood cells in the blood. ACD is a common cause of anemia. Some conditions that can lead to ACD include: Autoimmune disorders, such as Crohn disease, systemic lupus erythematosus, rheumatoid arthritis, and ulcerative colitis.
SAN DIEGO – Female patients with Sjogren's syndrome have significantly higher white blood cell counts than their male counterparts, according to a study presented Sunday at the annual Academy of Optometry meeting here.
Two common causes of this type of anemia are sickle cell anemia and thalassemia. These conditions produce red blood cells that don't live as long as normal red blood cells.
Warm autoimmune hemolytic anemia (WAHA) is an autoimmune disorder characterized by the premature destruction of healthy red blood cells (hemolysis). Autoimmune diseases occur when one's own immune system attacks healthy tissue.
In TTP, hemolytic anemia develops because red blood cells are broken into pieces as they try to squeeze around blood clots. When TTP is the cause of hemolytic anemia, the Coombs test is negative. The test is positive if antibodies (proteins) are destroying your red blood cells.
Autoimmune hemolytic anemia (AIHA) occurs when your immune system mistakes red blood cells as unwanted substances. As a result, your body produces antibodies that destroy red blood cells, which can lead to a low amount of red blood cells (known as anemia).
198.7 Metastasis to adrenal gland 198.5 Metastasis to bone and/or marrow 198.3 Metastasis to brain and/or spinal cord 197.7 Metastasis to liver 197.0 Metastasis to lung 196.9 Metastasis to lymph nodes NOS 198.4 Metastasis to meninges (carcinomatous meningitis) 197.3 Metastasis to pleura (malignant effusion) 197.6 Metastasis to retro/peritoneum
Note that billing codes with a * are not billable without the extra digit, which usually specifies anatomic distribution in the case of lymphoma.
V42.81 Bone marrow replaced by transplant (post-transplant) 996.85 Complications bone marrow transplant (e.g graft vs. host) V59.3 Donor, bone marrow V59.02 Donor, blood stem cells V42.82 Peripheral stem cells replaced by transplant (post-transplant)
The Autoimmune Registry supports research for Evans syndrome by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
red blood cells, platelets, and/or white blood cells ). If a person does not have enough healthy red blood cells ( anemia ), they may experience weakness, fatigue, paleness, light-headedness, shortness of breath, or rapid heartbeat. Low platelets ( thrombocytopenia) can cause easy or unexplained bruising; red or purple spots on the skin (petechiae); prolonged bleeding from small cuts; and purpura. People with a low white blood cell count (neutropenia) may be more susceptible to infections, and may experience symptoms such as fever or sores inside the mouth. [2]
Disorders that can be associated with Evans syndrome include but are not limited to: autoimmune lymphoproliferative syndrome (ALPS), lupus, antiphospholipid syndrome, Sjogren syndrome, common variable immunodeficiency, IgA deficiency, certain lymphomas, and chronic lymphocytic leukemia. [1] [2]
For example, people who need to be hospitalized due to severe anemia or thrombocytopenia are often treated with blood transfusions followed by therapy with corticosteroids or intravenous (IV) immune globulin. Other treatment options include immunosuppressive drugs. [1] Most affected individuals respond to these treatments; however, relapse is frequent. [6]
While Evans syndrome is not thought to be inherited in most cases and rarely occurs in more than one person in a family, there are a few cases in the medical literature describing " familial Evans syndrome.". The majority of familial cases involve siblings that are found to have Evans syndrome.
The long-term outlook for people with Evans syndrome can vary. Some affected people may experience periods of long remission in which the signs and symptoms of the condition disappear or become less severe. Others have chronic problems with no remissions. [8]
In people who do not respond to standard treatments, therapy with rituximab may be considered. Some people with Evans syndrome respond well to rituximab treatment and experience an extended period of remission, while others have little to no response.
Evans syndrome was first described in the medical literature in 1951 by Dr. Robert Evans and associates. For years, the disorder was considered a coincidental occurrence of AIHA with thrombocytopenia and/or neutropenia. However, researchers now believe that the disorder represents a distinct condition characterized by a chronic, ...
Evans syndrome is a rare disorder in which the body’s immune system produces antibodies that mistakenly destroy red blood cells, platelets and sometimes certain white blood cell known as neutrophils. This leads to abnormally low levels of these blood cells in the body (cytopenia). The premature destruction of red blood cells (hemolysis) ...
First-line therapy for Evans syndrome often consists of corticosteroids such as prednisolone. Corticosteroids help to suppress the immune system and decrease the production of autoantibodies. Initial results are often effective.
General symptoms may include fever, a general feeling of poor health (malaise) and sores (ulcers) on the mucous membranes of the mouth. Additional symptoms that may occur in individuals with Evans syndrome include enlargement of the lymph nodes, spleen and liver.
Some physicians recommend that children with Evans syndrome be screened for ALPS because of the high prevalence of these two disorders occurring together. Screening involves testing for the presence of double negative ??T-cells (DNTs) by flow cytometry, the presence of which is indicative of ALPS.
Evans syndrome can potentially cause severe, life-threatening complications. Evans syndrome may occur by itself as a primary (idiopathic) disorder or in association with other autoimmune disorders or lymphoproliferative disorders as a secondary disorder. (Lymphoproliferative disorders are characterized by the overproduction of white blood cells.) ...
Evans syndrome may occur in combination with another disorder as a secondary condition. Secondary Evans syndrome can be associated with other disorders including autoimmune lymphoproliferative syndrome (ALPS), lupus, antiphospholipid syndrome, Sjogren’s syndrome, common variable immunodeficiency, IgA deficiency, certain lymphomas, and chronic lymphocytic leukemia. (For more information on these disorders, choose the specific disorder name as your search term in the Rare Disease Database.)
Specialty. Hematology. Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. These immune cytopenias may occur simultaneously or sequentially. Its overall phenotype resembles a combination of autoimmune hemolytic anemia ...
The syndrome was first described in 1951 by R. S. Evans and colleagues.
It has been variously reported that between 7.8% and 23% of patients who have autoimmune hemolytic anemia, will also have thrombocytopenia and thus Evans syndrome. The two cytopenias may occur together or sequentially.
In a nationwide study of Evans syndrome the median survival was 7.2 years (primary Evans syndrome: 10.9 years; secondary Evans syndrome: 1.7 years). Secondary Evans syndrome was associated with higher mortality rate than primary Evans syndrome, with a 5-year survival of 38%. Among patients with Evans syndrome, the prevailing causes of death were bleeding, infections, and hematological cancer.
Evans syndrome is considered a very rare autoimmune disease. Only one study has estimated incidence and prevalence adults. In Denmark in 2016 the annual incidence was 1.8 per 1,000,000 person years, and the prevalence was 21.3 per 1,000,000 living persons. In pre-pubertal children the incidence has been estimated to be between 0.7 to 1.2 per 1,000,000 person-years.
Although Evans syndrome seems to be a disorder of immune regulation, the exact pathophysiology is unknown, but a gradual loss of self-tolerance is postulated. Autoantibodies targeted at different antigenic determinants on red cells and platelets are assumed to cause isolated episodes of hemolytic anemia and thrombocytopenia, respectively.
systemic lupus erythematosus ), it is then common to denote it as secondary Evans syndrome.