Aplastic anemia

And treatment of aplastic anemia
DESCRIPTION
Enough of the bone marrow to peripheral blood elamanlarını which is a type of anemia usually is a slow trend. Pure red cell aplasia, erythroid elements of the construction is due to inadequate selective. May be associated with thymoma. Constitutional (Fanconi), anemia, anemia, congenital anomalies birliktedir.Aplastik Ehrlich first in 1888 by German pathologist, who died of bone marrow failure due to work on pregnant woman results have been described. Aplastic anemia in 1904, the term has been used for the first time.
The SYSTEM EFFECTS AND ORGANS
Hematologic, Lymphatic, Immune System
GENETICS
Constitutional type, is autosomal recessive.
Incidence / Prevalence
Are 500 new cases each year in the United States. Rastlanmıştır.Fransa quite common in Asia a few times also been reported as 1.5 million.
AGE AND GENDER
Acquired type, may be at any age. Constitutional type, in Childhood and Youth
Is common. Male and female ratio is equal.
Symptoms
Shortness of breath
Ecchymosis
Petechiae
Fatigue
High fever
Bleeding, menorrhagia, Fecal Occult Blood, melena, Epistaxis
Paleness
Palpitation
Progressive weakness
Flame-shaped retinal hemorrhages
Systolic ejection murmur
Weight loss
Hyperpigmentation (Acquired Type) and
Constitutional
Short stature
Microcephaly
Radius and Elbaşparmak anomalies
Renal anomalies
Hypospadias
REASONS
Idiopathic (50%)
Stem Cells Plöripotent damage / destruction
Immunologic damage
Benzene, exposure to toxic substances such as inorganic arsenic
Infectious hepatitis
Radiation therapy
Drugs, particularly antibiotics, anticonvulsants, gold
Pregnancy
Inherited (constitutional)
RISK FACTORS
Viral Diseases
Exposure to toxins
Thymic tumors (red cell aplasia)
Differential Diagnosis
Other causes of pancytopenia
Myelodysplastic Disease
Acute Leukemia
Hairy Cell Leukemia
Systemic lupus erythematosus
Disseminated Infection
Hypersplenism
Temporary Eritroblastopenisi Childhood
LABORATORY
Pancytopenia
Anemia
Leukopenia
Neutropenia
Thrombocytopenia
Elongation of bleeding time
Decreased reticulocytes
Increased serum iron, depending on the transfusion of
Total Iron Binding Capacity to normal
The mean erythrocyte volume (MCV> 104) limit the increase
Hematuria
Increase in fetal hemoglobin (FA)
In special cases, increased chromosomal fractures (constitutional)
Pathological findings
Normocromic Erythrocytes
Bone marrow
Increased iron stores.
Selülarite decreased (<10%)
Decreased megakaryocytes
Null decreased
Erythrocyte precursors (precursors) were decreased.
OTHER TESTS TO be
Chromosome breakage (constitutional), fetal hemoglobin
RADIOLOGY / IMAGING
Computed tomography; thymoma with areas for red cell aplasia are suspected thymus
Radiology of the hands of the radius and thumb (constitutional)
Ultrasonography; a kidney for (constitutional)
Diagnostic procedures
Bone Marrow Biopsy
MAINTENANCE / PRECAUTIONS
And treated in hospital in a specialized center.
Serious measures to support
Oxygen therapy
Good oral hygiene
Avoidance of the causative agents
All patients and their families, Human Leukocyte Antigens (HLA) Test
Transfusion support
Donors severe aplastic anemia patients and HLA-identical bone marrow transplantation.
30 55 transplantations of restricting the upper age limit for living is variable.
Not suitable donors for patients, immunosuppressive therapy: 5.Siklosporin, corticosteroids, antithymocyte globulin (ATG)
Unrelated donor transplants when other treatments have failed
Thymectomy for thymoma
If less serious, androgen therapy
Neutropenic patients should be isolated.
A balanced diet can reduce susceptibility to infections.
Possible toxic agents should be avoided.
Security measures should be used when working with radiation.
TREATMENT
Antithymocyte globulin (ATG)
This is against human T cells is enhanced horse serum containing polyclonal antibodies. To determine any hypersensitivity skin test should be done. In elderly patients and donors are given to patients who did not. Diluted in 500 ml isotonic with serum, 10-20 mg / kg dose, 4-6 hours infusion, followed by 8-14 days consecutively is given. As can be given alone may be combined with corticosteroids.
Cyclosporine
Initially 10 mg / kg, then 50-10 mg / kg / day given in the form of reducing. Blood levels are monitored, according to the normal value measurement techniques used değişir.2-three months of the application may be sufficient.
And does not respond to other treatment options for patients with less severe disease is useful. Oksimetalon-1-2 mg / kg / day given orally.
2-3 months to assess implementation of the answer is sufficient.
Prednisone (pure red blood cell aplasia)
Received immunosuppressive therapy relapses may occur after the first response. Growth hormone and bone marrow transplantation between the treatment options. Supportive therapy; red blood cell and platelet transfusions containing blood, antibiotics for infection control and to prevent the accumulation of iron chelation therapy is applied.
Children with
Pure red blood cell anemia (Diamond-Blackfan anemia) and constitutional Anemia is common in childhood.
Idiopathic Aplastic Anemia is common in adolescence. Children, Secondary Aplastic Anemia, exposed to ionizing radiation or cytotoxic chemotherapeutic agents can be seen in those treated.
In ELDERLY Many elderly people have been exposed to drugs and therefore are more open to Secondary Aplastic Anemia.
In pregnancy Pregnancy, may lead to the Aplastic Anemia.
Complications of Bleeding
Infection
Transfusion Hemosiderozu
Transfusion hepatitis
Heart failure
Acute Leukemia
Neoplasia, constitutional can cause anemia.
The course of a disease
Treatment depends on the patient's condition.
Some drugs can make aplastic anemia;
amphotericin B, sulfonamides, Phenacetin, trimetadion, klordiazepoksid, tolbutamide, tiourasil, carbamazepine, chloramphenicol, tetracycline, oksifenbutazon, arsenic, chlorpromazine, primetamin, karbimazol, asetazolamid, colchicine, penicillin, aspirin, mefenitoin, bismuth, promazin, quinacrin, methimazole, klorotiazid, dinitrofenol, ristocetin, indomethacin, phenytoin, gold, trifluoperazin, karbutamid, perchlorate, chlorpheniramine, streptomycin, fenilbutazon, primidone, meprobamat, klorpropamid, tiosiyanat, tripelenamin, benzene