Aplastic anaemia

ALASTIC ANAEMIA IN THIS TYPE BONE MARROW UNABLE TO PRODUCE ENOUGH BLOOD IN BODY.

1. SEMINAR ON TOPIC – APLASTIC ANEMIA IN CHILDREN SUBJECT - CHILD HEALTH NURSING BY, MR. ABHIJIT P. BHOYAR M. SC. NURSING CHILD HEALTH NURSING

2. APLASTIC ANAEMIA INTRODUCTION Aplastic anemia  is a serious condition in which the bone marrow does not produce enough new blood cells. It may be passed down from the parents or develop sometime during  childhood . Aplastic anaemia (AA) is a rare condition in which the bone marrow fails to produce all three major types of blood cell. It is not a form of cancer.

3. DEFINITION Aplastic anemia is a type of anemia caused by problems with bone marrow. It can range from mild to severe.

4. CAUSES Unknown etiology Stem cell defect: intrinsic defects of hematopoietic stem cells Bone marrow defects Abnormal hematopoiesis related to  humoral or cellular immune function Adverse effects of certain drugs

5. CAUSES Exposure to certain chemicals and pathogens , such as contaminated water, animal fertilizer, and pesticides Inherited conditions : mutations such as those present in the Shwachman - Bodian -Diamond syndrome (SBDS) gene (occurs from  biallelic mutations) Infections that cause autoimmune pathogenesis : immune mediated hematopoietic cell destruction from conditions such as fulminant liver failure (FLF), which occurs secondary to some forms of hepatitis, echovirus, parvovirus, metabolic veno -occlusive disease and other pathologies

6. RISK FACTORS Exposure to certain environmental toxins High dose radiation and chemotherapy treatments Certain viruses Certain medications Bone marrow diseases Pregnancy

7. PAHOPHYSIOLOGY

8. Types of aplastic anaemia There are several types of aplastic anaemia : Acquired aplastic anaemia – this is the most common type where there is often no obvious or known cause. Inherited bone marrow failure syndromes – there are rare inherited conditions which may lead patients on to develop aplastic anaemia . The most common of these is Fanconi anaemia .

9. The classification is based on how low the numbers of blood cells have fallen, which can be found out by a blood count. To make a diagnosis of AA, at least two of the following must be present: Haemoglobin less than 100g/L Platelet count less than 50 x 109/L Neutrophil count less than 1.5 x 109/L AA is classified as non-severe, severe or very severe. Non-severe aplastic anaemia This meets the above criteria for aplastic anaemia but does not meet the criteria for severe or very severe aplastic anaemia . Severe aplastic anaemia Hypocellular bone marrow and any two of the following: A low platelet count (less than 20 x 109/L) A reticulocyte count less than 20 or 60 x 109/L A neutrophil count less than 0.5 x 109/L Very severe aplastic anaemia Hypocellular bone marrow and any two of the following: A low platelet count (less than 20 x 109/L) A reticulocyte count less than 20 or 60 x 109/L A neutrophil count less than 0.2 x 109/L The difference between severe and very severe AA is how low the number of neutrophils falls; which increases the risk of severe infection. Hypocellular bone marrow is defined as bone marrow cells <25%, or 25-50% with less than 30% of stem cells in your bone marrow.

10. CLINICAL MANIFESTATIONS Fatigue Shortness of breath with activity Weakness Rapid heart rate Pale skin Easy bruising Nosebleeds and bleeding gums Lengthy bleeding from cuts Skin rash Fever Shortened attention span

11. DIAGNOSTIC EVALUATION Medical history & physical examination Blood tests

12. Bone marrow examination Chromosomal studies Testing for immunological etiologies Testing for Shwachman - Bodian -Diamond syndrome

13. HLA-typed testing microarray analysis

14. MANAGEMENT Blood Transfusions Bone Marrow Transplantation Immune Suppressing Medication   Androgen therapy Antibiotics therapy

15. Erythropoietin treatment and pancreatic enzymes Close monitoring Bed rest

16. NURSING MANAGEMENT Frequent assessment Monitoring for adverse effects Assessment of coping abilities Provide emotional support Patient and family teaching

17. COMPLICATION Complications may include: Severe infections or bleeding Complications of bone marrow transplant Reactions to medicines Hemochromatosis (buildup of too much iron in the body tissues from many red cell transfusions)

18. PREVENTION Limiting exposure to certain environmental toxins Environmental toxins include those found in gasoline, paint, oil and coal emissions, and industrial solvents.

19. references