Aplastic anemia -rare disease caused by a decrease in or damage to marrow stem cells, damage to the microenvironment within the

marrow. And replacement of marrow with fat. -can be caused by toxins(chemo drugs depress bone marrow) -genetic disorder -T cells mediate an inappropriate attack against bone marrow. -bone marrow transplant or peripheral blood cell transplant Manifestations: fatigue, pallor, dyspnea -if the person is born with incapable bone marrowinadequacy of blood cells Stem cells: the start of blood cells Pathophysiology: dev. When damaged or destroyed stem cells Complications: Inadequate blood circulating in the body -immunospuression(kulang ang rbc..then kulang dn ang wbc that fights off infection)..more at risk for infection. Assessment findings: Fatigue-low oxygenation.. because less rbcs(oxygen carriers) Pallor: extremities are cold because of lacking blood cellsrbcs gives body heat Diff. breathing: heat compensates because of lacking oxygen from low rbcs.. Headacheless oxygenation in the brain Reduced thrombocytes=less platelets=risk for bruisesand ekimosis Melena(black stool)- g.i is system is at risk of bleeding Diagnostic findings: -Bone marrow biopsy/aspiration -fecal occult blood test Medical management: -bone marrow transplant( stem cells) -. dietary change( rich in vit. K, vitamins high protein and high calorie diet -transfusion: because lesser blood countgive packed rbcs and platelets Hemophelia: Type A-Classical hemophilia def. of clotting factor A Type B- def. of Christmas clotting factor 9 Cause of hemo. X link recessive disorder -ex. If father has hemophilia and mother doesnt..all daughters will be carriers - if mother is carrierthe sons have 50% chance of getting the disorderdaughter 50% of becoming carrier -abnormal bleeding( due to hemorrhage, and can result in hepatitisand liver damage. -because fragments of the liver are hit because liver is not protected as well as other organs

Assessment findings; -Bleeding mouth.throat, thorax -hemarthrosis- bleeding in the synovial area or in joints -multiple bruises -Prolonged bleeding after circumcision, immunization, and other minor injuries. -continuous bleeding when injected because they have no or weak clotting factor due to lack of conversion of fibrinogen=no platelet clotting Diagnostic test: Hemophelia a: Prolonged partial thrombin time Hemophelia b: Same Medical management: -Avoid suturing or cotorization bec. It will aggravate bleeding -Administration of fresh frozen plasma Megaloblastic Anemia -deficiency of b12 or folic acididentical bone marrow and peripheral blood changes occur because both vitamins are essential for normal DNA synthesis. -erythrocytes are abnormally large and are called megaloblastic red cells. -and not functioning -can happen all at the same time with pernicious anemia (bec. Def. also in b12 and folic acid which are needed for RBC) Folic acid deficiency -Folic acid is stored as compounds referred to as folates. The folate stores in the body are much smaller than those of vitamin b12, and are quickly depleted when the dietary intake of folate is deficient( within 4 months) -occur in people who rarely eat uncooked vegetables -folate is found in green vegetables and liver. -folic acid req. are increased in patients with chronic haemolytic anemias and in women who are pregnant. And in patients with malabsortive diseases -increasing folic acid in diet -folic acid given IM for people with malabsorbtion problems Vitamin b12 deficiency: -can occur with inadequate dietary intake -vegetarians who consume no meat or dairy products -faulty absorbtion in gi tract - occurs in conditions such as crohns disease or after ileal resection or gastrectomy -without instrinsic factor orally consumed b12 will not be absorbed and erythrocyte production is eventually diminished. -even if adequate b12 and intrinsic factor are present, a def. may occur if disease involving the ileum or pancreas impairs absorbtion. -treated by vitamin b12 replacements

-for vegetarians..oral supplements or soy milk -IM for malabsorbtion problems Myelodysplastic anemia -myeloid stemcells are abnormal -most common feature is dysplasia of erythrocytes -prolonged=acute myeloid leukaemia MDS:fatigue at varying levels, risk for infection and bleeding Pernicious anemia- pallor, sore tongue, weight loss, peristesia(numbness of hands and feet) Diagnostic studies for p. anemia- shillings test ( admin. Of small radioactive vit b12 and 8% should be excreted in urine w/in 24 hoursbec. Not absorbed due to lack of intrinsic factor. Haemolytic: (sickle cell anemia) Erythrocytes have shortened life span; thus circulation is reduced Sickle cell anemia: -shape is biconcave(like xo candy)..so it can easily bypass capillaries -congenital haemolytic disease which= defective hemoglobulin molecule -shape becomes-slug like shapedeasily adheres to blood vessel wall. -there are painful attacks called sickle cell crisis -happens when child is dehydratedwhen child is lacking oxygen or experience respiratory acidosis -there will be tissue ischemia-because it sticks to blood vessels -at risk of infarction, swelling(fluid shifting) Asses. Findings -Cawlik pain -splenomegaly(spleen does the recycling of sickled rbcsrapid destruction of rbcs increase the size of the spleen=sequestration -hypvolemia- bec. Rbcs are destroyedlacking of blood cells in circulation leading to hypovolemia Pain- vasooclusiontisses are deprived of oxygenthere will be ischemia which results to pain. Polycythemia: -is the increase erythrocyte production above N range. -decrease plasma bec. Of increased rbc(dehydrated yng plasma) -blood will be viscos(patient is at risk of thrombosis because of blockage and can stick and clog a certain vessel can lead to stroke Nursing goal:observe for signs of thrombus formation -signs and symptoms-congestive heart failure , hemmorhage(bec. Of thrombus) patient at risk of shock bec. Of hemorrhage.( should ambulate patient to minimize possib. Of thrombosis) -protect from infection- bec. Of resistance to immunosuppressive agents

-assess resp. system bec. Of increased viscosity the heart is having a hard time pumpingwhich affects the lungs which can lead to resp. infection -nutritional management- if there is dehydration of plasma-increase fluid intake, limit food rich in vit. K and food rich in iron. Polycythemia vera: -increase in rbcs and haemoglobin -blood is concentrated due to dehydration -there may be chronic cardio pulmonary disease( blood is so viscus and adheres to lung pharencaima) Clinical manifestations: Weakness-bec. Of viscosity Headache-lack of oxygen Loss of weight and strength(because all systems are affected..blood can hardly circulate..lacking of oxygen to muscles ) Shortness of breath- impaired blood circulation to all systems in the body Itching-increased rbcs Visual disturbances- increased viscosity of blood Complications: Cerebral hemorrhage-bleeding from nose,mouth and g.i tract. Epistaxis=collapse of capillaries Dont give aspirine and treatment is phlebotomy