Hematology Notes PDF

Mrs. Danielle Damianos, MSN, RN AUB-HSON  Hemopoiesis production of formed elements of blood-occurs  Blood plasma 90% water, 6-8 % protein, albumin, gamaglobulin, fibrinogen, prothrombin. 1-2 % glucose, aminoacids & hormones  Cellular components Erythrocytes(RBC)...

Mrs. Danielle Damianos, MSN, RN AUB-HSON  Hemopoiesis production of formed elements of blood-occurs  Blood plasma 90% water, 6-8 % protein, albumin, gamaglobulin, fibrinogen, prothrombin. 1-2 % glucose, aminoacids & hormones  Cellular components Erythrocytes(RBC) Leukocytes(WBC) Thrombocytes (Platelets)  Review compo th e ne blood f nts of ro m th book e  The formation of blood cells begins in: - The fetal yolk sac as early as week 2 of intrauterine life - Month 2 of intrauterine life, the liver and spleen begin forming blood component - Month 4 of intrauterine life, the bone marrow becomes and remains the active center for the origination of blood cells - In extrauterine life, the spleen serves as the organ for destruction of blood cells once their normal life span has passed  Anemia Clinical symptoms appear when Hb =7 to 8 g/ml in nb of erythrocytes Production < Destruction Acute blood loss anemia Anemia of acute infection Anemia of renal disease Anemia of neoplastic disease Hypersplenism Congenital (Fanconi) Aplastic anemia Acquired (radiation, drugs, chemical) Hemolytic anemia  History  national heritage  family history  Nutrition  Physical examination  Lab tests  CBC, reticulocyte count, peripheral smear  MCV & MCH  Lab. levels  Supply the missing element (blood transfusion, Packed cells, or platelets.)  Prevent infection  Give iron supplements  Splenectomy  Bone marrow transplant  Stem Cell transplantation  Quite rare inherited as autosomal recessive  The child is born with several congenital anomalies, such as skeletal and renal abnormalities, hypogenitalism, and short stature  4 -12 years of age, a child begins to manifest symptoms of pancytopenia: reduction of all blood cell components  Treatment: Supportive, transfusions, Bone Marrow Transplant ,75% die.  Iron Deficiency Anemia The most common anemia of infancy and childhood, occurs when the intake of dietary iron is inadequate  Hb synthesis is impaired and the RBC are pale and small. serum ferratin of less 10mg/ml and Hb less than 11 Necessary daily intake of 6 to 15 mg of iron Most often between ages 9 months and 3 years Rises again in adolescence: Menstruating girls. overweight teenagers if they ingest more carbohydrates than iron- rich foods  Causes Lack of good diet Rapid growth Increase milk intake with decreased food intake. More common in premise ( iron stores appear in the last weeks of gestation) Low SES families GI lesions such as polyps, ulcerative colitis, Crohn’s disease, parasitic or frequent epistaxis Adequate diet: breastfed start mom has to stay on prenatal vitamins Cereal at 4-6 months, if not baby receives 2-3 mg/kg/day, iron-fortified formula Preterm should start on iron at 2 months 2-3mg/kg/day until 12-18 months.  with vitamin C Treat underlying cause  minimize the child’s activities to prevent fatigue  Teach about food rich in iron. (Lentils, Parsley & red meat.)  Substitute meat with cheese, eggs, green vegetables, or fortified cereal.  reminder sheets so they can manage to give the supplement over a long period  Deficiency in the enzyme G6PD carried by the RBC  more common in Mediterranean people  Sex linked transmission, on X chromosome. It shows with males.  Occurs after ingesting fava beans, sulfonamides, aspirin, Benadryl, vit C.  Blood hemolysis,  Jaundice, back pain, fever, hematuria  TR: Avoid triggers! Teaching,  There is deficiency in the G6PD enzyme, so there is not enough Gluthatione to fight the free radicals that are harmful molecules & can damage RBC under certain conditions.  Sickle Cells Anemia  Hereditary- autosomal recessive mode  Abnormal Hb in RBC called HbS( has valine amino acid in the sixth position of the beta polypeptide chain instead of glutamic acid)  Pathophysiology & Clinical Manifestations 1. Increase blood viscosity, stasis of blood 2. Increase RBC destruction  Assessment Hemoglobin electrophoresis is used to diagnose sickle-cell anemia at birth 6 months of age, signs of fever and anemia Swelling of the hands and feet (a hand-foot syndrome) Slight build and characteristically long arms and legs. Protruding abdomen: enlarged spleen and liver. An atrophic spleen: repeated infarctions  Susceptible to infections Acute chest syndrome: pulmonary infiltrates (chest pain, fever, tachypnea, wheezing, or cough ) Pneumonia  A sickle-cell crisis can occur when a child has dehydration or a respiratory infection that results in lowered oxygen exchange and a lowered arterial oxygen level, or after extremely strenuous exercise  Symptoms are sudden, severe, and painful  Complications: Aseptic necrosis of the head of the femur or humerus with increased joint pain or a cerebrovascular accident that occurs from a blocked artery, causing loss of motor function, coma, seizures, or even death  Treatment  Family Counseling  Prevent sickling  Treat sickle cell crisis Pain MGT O2 Hydration  Gene therapy, exchange transfusion, anti-platelet therapy  Correct acidosis by electrolytes. No K until kidney functions checked  Hydroxyurea, an antineoplastic agent increases the production of hemoglobin F (Anorexia)  prophylactic antibiotics and pneumococcal vaccine  Autosomal recessive inheritance  Alpha or beta chain Hb syntheses is impaired  Mediterranean anemia.  Symptoms begin at 6 months, child’s fetal Hb is replaced by adult Hb.  Change in the shape of the skull; Protrusion of upper teeth & marked malocclusion; base of nose is broad & flattened; eyes are slanted.  Protruded abdomen, splenomegaly  Transfusion: maintain Hb >10 transfuse q 2-3 weeks.  Deferoxamine therapy (iron chelating agent over 8 hours at least 5days a week.  Splenectomy indicated when transfusions exceed 200-250 mL/kg of body Wt. Vaccines & Antibiotics  BMT indicated in the low risk child with compatible donor  Genetic counseling Overall prognosis is improving but is still grave. Most Pt. die from cardiac failure as young adults or adolescence.  Diabetes Mellitus (deposition of iron in the pancreas  Myocardiopathy (heart failure due to circulatory overload)  Growth Failure  Delayed puberty Most are from deficiencies in vitamin K or from deficient coagulation factors Many infants are born with bruises More serious disorders are not found till later Disorders of Blood Coagulation (Deficiency in one of the clotting factors)  Deficiency in one of the clotting factors (factor VIII deficiency is in75% of cases)  X-linked inheritance shows in males  Prolonged bleeding (circumcision)  bruising, intracranial & GI bleed, Joint bleeding Platelets & PT time is normal PTT, Clotting time abnormal  Apply cold compressors.  Replacement factor, Cryoprecipitate, or factor VIII deficiency.  Splinting to prevent bleeding into joints  Accidents such as head trauma need careful assessment  Fresh Frozen plasma when type of hemophilia is not known  Parental teaching have a card “I have Hemophilia and this factor is missing.”  Avoid Taking Aspirin