Anemias

Dr. Pablo Parenti

• La evaluación del hematocrito depende del volumen– Deshidratación– embarazo

Complicaciones

• Hipoxia– Shock– Hipotensión– Insuficiencia coronaria y pulmonar

Etiologías

Pérdida de sangreHemólisisProducción

Genéticas

• Hemoglobinopatías• Talasemias• Anormalidades enzimáticas• Defectos del citoesquelétdo del GR• Anemia diseritropoyética congénita• Enfermedad Rh• Xerocitosis hereditaria• Abetalipoproteinemia• Anemia de Fanconi

Nutricionales

• Hierro• Vitamina B-12• Folato• Malnutrición

Físicas

• Trauma• Quemaduras• Congelamiento• Válvulas protésicas

Neoplasias y enfermedades crónicas

• Renal • Hepática• Infecciones crónicas• Neoplasias• Colagenopatías

Infecciosas

• Viral – Hepatitis, mononucleosis, citomegalovirus

• Bacteriana– Clostridia, sepsis gram-negativo

• Protozoos– Malaria, leshmaniasis, toxoplasmosis

Factores demográficos

• Geográficas• Raciales• Género• Edad

Anamnesis

• Duración anemia• Historia familiar• Ocupación, hábitos, exposición, drogas.• Historia gineco-obstétrica• Síntomas gastrointestinales• Nutrición, deficiencias vitamínicas• Fiebre, lesiones piel, enfermedades metabólicas,

colagenopatías• Orina• Anamnensis por aparatos

Examen físico

• Aspecto general• Piel• Ojo• Adenopatías y edemas• Hepatoesplenomegalia• Recto y pelvis• Sistema nervioso• Aparato cardiovascular

Hb g/dl OMS USA

Adultos 12.5 13.5

Femeninos 12.5

6m-6a 11

6ª-14a 12

Frotis

Hipocrómica microcítica Condition Serum Iron

Total Iron-Binding Capacity

(TIBC)Bone Marrow

Iron Comment

Iron deficiency ↓ ↑ 0 Responsive to iron therapy

Chronic inflammation

↓ ↓ ++ Unresponsive to iron therapy

Thalassemia major

↑ N ++++ Reticulocytosis and indirect bilirubinemia

Thalassemia minor

N N ++ Elevation of A of fetal hemoglobin, target cells, and poikilocytosis

Lead poisoning N N ++ Basophilic stippling of RBCs

Sideroblastic ↑ N ++++ Ring sideroblasts in marrow

Hemoglobin N N ++ Hemoglobin electrophoresis

Macrocitosis (MCV >95) Megaloblastic bone marrow Deficiency of vitamin B-12

Deficiency of folic acid

Drugs affecting deoxyribonucleic acid (DNA) synthesisInherited disorders of DNA synthesis

Nonmegaloblastic bone marrow Liver disease

Hypothyroidism and hypopituitarism

Accelerated erythropoiesis (reticulocytes)

Hypoplastic and aplastic anemia

Infiltrated bone marrow

Alteración en la forma GRMacrocyte Larger than normal (>8.5 µm diameter). See Table 2.

Microcyte Smaller than normal (< 7 µm diameter). See Table 1.

Hypochromic Less hemoglobin in cell. Enlarged area of central pallor. See Table 1.

Spherocyte Loss of central pallor, stains more densely, often microcytic. Hereditary spherocytosis and certain acquired hemolytic anemias

Target cell Hypochromic with central "target" of hemoglobin. Liver disease, thalassemia, hemoglobin D, and postsplenectomy

Leptocyte Hypochromic cell with a normal diameter and decreased MCV. Thalassemia

Elliptocyte Oval to cigar shaped. Hereditary elliptocytosis, certain anemias (particularly vitamin B-12 and folate deficiency)

Schistocyte Fragmented helmet- or triangular-shaped RBCs. Microangiopathic anemia, artificial heart valves, uremia, and malignant hypertension

Stomatocyte Slitlike area of central pallor in erythrocyte. Liver disease, acute alcoholism, malignancies, hereditary stomatocytosis, and artifact

Tear-shaped RBCs

Drop-shaped erythrocyte, often microcytic. Myelofibrosis and infiltration of marrow with tumor. Thalassemia

Acanthocyte Five to 10 spicules of various lengths and at irregular intervals on surface of RBCs

Echinocyte Evenly distributed spicules on surface of RBCs, usually 10-30. Uremia, peptic ulcer, gastric carcinoma, pyruvic kinase deficiency, and preparative artifact

Sickle cell Elongated cell with pointed ends. Hemoglobin S and certain types of hemoglobin C and l

evaluación

• Pérdida

hemólisisHereditary Acquired

Intracorpuscular defect Hereditary spherocytosis

Hereditary elliptocytosis

Hemoglobinopathies

Thalassemias

Congenital dyserythropoietic anemias

Hereditary RBC enzymatic deficiencies

Rarer hereditary abnormalities

Vitamin B-12 and folic acid deficiency

Paroxysmal nocturnal hemoglobinuria

Severe iron deficiency

Hereditario adquirido

Extracorpuscular defect

Physical agents: Burns, cold exposure

Traumatic: Prosthetic heart valves, march hemoglobinemia, disseminated intravascular coagulation (DIC), graft rejection

Chemicals: Drugs and venoms

Infectious agents: Malaria, toxoplasmosis, mononucleosis, hepatitis, primary atypical pneumonia, clostridial infections, bartonellosis, leishmaniasis

Hepatic and renal disease

Collagen vascular disease

Malignancies: Particularly hematologic neoplasia

Transfusion of incompatible blood

Hemolytic disease of the newborn

Cold hemagglutinin

disease

Autoimmune hemolytic anemia Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS)

Exámenes complementarios

• Imágenes

• Endoscopías digestivas y RX

• Medula osea

Tratamiento

Tratamiento

• Transfusión • Sulfato ferroso• Nutrición (hierro, vit B12, Ac fólico)• Aplasias• Esplenectomía• Transplante MO y células madre

Productos sanguineos

Fresh frozen plasma

coagulation factors, as well as protein C and protein S.treatment of coagulopathies and TTP and the reversal of Coumadin. FFP does not transmit infections.

Cryoprecipitate

treatment of Von Willebrand disease. It contains fibrinogen, factor VIII, and von Willebrand factor and can be used in lieu of factor VIII concentrate if the latter is unavailable.

Platelets

Patients who are thrombocytopenic and have clinical evidence of bleeding should receive a platelet transfusion. Patients with platelet counts of less than 10,000/mcL are at risk for spontaneous cerebral hemorrhage and require a prophylactic transfusion.The preferred treatment for TTP and hemolytic-uremic syndrome is large-volume plasmapheresis with FFP replacement. ITP is rarely treated with transfusion, as the transfused platelets are destroyed rapidly. In stable patients, initial treatment is with prednisone. High-dose immunoglobulin and splenectomy are very effective treatments.

Factor IX (BeneFix, Mononine)

Hemophilia B is treated with factor IX concentrate. Recombinant factor IX currently is undergoing clinical trials (the current treatment is FFP or prothrombin-rich plasma concentrate).

Recombinant factor VIII (Advate, Helixate FS, Xyntha)

treat hemophilia A.

Suplementos minerales

Ferrous sulfate (MyKidz Iron 10, Fer-Iron, Slow-FE)

Mineral supplements are used to provide adequate iron for hemoglobin synthesis and to replenish body stores of iron. Iron is administered prophylactically during pregnancy because of the anticipated requirements of the fetus and the losses that occur during delivery.

Carbonyl iron (Feosol, Iron Chews, Icar)

Csrbonyl iron is used as a substitute for ferrous sulfate. It has a slower release of iron and is more expensive than ferrous sulfate. The slower release affords the agent greater safety if ingested by children. On a milligram-for-milligram basis, it is 70% as efficacious as ferrous sulfate. Claims are made that there is less gastrointestinal (GI) toxicity, prompting use when ferrous salts are producing intestinal symptoms and in patients with peptic ulcers and gastritis. Tablets are available containing 45 mg and 60 mg of iron.

Dextran-iron (INFeD, Dexferrum)

Dextran-iron replenishes depleted iron stores in the bone marrow, where it is incorporated into hemoglobin. Parenteral use of iron-carbohydrate complexes has caused anaphylactic reactions, and its use should be restricted to patients with an established diagnosis of iron deficiency anemia whose anemia is not corrected with oral therapy.The required dose can be calculated (3.5 mg iron/g of hemoglobin) or obtained from tables in the Physician's Desk Reference. For intravenous (IV) use, this agent may be diluted in 0.9% sterile saline. Do not add to solutions containing medications or parenteral nutrition solutions.

Vitaminas

Cyanocobalamin ( Calo-Mist, Ener-B, Nascobal)

Deoxyadenosylcobalamin and hydroxocobalamin are active forms of vitamin B12 in humans. Microbes synthesize vitamin B12, but humans and plants do not.Vitamin B12 deficiency may result from intrinsic factor (IF) deficiency (pernicious anemia), partial or total gastrectomy, or diseases of the distal ileum.

Folic acid (Folvite)

Folic acid is an essential cofactor for enzymes used in the production of red blood cells (RBCs).

Vitamin K

A decrease in levels of vitamin K–dependent factors (II, VII, IX, X, protein C, protein S) can lead to bleeding. Vitamin K is also used to treat hemorrhagic disease of the newborn, Coumadin-induced bleeding, and hypothrombinemia from other causes (eg, antibiotic, aspirin).

electrolítos

Potassium Chloride (K-Tab, Klor-Con, microK, Epiklor)

Essential for transmission of nerve impulses, contraction of cardiac muscle, maintenance of intracellular tonicity, skeletal and smooth muscles, and maintenance of normal renal function. Gradual potassium depletion occurs via renal excretion, through GI loss or because of low intake.Depletion usually results from diuretic therapy, primary or secondary hyperaldosteronism, diabetic ketoacidosis, severe diarrhea, if associated with vomiting, or inadequate replacement during prolonged parenteral nutrition.Potassium depletion sufficient to cause 1 mEq/L drop in serum potassium requires a loss of about 100 to 200 mEq of potassium from the total body store.

vasopresores

Vasopressin (Pitressin)

Vasopressin causes vasoconstriction of vascular smooth muscles and increases water permeability and reabsorption in the collecting tubules. It decreases portal pressure in patients with portal hypertension.

Somatostatin (Zecnil)

Somatostatin diminishes blood flow to the portal system due to vasoconstriction, thus decreasing variceal bleeding. It has similar effects to vasopressin but does not cause coronary vasoconstriction.

Antihistamínicos H2

Cimetidine (Tagamet)

The primary indication is to reduce symptoms and accelerate healing of gastric ulcers. In the acutely bleeding patient, it has limited benefit.

Ranitidine (Zantac)

Ranitidine inhibits histamine stimulation of the H2 receptor in gastric parietal cells, which, in turn, reduces gastric acid secretion, gastric volume, and hydrogen ion concentrations.

Famotidine (Pepcid)

Famotidine competitively inhibits histamine at H2 receptor of gastric parietal cells, resulting in reduced gastric acid secretion, gastric volume, and hydrogen ion concentrations.

Nizatidine (Axid)

Nizatidine competitively inhibits histamine at the H2 receptor of the gastric parietal cells, resulting in reduced gastric acid secretion, gastric volume, and reduced hydrogen concentrations.

Glucocorticoides

Prednisone

Glucocorticoids inhibit phagocytosis of antibody-covered platelets. Treatment of ITP during pregnancy is conservative unless the condition is severe. For severe cases, use the lowest dose of glucocorticoids. In neonates, if the platelet count drops below 50,000-75,000 platelets/µL, consider prednisone and exchange transfusions and immunoglobulin.