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Anemias




          Dr. Pablo Parenti
• La evaluación del hematocrito depende del
  volumen
  – Deshidratación
  – embarazo
Complicaciones
• Hipoxia
  – Shock
  – Hipotensión
  – Insuficiencia coronaria y pulmonar
Etiologías

Pérdida de sangre
Hemólisis
Producción
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
                                       Total Iron-
                                                           Bone Marrow
   Condition           Serum Iron   Binding Capacity                        Comment
                                                               Iron
                                         (TIBC)
Iron deficiency    ↓                ↑                  0                 Responsive to
                                                                         iron therapy
Chronic            ↓                ↓                  ++                Unresponsive to
inflammation                                                             iron therapy
Thalassemia        ↑                N                  ++++              Reticulocytosis
major                                                                    and indirect
                                                                         bilirubinemia
Thalassemia        N                N                  ++                Elevation of A of
minor                                                                    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) synthesis
                               Inherited 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 GR
Macrocyte     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   Drop-shaped erythrocyte, often microcytic. Myelofibrosis and infiltration of marrow with
RBCs          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
hemólisis
                                     Hereditary                         Acquired
Intracorpuscular defect   Hereditary spherocytosis         Vitamin B-12 and folic acid
                                                           deficiency

                          Hereditary elliptocytosis
                                                           Paroxysmal nocturnal
                                                           hemoglobinuria
                          Hemoglobinopathies

                                                           Severe iron deficiency
                          Thalassemias


                          Congenital dyserythropoietic
                          anemias


                          Hereditary RBC enzymatic
                          deficiencies


                          Rarer hereditary abnormalities
Hereditario   adquirido

Extracorpuscular                 Physical agents: Burns, cold exposure
defect
                                 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.
                                treatment of Von Willebrand disease.
Cryoprecipitate                 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         treat hemophilia A.
(Advate, Helixate FS, Xyntha)
Suplementos minerales
                              Mineral supplements are used to provide adequate iron for hemoglobin
Ferrous sulfate (MyKidz       synthesis and to replenish body stores of iron. Iron is administered
Iron 10, Fer-Iron, Slow-FE)   prophylactically during pregnancy because of the anticipated requirements of
                              the fetus and the losses that occur during delivery.



Carbonyl iron (Feosol, Iron   Csrbonyl iron is used as a substitute for ferrous sulfate. It has a slower release of
Chews, Icar)                  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,          Dextran-iron replenishes depleted iron stores in the bone marrow, where it is
Dexferrum)                    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

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

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

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        Essential for transmission of nerve impulses, contraction of cardiac muscle,
Chloride (K-     maintenance of intracellular tonicity, skeletal and smooth muscles, and
Tab, Klor-Con,   maintenance of normal renal function.
microK,          Gradual potassium depletion occurs via renal excretion, through GI loss or
Epiklor)         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    Vasopressin causes vasoconstriction of vascular smooth muscles and
(Pitressin)    increases water permeability and reabsorption in the collecting tubules.
               It decreases portal pressure in patients with portal hypertension.



Somatostatin   Somatostatin diminishes blood flow to the portal system due to
(Zecnil)       vasoconstriction, thus decreasing variceal bleeding.
                It has similar effects to vasopressin but does not cause coronary
               vasoconstriction.
Antihistamínicos H2

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

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

Famotidine          Famotidine competitively inhibits histamine at H2 receptor of gastric parietal
(Pepcid)            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.

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Understanding and Treating Anemias

  • 1. Anemias Dr. Pablo Parenti
  • 2. • La evaluación del hematocrito depende del volumen – Deshidratación – embarazo
  • 3. Complicaciones • Hipoxia – Shock – Hipotensión – Insuficiencia coronaria y pulmonar
  • 5. Genéticas • Hemoglobinopatías • Talasemias • Anormalidades enzimáticas • Defectos del citoesquelétdo del GR • Anemia diseritropoyética congénita • Enfermedad Rh • Xerocitosis hereditaria • Abetalipoproteinemia • Anemia de Fanconi
  • 6. Nutricionales • Hierro • Vitamina B-12 • Folato • Malnutrición
  • 7. Físicas • Trauma • Quemaduras • Congelamiento • Válvulas protésicas
  • 8. Neoplasias y enfermedades crónicas • Renal • Hepática • Infecciones crónicas • Neoplasias • Colagenopatías
  • 9. Infecciosas • Viral – Hepatitis, mononucleosis, citomegalovirus • Bacteriana – Clostridia, sepsis gram-negativo • Protozoos – Malaria, leshmaniasis, toxoplasmosis
  • 10. Factores demográficos • Geográficas • Raciales • Género • Edad
  • 11. 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
  • 12. Examen físico • Aspecto general • Piel • Ojo • Adenopatías y edemas • Hepatoesplenomegalia • Recto y pelvis • Sistema nervioso • Aparato cardiovascular
  • 13. Hb g/dl OMS USA Adultos 12.5 13.5 Femeninos 12.5 6m-6a 11 6ª-14a 12
  • 15. Hipocrómica microcítica Total Iron- Bone Marrow Condition Serum Iron Binding Capacity Comment Iron (TIBC) Iron deficiency ↓ ↑ 0 Responsive to iron therapy Chronic ↓ ↓ ++ Unresponsive to inflammation iron therapy Thalassemia ↑ N ++++ Reticulocytosis major and indirect bilirubinemia Thalassemia N N ++ Elevation of A of minor 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
  • 16. Macrocitosis (MCV >95) Megaloblastic bone marrow Deficiency of vitamin B-12 Deficiency of folic acid Drugs affecting deoxyribonucleic acid (DNA) synthesis Inherited disorders of DNA synthesis Nonmegaloblastic bone marrow Liver disease Hypothyroidism and hypopituitarism Accelerated erythropoiesis (reticulocytes) Hypoplastic and aplastic anemia Infiltrated bone marrow
  • 17. Alteración en la forma GR Macrocyte 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 Drop-shaped erythrocyte, often microcytic. Myelofibrosis and infiltration of marrow with RBCs 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
  • 19. hemólisis Hereditary Acquired Intracorpuscular defect Hereditary spherocytosis Vitamin B-12 and folic acid deficiency Hereditary elliptocytosis Paroxysmal nocturnal hemoglobinuria Hemoglobinopathies Severe iron deficiency Thalassemias Congenital dyserythropoietic anemias Hereditary RBC enzymatic deficiencies Rarer hereditary abnormalities
  • 20. Hereditario adquirido Extracorpuscular Physical agents: Burns, cold exposure defect 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)
  • 21.
  • 22. Exámenes complementarios • Imágenes • Endoscopías digestivas y RX • Medula osea
  • 24. Tratamiento • Transfusión • Sulfato ferroso • Nutrición (hierro, vit B12, Ac fólico) • Aplasias • Esplenectomía • Transplante MO y células madre
  • 25. 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. treatment of Von Willebrand disease. Cryoprecipitate 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 treat hemophilia A. (Advate, Helixate FS, Xyntha)
  • 26. Suplementos minerales Mineral supplements are used to provide adequate iron for hemoglobin Ferrous sulfate (MyKidz synthesis and to replenish body stores of iron. Iron is administered Iron 10, Fer-Iron, Slow-FE) prophylactically during pregnancy because of the anticipated requirements of the fetus and the losses that occur during delivery. Carbonyl iron (Feosol, Iron Csrbonyl iron is used as a substitute for ferrous sulfate. It has a slower release of Chews, Icar) 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, Dextran-iron replenishes depleted iron stores in the bone marrow, where it is Dexferrum) 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.
  • 27. Vitaminas Cyanocobalam Deoxyadenosylcobalamin and hydroxocobalamin are active forms of vitamin in ( Calo-Mist, B12 in humans. Ener-B, Microbes synthesize vitamin B12, but humans and plants do not. Nascobal) Vitamin B12 deficiency may result from intrinsic factor (IF) deficiency (pernicious anemia), partial or total gastrectomy, or diseases of the distal ileum. Folic acid is an essential cofactor for enzymes used in the production of red Folic acid blood cells (RBCs). (Folvite) 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).
  • 28. electrolítos Potassium Essential for transmission of nerve impulses, contraction of cardiac muscle, Chloride (K- maintenance of intracellular tonicity, skeletal and smooth muscles, and Tab, Klor-Con, maintenance of normal renal function. microK, Gradual potassium depletion occurs via renal excretion, through GI loss or Epiklor) 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.
  • 29. vasopresores Vasopressin Vasopressin causes vasoconstriction of vascular smooth muscles and (Pitressin) increases water permeability and reabsorption in the collecting tubules. It decreases portal pressure in patients with portal hypertension. Somatostatin Somatostatin diminishes blood flow to the portal system due to (Zecnil) vasoconstriction, thus decreasing variceal bleeding. It has similar effects to vasopressin but does not cause coronary vasoconstriction.
  • 30. Antihistamínicos H2 Cimetidine The primary indication is to reduce symptoms and accelerate healing of (Tagamet) gastric ulcers. In the acutely bleeding patient, it has limited benefit. Ranitidine Ranitidine inhibits histamine stimulation of the H2 receptor in gastric parietal (Zantac) cells, which, in turn, reduces gastric acid secretion, gastric volume, and hydrogen ion concentrations. Famotidine Famotidine competitively inhibits histamine at H2 receptor of gastric parietal (Pepcid) 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.
  • 31. 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.