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Dr. Judit Demeter Semmelweis Egyetem ÁOK., I.sz. Belgyógyászati Klinika

Anaemias I Iron deficiency anaemia, ACD. Dr. Judit Demeter Semmelweis Egyetem ÁOK., I.sz. Belgyógyászati Klinika. History taking in patients with h aematological disease 1. History taking in patients with h aematological disease 2. Normal RBC és PLT values (adult).

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Dr. Judit Demeter Semmelweis Egyetem ÁOK., I.sz. Belgyógyászati Klinika

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  1. Anaemias I Iron deficiency anaemia, ACD Dr. Judit Demeter Semmelweis Egyetem ÁOK., I.sz. Belgyógyászati Klinika

  2. History taking in patients with haematological disease 1.

  3. History taking in patients with haematological disease 2.

  4. Normal RBC és PLT values (adult)

  5. Normal peripheral blood smear, thin, homogenous part

  6. Normal RBC morphology

  7. Red blood cell maturation 1. 2. 3. 4. 5. 6. 1.: proerythroblast; 4.: oxyphil normoblast; 2.: basophil normoblast; 5.: polychromatophil RBC; 3.: polychromatophil normoblast; 6.: mature RBC

  8. Anaemias: Kinetic approach Reduced RBC production deficiency anaemias (hematinic deficiency) (iron, B12, folic acid) bone marrow disorders ( aplastic anaemia, isolated RBC-aplasia, myelodsyplasia, neoplastic infiltration) Increased RBC destruction (hemolysis: RBC lifespan < 100 days) congenital hemolitic anaemias (hereditary shaerocytosis, sickle cell disease, thalassaemia maior) acquired hemolytic anaemias (Coombs positiv hemolytic anaemia TTP-HUS) Blood loss apparent bleeding - hematemesis, melaena, metrorrhagia, epistaxis occult bleeding - slowly bleeding ulcus or carcinoma. Az anemiás beteg

  9. Examination of the anaemic patient • searching for signs of bleeding (at present or earlier) • Signs of increased RBC destruction ( hemolysis?) • Bone marrow suppression? • Iron deficiency? Why? • Folic acid or B12 deficiency?

  10. Examination of the anamic patient II Medical history melaena? since what time? drug intake? (NSAID-s) geographical localisation? Physical examination severity ? tachycardia, dyspnoe, fever, hypotension hepatosplenomegaly, lymphadenopathy? (pallor) icterus? petechiae? Infections?

  11. Anaemia in pregnancy • Dilutional anaemia: red cell mass ↑ by ~ 30% plasma volume ↑ by ~ 60% The normal Hb concentration: 10-11g/dl • Iron deficiency is a common problem

  12. Reticulocytes in the peripheral blood smear – brillantcresilblue staining

  13. Retikulocyte shift in anaemia With increasing severity of the anaemia and increasing erythropoetic stimulation, the reticulocytes leave BM increasingly earlier . Thus their maturation time in theperiphery increases from 1 to 3 days.

  14. Polychromatophilia in reticulocytosis A retiukulociták nagyobbak és nincs centrális halvány középük (nem bikonkávak)

  15. Iron homeostasis The amount of iron passing from mucosal cells into the body is determined by the rate of erythropoiesis and the state of body iron stores.

  16. Anaemia is typically the first clue to iron deficiency, but an isolated haemoglobin measurement has both low specificity and low sensitivity. • - improve sensitivity by • including measures of iron-deficient erythropoiesis such as • - the transferrin iron saturation, • - mean corpuscular haemoglobin concentration, - erythrocyte zinc protoporphyrin, • - percentage of hypochromic erythrocytes or • - reticulocyte haemoglobin concentration. • However, these results changes in iron def. indistinguishable from ACD. • The optimal dg: • serum ferritin as an index of iron stores and • serum transferrin receptor as a index of tissue iron deficiency.

  17. Testing for iron-deficiency Receiver operating characteristic (ROC) curves showing the sensitivity and specificity of various tests for the diagnosis of iron deficiency [ferritin, red cell protoporphyrin, transferrin saturation, mean red cell volume (MCV), and red cell volume distribution width (RDW)]. Note that, at any given level of sensitivity or specificity, serum ferritin outperforms all the other tests shown

  18. Hypochromasia, anulocytes

  19. Causes of iron deficiency

  20. Blood loss in gastrointestinal disease

  21. Control of iron absorption Pathways of iron absorption and excretion in an intestinal mucosal cell. Normal iron absorption is 1 mg/day with a total daily intake of 10 mg. Iron absorption: enhanced in iron deficiency, and diminished in iron overload.

  22. Factors influencing the absorption of dietary iron Absorption of heme iron Amount of heme iron, especially in meat Content of calcium in the meal Food preparation (time, temperature) Absorption of nonheme iron Iron status Amount of potentially available nonheme iron Balance between positive and negative factors Positive factors Ascorbic acid Meat or fish Negative factors Phytate (in bran, oats, rye fiber) Polyphenols (in tea, some vegetables and cereals) Dietary calcium Soy protein

  23. Role of specific proteins in iron metabolism • Transferrin (Tf) • Transferrin receptor (TfR) • Ferritin • Iron regulatory protein 1 and 2 (IRP1 and IRP2), the cellular iron sensing proteins • Divalent metal transporter 1 (DMT1, Nramp2, DCT1, Solute carrier family 11, member 2 (Slc11a2)), the duodenal iron transporter • Ferroportin (Ireg1, Slc11a2, Mtp1), the cellular iron exporter • Hephaestin, which likely cooperates with ferroportin for exporting iron to transferrin. • HFE, mutations responsible for the common form of hereditary hemochromatosis • TFR2, mutations responsible for a rare form of hereditary hemochromatosis • Hemojuvelin, a hepcidin regulator, mutations responsible for the common form of juvenile hemochromatosis • Hepcidin, the key negative regulator of • intestinal iron absorption + macrophage iron release. • Mutations cause a rare form of juvenile hemochromatosis.

  24. Some truths’ about ferritin — the cellular storage protein for iron • - a huge protein, consisting of light and heavy chains, which can store up to 4500 atoms of iron within its spherical cavity • an acute phase reactant, and, along with transferrin and the transferrin receptor, is a member of the protein family that orchestrates cellular defense against oxidative stress and inflammation • Much of the stored iron is accessible for metabolic needs. Ferritin within erythroid precursors may be of special importance in the donation of iron for heme synthesis, • Ferritin measured clinically in plasma is usually apoferritin, a non-iron containing molecule. • The plasma level generally reflects overall iron storage • 1 ng of ferritin per mL → 10 mg of total iron stores. • A normal adult male: plasma ferritin level of • 50 to 100 ng/mL = iron stores of appr. 500 to 1000 mg • A serum ferritin <10 to 15 ng/mL is 99 percent specific for making a diagnosis of iron deficiency. • An elevated serum ferritin ( in the absence of infection or inflammation) suggests the presence of an iron overload state.

  25. Distribution of body iron in men and women 70 kg man 60 kg woman _________ ___________ Iron stores - transferrin, ferritin, hemosiderin 1.4 g 0.3 g* Hemoglobin 2.5 g 1.9 g Myoglobin 0.14 g 0.13 g Heme enzymes 0.01 g 0.01g _______________________________________________________________ TOTAL4.05 g 2.34 g

  26. Laboratory tests in iron deficiency of increasing severity

  27. KN born: 1961 2007 Abdominal cramps, bloody stool, colonoscopy : M Crohn 2009 aug prolapsus recti Status: pallor no perimalleolar anasarca no tachycardia, but systolic murmur RDV: stool Weber: + FBC: Ht: 0,18 Hb: 54 g/l ret: 35 % MCV: 65 fl (n: 80-97) WBC : norm CRP: 0 Plt : 582 G/l INR norm serumiron: 3,0 TIBC: 87,8 serum folic acid, serum B12 : normal soluble transferrin receptor :27,6 (norm: 1,9- 4,4) serumferritin: 7 ng/ml (norm: 10-120)

  28. Normal curve Mikrocytosis Cell-Dyn 3500

  29. Target RBCs, slight hypochromasia

  30. Target RBCs in thalassaemia minor

  31. Anisocytosis, microcytosis in irondeficiency

  32. Anulocytes, hypochromasia in iron-deficiency

  33. Anaemias – morphological approach

  34. Change of RBC indices in the course of successful iron treatment 1999.10.01: vvs : 4.16 Hgb :72 Ht : 25 MCV:61 1999.10.01: vvs : 4.16 Hgb :72 Ht : 25 MCV:61 1999.10.30: vvs : 4.56 Hgb :107 Ht : 34 MCV:74 1999.10.30: vvs : 4.56 Hgb :107 Ht : 34 MCV:74 1999.12.10: vvs : 4.87 Hgb :125 Ht : 39 MCV:79 1999.12.10: vvs : 4.87 Hgb :125 Ht : 39 MCV:79 2000.04.19: vvs : 4.83 Hgb :138 Ht : 41 MCV:86 2000.04.19: vvs : 4.83 Hgb :138 Ht : 41 MCV:86

  35. Causes of failure to respond to oral iron therapy Coexisting disease interfering with marrow response Infection Inflammatory disorder (eg, rheumatoid arthritis) Concomitant malignancy Coexisting folic acid and/or vitamin B12 deficiency Bone marrow suppression from another cause Dg. incorrect, diff. diagnosis Thalassemia Lead poisoning Anemia of chronic disease (anemia of chronic inflammation) Copper deficiency (zinc toxicity) Myelodysplastic syndrome/refractory sideroblastic anemia Patient not taking the medication Medication is being taken but is not being absorbed cont’d

  36. Cont. Medication is being taken but is not being absorbed Enteric coated product: coating is not dissolving Malabsorption of iron (eg, sprue, atrophic gastritis) Agents interfering with absorption (eg, antacids, tetracycline, tea) Continued blood loss -Cause treatable (eg, bleeding peptic ulcer) -Cause not treatable (eg, Osler Weber Rendu disease) or not by oral iron (eg, renal failure responding to Epo)

  37. CALCULATION OF PARENTERAL IRON DOSE Body weight (kilograms) = BW68 kg Hemoglobin concentration (g/dL) = Hgb5,5 g/dl Concentration of elemental iron in the parenteral product (mg/mL) = C*12,5 mg/ml Assumptions: Blood volume is 65 mL per kilogram Hemoglobin concentration to be corrected to 14.0 g/dL No additional iron to be given for repletion of body stores Intermediate calculations: Blood volume (dL) = 65 (mL/kg) x body weight (kg) ÷ 100 (mL/dL)65x68/100=44,2 dl Hemoglobin deficit (g/dL) = 14.0 - patient hemoglobin conc.8,5 g/dl Hemoglobin deficit (g) = hemoglobin deficit (g/dL) x blood volume (dL)8,5x44,2=375 g Iron deficit (mg) = hemoglobin deficit (g) x 3.3 (mg Fe/g Hgb)375x3.3=1237,5 mg Volume of parenteral iron product required (mL) = Iron deficit (mg) ÷ C(mg/mL)99 ml= 20 ampulla Final calculations: Hemoglobin iron deficit (mg) = BW x (14 - Hgb) x (2.145) Volume of product required (mL) = BW x (14 - Hgb) x (2.145) ÷ C

  38. Anaemia of chronic diseases (ACD)

  39. Anemia of chronic disease - bone marrow iron stain

  40. Anaemia in rheumatoid disease

  41. Determination of soluble transferrin receptor irondeficiency ACD: Anaemia of chronic disorders Combined causes Normal

  42. Myelodysplastic syndrom- refractory anaemia with ring sideroblasts

  43. GUIDELINES FOR RED CELL TRANSFUSIONS IN ADULT Need based on estimation of lost blood volume: Ø Need based on hemoglobin concentration: Hgb <7 g/dL: RBC transfusion indicated. If the patient is otherwise stable, the patient should receive 2 units of packed RBC, following which the patient's clinical status and circulating HgB should be reassessed Hgb 7 to 10 g/dL: Correct strategy is unclear Hgb >10 g/dL: RBC transfusion not indicated High risk patients: Patients >65 and/or those with cardiovascular or respiratory disease may tolerate anemia poorly. Such patients may be transfused when Hgb <8 g/dL.

  44. reticulocyta Microcytás an. Hemolizis Microcytás an.E Fe n/ TVK n/  ferritin  Lép nem tap Vashiányos an. Krónikus betegséget kísérő anaemia Ólommérgezés

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