بسم الله الرحمن الرحيم

ANEMIA Dr Mokarian ICM 83/2/7

PROPERTIES OF THE HUMAN HEMOGLOBINS Dr mokarian PROPERTIES OF THE HUMAN HEMOGLOBINS HEMOGLOBIN STRUCTURE: 1- a tetramer of globin polypeptide chains A pair of a-like chains 141 amino acids long A pair of b-like chains 146 amino acids long HbA( a2b2) HbF (a2g2) HbA2 (a2d2) 2-single heme moiety / every globin chain heme=protoporphyrin IX ring +a single iron atom in the ferrous state (Fe2+): every molecule of hemoglobin can thus transport up to four oxygen molecules

PROPERTIES OF THE HUMAN HEMOGLOBINS Dr mokarian PROPERTIES OF THE HUMAN HEMOGLOBINS FUNCTION OF HEMOGLOBIN: hemoglobin must bind O2 efficiently at the partial pressure of oxygen (PO2) of the alveolus, retain it, and release it to tissues at the PO2 of tissue capillary beds.

Dr mokarian

HbA has a reasonably high affinity for 2,3-BPG. Dr mokarian The Bohr effect arises from the stabilizing action of protons on deoxyhemoglobin, which binds protons more readily than oxyhemoglobin because it is a weaker acid. HbA has a reasonably high affinity for 2,3-BPG. HbF does not bind 2,3-BPG, so it tends to have a higher oxygen affinity in vivo.

HEMOGLOBIN STRUCTURE: Dr mokarian HEMOGLOBIN STRUCTURE:

DEVELOPMENTAL BIOLOGY Dr mokarian DEVELOPMENTAL BIOLOGY first appearing at about 6 weeks after conception HbPortland (z2g2), Hb Gower I (z2e2), and Hb Gower II (a2e2) At 10 to 11 weeks, fetal hemoglobin (HbF; a2g2) becomes predominant. The switch to nearly exclusive synthesis of adult hemoglobin (HbA; a2b2) occurs at about 38 weeks Fetuses and newborns therefore require a-globin but not b-globin for normal gestation.

CLINICAL PRESENTATION OF ANEMIA Anemia is most often recognized by abnormal screening laboratory tests. the gradual onset of anemia ,particularly in young patients,may not be associated with signs or symptoms until the anemia is severe [Hemoglobin <70 to 80 g/L (7 to 8 g/dL)] DIFINATION : MALE Hb< 14-16 Mg/dl FEMALE Hb< 12-14 Mg/dl

CLINICAL PRESENTATION OF ANEMIA SYMPTOM: FATIGUE SKIN NEUROLOGIC CARDIOLOGIC RESPIRATORY GASTROINTESTINAL GUT

CLINICAL PRESENTATION OF ANEMIA SIGN Weakness Skin Neurologic Cardiovascular Respiratory Gastrointestinal GUT

Primary Labortory Test For Anemia CBC –PLT HB HCT RBC MCV 80-100fl HCT(G//L) / RBC (* 1000,000,000,000/L) MCH 30-34 PG HB(G/L) /RBC (*1000.000,000,000) MCHC 30-36G/DL) HB( G/L) / HCT ( L/L) RDW 12 - 14 WBC & DIF PERIPHERAL BLOOD SMEAR RET% BUN/CR

Labortory test for anemia RETICULOCYTE PRUDUCTION INDEX (RPI) RPI=RET%*PHCT/NHCT / SI HCT SHIFT INDEX 45 1 35 1.5 25 2 15 2.5

EXAMPLE FOR RPI CALCULATION 35 YEARS AGED MALE WITH : HB=8 G/DL MCV=105 fl RET%=3% CRC=24 / 45 * 3=1.6 RPI=1.6 / 2 = 0.8

IDA B THAL MIN MCV DECREASE VERY DECREASE RBC/MCV <13 >13 RBC <5000000 >5000000

CALCULATTION USED FOR INTERPRETING RBC INDECES IN MICROCYTIC ANEMIA CALCULATION IDA THAL NINOR MCV-(5*HB)-RBC-3..4 >0 <0 MCV/RBC >13 <13 MCH/RBC >3..8 <3.8 RBC <5000,000 >5000,000 MCH*(MCV)/100 >1530 <1530

Labortory test for anemia IRON RESERVE TESTS SERUM IRON TIBC TRANSFERIN SATURATION FERITIN FREE ERYTROCYTIC PROTOPURPHIRIN BMB STAINING

Labortory test for anemia EVALUTION FOR HEMOLYSIS RETICULOCYTE PRODUCTION INDEX LDH HAPTOGLUBULIN HEMOPEXIN HEMALBUMIN URIN HEMOCIDERIN HEMOGLUBINUREA

APPROUCH TO ANEMIA RPI LOW RPI INCREASED RPI <2 > OR = 2 MCV HEMOLYTIC ANEMIA POST TREATMENT DECREASED NORMAL INCREASED FE TIBC FERITIN MEGALOBLASTIC ANEMIA IDA NO IDA & MDS HB ELECTEROPHERESIS & AA R/O ANEMIA OF CHRONIC DISEASE

36 YRS MALE WITH ANEMIA HB=12 MCV=65 RBC =5600000 MCH=23 28 FEMALE WITH ANEMIA HB=9 MCV=76 RBC= 4800000 14 YRS BOY HB=7 MCV=108 RET=15% RBC=45000000