Anemia PDF

September 11th, 2022  only platelet count remains the same with age Anemia neonates [14 infants ↓ Hb can't meet the demand...

September 11th, 2022  only platelet count remains the same with age Anemia neonates [14 infants ↓ Hb can't meet the demand heart tries to compensate by 1HRU but this puts a lot ofwork on heart and o OPGaffinity fHybro leads I'll 2,3 Tecrere shitty to a hyperdynamic circulation - over it will time. fail EPOlevels so RBC  Should which not causes be crying conjunctival injection Pallor congestion tonguetmulontagnes v 75 8 Hgb NO  Mcv femtoliter unidaccording to age according to RBC size ( MCV ) < 20 70-90 790 f- L Decreased RBC production: production , but ↑ destruction inside ↑ Progenitor bone marrow ↓ Precursors Increased destruction: ↓ few RBC survives to be released ( hyper spleuism ) into blood ( blood loss )  Diman 75% 20% Malignancy bleeding ( ↑ reticulocyte ] Hypothyroidism → normocytic Casually ] → micro epic Macroeytie Microcytic less 7o fl TAIL 1-Iron deficiency anemia 2-Thalassemias T.tn 3-Lead poisoning hernia of microytic 4- Chronic Disease 201 Normocytic 801 EBD  Macrocytic > 90fl VIT B12 OR FOLIC ACID DEFICIANCYMegaloblotic LIVER DISEASE v HYPOTHYRODISIM Diamond blackfon anemia Fanconi anemia Aplastic anemia Down syndrome  Hemolysis → ↑ Reticulum O ( early ) Transient erythroblastopenia of childhood ( hypothyroid ) ↓ ↓ SCD Hb sickle C  1St 9months goodiron storesbeofgraders destruction ofHgh ID A Ination earlylifeanemia pre mature 1 1/3 of world population are iron deficient but not necessarily anemic Four Stages: 1. Storage iron depleted (↑TIBC,↓Ferritin) 2. Serum iron depleted (plus↓serum iron level) 3. Normocytic anemia (plus ↓Hb, ↓RBC count, normal MCV) 4. Microcytic, hypochromic anemia (all above plus ↓MCV, MCH,↑RDW) 1ˢᵗ 2yearsof life Growth spurts in children and infants puberty Decrease store in prematurity. 2-Decreased intake Poor diet Poor absorption. 3- Increased loss Chronic GI bleeding. hemangioma polyps chronic Hemolysis. Surgical. Worm infestation. Ancylostoma duodenale & Necator americanus 21 year cow milk protein allergy Inflamalen of God  in newborn Hb is high iron it's breakdown gives Iron Absorption is enhanced orange rice system vinon Inhibitors of Iron Absorption Phytate :. Femtinoiivertseentnm.me  00 persistent craving and Pica for ice episodic apnea in children, possibly associated with loss of consciousness, compulsive eating of changes in postural tone & cyanosis. nonfood substances  IDA Ethiopia PlauerLimonsu Other features: Koilonychia (spoon shaped nails) Atrophic glossitis (beefy red tongue), r Cheilosis (angular stomatitis) s Plummer-Vinson syndrome (triad of IDA, esophageal webs, and dysphagia). anur stands iEIT Y  3 6mg kg resolving of Symptoms Appetite ferittin I appetite 0 4-30 days -3 months I to correct iron stores Iron stoves  Macrocystic Autoromaldominal Is a rare, congenital bone marrow failure syndrome that usually becomes symptomatic in early infancy. BM failure More than 90% of cases are recognized in the 1st yr of life. Characteristically there are macrocytic anemia, reticulocytopenia, and deficiency or absence of red blood cell c (RBC) precursors in an otherwise normally cellular bone marrow. Fanconi Paneytopeuier I anemia → IN bone Manon  p Nomal 35 37 cm at birth Associated anomalies 1 Headcircultera23rdcentile smallmandible forage 1-face and head(microcephaly,micrognathia) normally 2 Phalanx of thumb 2- arm and hand(triphalangeal.bifid thumb) 50% 3-heart(VSD,ASD,COARACTATION) 50% 4-Genitourinary tract(absence kidney,horse shoe,hypospadius 301. 5- Short stature Diagnosis Complete blood cell count Avery low number of red blood cells as well as low hemoglobin RBC Low retic count 719B Inetics Bone marrow sample (biopsy ) NONEWRBI would show that few new red blood cells were being created Nohyperregmentedmet.is agonemunowchromountstrined unhlobotic todiff no infunco TEC is after lyen.ihhevitedpancytopem.co Treatment Apltiffs 1-prednesolone 2-androgen o 3-antithymocyte globulin Ab against thymoctes 4- transfusion 5ˢTEMmGFat Bad prognosis  Heme ← Hemet Globin ^ ^ -2 29 213 Porphyrin Fe ↑ ↑ ↑ 9 IDA ✗ thalassemia p thalassemia 139,1312 SCD deficiency  6types of Hob inuten Goveri Govern rivers the Then replaced byHatThen replaced toAbugouthham II Embryonic Normal Hb are tetrameric molecules containing pairs of alpha or Beta globin-heme subunit. Normal postnatal Hb are: > 6 months Alpha Beta Hb A 2 α , 2 Bβ more than 95% gammer HbF 2α,2γ less than 2.5% delta Hb A2 2α,2δ 2 - 3.4%  The thalassaemia are heterogeneous group of inherited disorders, which are characterized by reduced or absent synthesis of one or more globin ↓ > chain type. or absent of one or More globin Chin th res The imbalance of globin chain synthesis, which result leads to ineffective erythropoiesis and a shortened red cell lifespan. (shape change -> engulfed splenic macrophages (  B globin gene The β-thalassemias onchromoso.me β-thalassemia is commonly caused by mutations in the β globin gene on chromosome 11 8 complete Aber major 85 deared minor  B B Types of B- thalassemia ↓ 10wc B Comin Silent Carrier State for β Thalassemia " 1/ is BIP-mild anemia B-Thalassemia Minor B/B or is*/B is/B" Beta Thalassemia Intermedia mild or to severe Beta Thalassemia Major B/B0- severe 'S Cooley an emier Coollys anein  Characterized by severe microcytic, hypochromic anemia. Detected early in childhood: after 6 months – Infants fail to thrive. hemolysis survival → ↓ RBC – Have pallor, variable degree of jaundice, abdominal enlargement, and hepatosplenomegaly. EMH Severe anemia causes marked bone changes due to expansion of marrow space for increased erythropoiesis. 6 Characteristic changes in skull, long bones, and hand bones. in Skull longbones 29 charges havidbous crew cut or Hair on End  Diagnosis Here’s a more detailed explanation of the diagnosis of thalassemia, broken into its key components: 1. Clinical Evaluation Thalassemia presents with varying severity, depending on whether it is alpha or beta-thalassemia, and whether the patient is a carrier or has the disease. Mild Thalassemia (Trait): Often asymptomatic or mild anemia. Moderate to Severe Thalassemia (Intermedia/Major): Severe anemia (Hb basically any organ except brain, luny) -> main cause of death is CMPO Arrhythmia  4 genes on α 2 geus on 8 Normally 4x/GO More than 95% of a thalassemia's result from the deletion of one or both More of a globin genes located on chromosome 16. This gives rise to four possible genotypes: gene detection § Carrier state (silent state ) Minima 2x1x- 2 genedeletion § α Thalassemia Trait (Alpha Thalassemia Minor) Anemia, CNS Crisis & Sequestration crisis Hydroxyurea ->i Hbf produced which do not have B so a crisis; bhemolysis Bone Marrow Transplant -> Cure read acid Any hemolysis folic Meshadalann Favism Deficiency of G6PD is the most common red cell enzyme defect that causes hemolytic anemia. The disorder has X-linked recessive inheritance and occurs with high frequency among persons of African, Mediterranean, and Asian ancestry. including us Hundreds of different G6PD variants have been characterized. X linked 0 affected meno TouchtonL Turner syndrome unless carrier 1- are q G6PD catalyzes the conversion of glucose76-phosphate to 6- phosphogluconic acid. This reaction produces NADPH, which maintains GSH in the reduced, functional state, necessary to protect the red cells against oxidant stress. No protection of glutathione HemolyI q In most instances, the deficiency is due to enzyme instability; thus, older red cells are more deficient than younger ↳ early degradation of enzyme ones ↓ its half life o  FYING Episodic hemolytic anemia (with occident stress) Chronic nonspherocytic hemolytic anemia Ø The most common manifestations of this disorder are neonatal jaundice and episodic acute hemolytic anemia, ①most commonly ⑤ ⑪ beans. close dependent ( 00 which is induced by infections, certain drugs, and ② Stress fava (menstruation ( Do not mention fava bean on top of the list smartXana Ø Episodes of hemolysis are associated with pallor, jaundice, compromise. o hemoglobinuria, dark H3 and urine sometimes Hemaglopinain & Urobilinogen cardiovascular Darkurine Glomerulonephritis AutoimmuneHemolytic Hematin Ø Children with G6PD deficiency are asymptomatic and appear normal between episodes of hemolysis ØA typical sign is the dramatic darkening of the urine with hemoglobin and urobilinogen to produce so-called ‘Coca-Cola coloured urine.  u r r  2 Antibacterials Antimalarials Sulfonamides Primaquine I ÷ Trimethoprim- Pamaquine sulfamethoxazole Chloroquine Nalidixic acid Quinacrine Chloramphenicol I Nitrofurantoin  used Phenacetin ⁿ° old analgesic not paracetamol , only in large doses Vitamin K analogs Methylene blue Probenecid Acetylsalicylic acid Phenazopyridine urologic analgesic CUTI ) CHEMICALS Phenylhydrazine is Benzene Naphthalene ILLNESS Diabetic acidosis  Diagnosis fall in hemoglobin and hematocrit. Hm free hemoglobin may appear in the plasma and Heinbodies subsequently in the urine. ¢0s ( Heinz body Heinz bodies (precipitated hemoglobin) bite cells removed ) 0 The blood film reveals a few fragmented and polychromatophilic cells (bluish, large RBCs), representing reticulocytosis, which may be substantial (5–15%). The diagnosis depends on direct or indirect demonstration of reduced G6PD activity in RBCs. Heinbodies Biked  I Prevention of hemolysis constitutes the most important therapeutic measure. They will be given a chart written on it all the drugs & foods to avoid. When hemolysis has occurred, supportive 4170 therapy may require blood transfusions, if Hb < 7 gldl although recovery is the rule when the oxidant agent is discontinued. ( stop causative drug treat infectious  autosomal dominant It is the most common inherited abnormality of the red blood cell (RBC) membrane. It is usually transmitted as an autosomal dominant or, less commonly, as an autosomal recessive disorder. As many as 25% of patients have no previous family mutation or de novo I spte history. - undetected mild disease in Parents ( variable expression ) It is the result of a partial deficiency of spectrin or ankyrin an important structural proteins of the red cell membrane skeleton that weakens the attachment of the cell membrane to the underlying membrane skeleton and causes the red cell to lose membrane surface area. This process creates spherocytes that are poorly deformable and have a shortened life span because they are trapped in the microcirculation of the spleen and engulfed by splenic macrophages  Hyperbicialmia Hemolytic ahem Splenomegaly Ø In the neonatal period hyperbilirubinemia sufficiently severe to require phototherapy or exchange transfusions. Ø Features of anemis Hemolytic anemia Øsplenomegaly  Lab test 0th Normochromic normocytic I Flow cytom Hb maintained 6-9 gmldl not Pathettstancysors Sherocytosis,reticulocytosis Mp not Pathognomatic other of spherocytes: - - causes Treatment EE motheriso ABO (Isoimmune) incompatibility snake bite splenectomy Wilson disease thermal injury (severe hypothermian)  flow cytometry or osmotic fragility muy Blood film be used if in doubt