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macrocytic anemia
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MACROCYTIC ANEMIASahyuddinTutik Harjianti A. Fachruddin B
Div. of Hematology & Medical Oncology Dept. of Internal Medicine, Medical Faculty Hasanuddin University
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicineDeficiency CyanocobalaminB12 : all of it made from dietA food ingredients may from animal.Absorbtion : 5 ug / daysCofactor at 2 important reaction in a body
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicineThe role of CyanocobalaminMethyl-Cobalamin is a cofactor for methionine-synthetase at rx change of homosystein metyonin.Adenosyl-Cobalamin is a cofactor at rx change of methyl-malonyl CoA succinyl-CoA
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicineDeficiency CyanocobalaminAn important sign :
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicineMacrocyticNormocytic
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicineThe Cause Deficiency vit B12 (diet)The decrease production of intrinsic factor (Anemia perniciosa, post-gastrectomy)The decrease absorbtion of vit B12 at the ileum (Post-op, Crohn ds)Helmynthyasis (tape-worm)Deficiency Transcobalamin II
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicinePhysiologyVit B12 come in from IT binding with intrinsic factor (made from parietal mucosa gaster cell) abs in ileum terminal by spesific receptor come in to the plasma liver .There are 3 protein transporter in the plasma : Trans-cobalamin I, II & III (by leukocyte). Only Trans-cobalamin II that can transport vit B12 into the cell.
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal Medicine
PhatogenesisHepar consist 2.000 5.000 ug vit B12
Need : 3 5 ug / hari
Defs vit B12 will be happen in 3 years after no more absorpsi.
Defs caused by diet less vit B12 vary rare ( vegetarian )
Example :Gastrectomy the area produce factor intrinsik will decreaseOver-growth bactery in intestinalReseksi ileum the area of absorpsi vit B12 will decreaseHelmenthyasisCrohns disease ileum destruction the area of absorpsi vit B12 will decrease
Anemia Perniciosa Often cause defs B12Abnormality Auto-Imun herediterSeldom show before 35 years oldScandinavia / Eropa UtaraA black skin teenager, a hispanic woman
Anemia PerniciosaClinic illustration : Likely anemia caused byndefs vit B12, - Gastritis atrophic - Abnormal Auto-Imun ( rheumatoid arthritis Graves disease, defs IgA ) - After several years some patient Gastritis Atrophic => Carcinoma Gaster
CLINIC ILLUSTRATION DEFS. VIT B12Megaloblastic anemia
May a hard anemia ( hematokrit < 10 % )
A change mucosa cell : glossitis, anorexia, diare.Neurologic disturb: 1. Perifer parestesi 2. Cerebral difunct
Lab. Abnormal1. Megaloblastic Anemia2. MCV between 110 140 fl (increase) at some patient : MCV normal ( thalassemia , defs Fe )
3. Blood Perifer : anisocitosis & poikilocitosis. Specif : makro-ovalosit.
Blood Perifer4.Morfologi eritrosit very abnormal Likely Hemolytic Anemia 5. Hypersegmentasi netrofil
6. Reticulocit amount decrease
Bone Marrow AspEritropoesis in-efektif ( ggn produksi RBC ) hiperplasi eritroid ( as respons )
Cell megaloblast abnormal in SST diferent shape : * big abnormal size, * maturasi inti & sitoplasma tdk.sinkron. Maturasi cytoplasm is normal, DNA synthesis is bother
Seri mieloid : Giant sel meta-mielosit
Other Lab. Abnormal : In-efektif eritropoesis in SST may happen destruction eritroid cell that in the development period level LDH ( lactic-dehydrogenase ) very increase, and Bilirubin indireck increase just for a little
Diagnosis1. Level vit B12 serum is less ( normal : 150 -350 pg / mL )2. Schilling test ( for dx A Perniciosa / the decrease absorpsi vit B12 oral ) 1st step : the patient injection w/ Vit B12 i.m Then give vit B12 that has di-label p.o Retain urine 24 hours ( Normal : > 7 % )
Test Schilling 2nd stepGive vit B12 that has , with the intrinsic factor.If the patient Anemia Perniciosa ( defs intrinsic factor ), so absorpsi will fixed.At a hard case ( epitel usus abnormal ) malabsorption the result of test Schilling II may still abnormal sp defek mukosa usus pulih.
THERAPY* At Anemia Pernisiosa ( oral absorpsi disfun)Intra-muscular Inj. Vit B12 ( IM )Dosis : 200 ug 1st week : every day (replacement tx)2nd 4th week : every weekOnce a month
Respons TherapyUsually easy to show, GC better, the complain decrease.Retyculocitosys happen in 5 7 days.
Abnormalitas hermatologic will be dissappear after 2 months.SSP disorder will be dissappear if we give therapy before 6 months sick.
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal Medicine
Division of Hematology & Medical Oncology Dept. of Internal MedicinePathogenesis
Division of Hematology & Medical Oncology Dept. of Internal Medicine
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