RED CELL DISORDERSRED CELL DISORDERS
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PriaPria WanitaWanita
Sel darah merah / Red Blood Cell, RBC Sel darah merah / Red Blood Cell, RBC (10(1066/ul)/ul)
5,45,4 4,84,8
Hematokrit (Hct) %Hematokrit (Hct) % Vol RBCVol RBC-------------- X 100-------------- X 100
vol darah vol darah
4747 4242
Hemoglobin/ Hb (g/dL)Hemoglobin/ Hb (g/dL) 1616 1414
Volume eritrosit rata-rata/ Volume eritrosit rata-rata/ Mean Corpuscular Volume/MCVMean Corpuscular Volume/MCV (fL) (fL)
Hct X10Hct X10= ----------------= ----------------RBC(10RBC(1066/uL)/uL)
8787 8787
Hemoglobin eritrosit rata-rata/ Hemoglobin eritrosit rata-rata/ Mean Corpuscular Hemoglobin/MCHMean Corpuscular Hemoglobin/MCH (pg)(pg)
Hb X10Hb X10=----------------=----------------RBC(10RBC(1066/uL)/uL)
2929 2929
Konsentrasi hemoglobin eritrosit rata-Konsentrasi hemoglobin eritrosit rata-rata/ rata/ Mean Corpuscular HemoglobinMean Corpuscular Hemoglobin Concentration /MCHC (g/dL)Concentration /MCHC (g/dL)
Hb X10Hb X10=----------------=----------------
HctHct
3434 3434
Diameter sel rata-rata/ Diameter sel rata-rata/ mean cell mean cell diameterdiameter, MCD (um), MCD (um)
=diameter rata-rata 500 =diameter rata-rata 500 sel pada sediaan hapussel pada sediaan hapus
7,57,5 7,57,5
Tabel Karakteristik RBC Manusia (indikator kapasitas pembawaan O2)
Normal Blood Cells:Normal Blood Cells:
Haemopoiesis:Haemopoiesis:
Pembentukan eritrosit, sebagian besar lekosit, dan trombosit dewasa di sumsum tulang belakang (sel multipoten);
Janin: di hati dan limpaAnak-anak di rongga sumsum tulang seluruh tulang ad 20 tahun, kecuali tulang humerus atas dan femur.
Sumsum tulang seluler aktif = sumsum merah.Sumsum tulang inaktif = sumsum kuning (diinfiltrasi lemak).
Proerythroblast(Pronormoblast)
BasophilicNormoblast
PolychromatophilicNormoblast
OrthochromatophilicNormoblast
Reticulocyte
Erythrocyte
Early Intermediate Late
Steps in ErythropoisisSteps in Erythropoisis
RED CELL DISORDERS
ANEMIA ERYTHROCYTOSIS
““Anemia is decreased red Anemia is decreased red cell mass affecting tissue cell mass affecting tissue
oxygenation”oxygenation”
Practical - Low Hb* or Low Hematocrit*Practical - Low Hb* or Low Hematocrit*
AnemiaAnemia
• A reduction below normal in the concentration of
hemoglobin or red blood cells in the blood.
• Hematocrit (<40% in men,<36% in women)
• Hemoglobin (13.2g/dl in men, 11.7g/dl in women)
CAUSACAUSA
Acquired disorders:Acquired disorders:Decreased productionDecreased productionIncreased lossIncreased loss
Congenital disorders:Congenital disorders:Membrane, Hb & enzyme disorders.Membrane, Hb & enzyme disorders.
Mechanism of Anemia :Mechanism of Anemia : Decreased Production:Decreased Production:
Nutrient Deficiency. Nutrient Deficiency. Iron, B12/FolateIron, B12/Folate
Hemopoietic cell damage:Hemopoietic cell damage:Aplastic, Hypoplastic – Neoplasms, Aplastic, Hypoplastic – Neoplasms,
radiation, drugsradiation, drugs Lack of erythropoiesis: Kidney diseaseLack of erythropoiesis: Kidney disease
Increased loss / destruction:Increased loss / destruction: Blood loss anemias : parasites, bleedingBlood loss anemias : parasites, bleeding Hemolytic anemias : Autoimmune, Hemolytic anemias : Autoimmune,
mechanical, drugs, parasites.mechanical, drugs, parasites.
Congenital RBC Disorders:Congenital RBC Disorders:Membrane Disorders:Membrane Disorders:
Spherocytosis, ElliptocytosisSpherocytosis, Elliptocytosis
Hemoglobin Disorders:Hemoglobin Disorders:Hemoglobinopathies : Sickle cell, Hemoglobinopathies : Sickle cell,
HbC etc.HbC etc.Thalassemia Syndromes: Thalassemia Syndromes: , , , ,
Enzyme disorders:Enzyme disorders:G6PD deficiencyG6PD deficiency
Anemia with Low MCVAnemia with Low MCV(Microcytic Hypochromic)(Microcytic Hypochromic)
Differential diagnosisDifferential diagnosis Iron deficiency Iron deficiency Thalassemia Thalassemia
Laboratory evaluationLaboratory evaluation Iron, iron-binding capacity, and ferritin Iron, iron-binding capacity, and ferritin Hb electrophoresis for ThalassemiaHb electrophoresis for Thalassemia
Anemia with High MCVAnemia with High MCV(Macrocytic anemia)(Macrocytic anemia)
Differential diagnosisDifferential diagnosis Megaloblastic anemiaMegaloblastic anemia Nonmegaloblastic anemiaNonmegaloblastic anemia
Laboratory evaluationLaboratory evaluation Serum vitamin B12, RBC folate Serum vitamin B12, RBC folate Examination of peripheral smearExamination of peripheral smear
Anemia with Normal MCVAnemia with Normal MCV(Normocytic anemia)(Normocytic anemia)
Differential diagnosisDifferential diagnosis Primary bone marrow failurePrimary bone marrow failure
Aplastic anemia Aplastic anemia Secondary bone marrow failure Secondary bone marrow failure
Uremia, HIV infection etc. Uremia, HIV infection etc.
Laboratory evaluationLaboratory evaluation Blood smear for morphology Blood smear for morphology Erythropoietin level Erythropoietin level Bone marrow aspirate and biopsyBone marrow aspirate and biopsy
Anemia aplastikAnemia aplastik
Pansitopenia (DT)Pansitopenia (DT)Deplesi BM cell & replacement BM with fatDeplesi BM cell & replacement BM with fatOccur at any age, more frequent in age 50Occur at any age, more frequent in age 50Etiologi :Etiologi :
1, Primer : congenital, idiopathic1, Primer : congenital, idiopathic
2, sekunder : chemical, drugs, radiation. 2, sekunder : chemical, drugs, radiation. Infectius,imunologicInfectius,imunologic
Drugs associated with AADrugs associated with AA
dose dependentdose dependent
- benzene- benzene
- alkohol- alkohol
- chemotherapeutic agents- chemotherapeutic agents Idiosyncratic :Idiosyncratic :
-cholamphenicol-cholamphenicol
- fenilbutazon- fenilbutazon
-Carbonic anhidrase inhibitor-Carbonic anhidrase inhibitor
Iron Deficiency AnemiaIron Deficiency Anemia(IDA)(IDA)
Katabolisme iron
Eritrosit (100-120 hari)
Difagositosis oleh sistem makrofag
Globin heme
Porfirin iron
Biliverdin utk sistesis Hb baru
bilirubin
Ekskresi (empedu)
IDA - EtiologyIDA - Etiology
Blood loss Blood loss Bleeding – Parasites, Gynecologic, Bleeding – Parasites, Gynecologic,
ulcers…ulcers… Increased needIncreased need
Pregnancy, childrenPregnancy, children Poor diet / poor absorptionPoor diet / poor absorption
Malnutrition (greens & meat), Malnutrition (greens & meat), malabsorption, intestinal surgery, malabsorption, intestinal surgery, gastric atrophy.gastric atrophy.
IDA - Pathogenesis:IDA - Pathogenesis:
Decreased Iron storesDecreased Iron storesDecreased Hb SynthesisDecreased Hb SynthesisDelayed maturation of erythroblasts Delayed maturation of erythroblasts
(cytoplasmic)(cytoplasmic)Decreased cytoplasm, more division Decreased cytoplasm, more division
(microcytes)(microcytes)Decreased hb content (hypochromia)Decreased hb content (hypochromia)Anemia.Anemia.
Laboratory tests:Laboratory tests:
Hematologi rutinHematologi rutinSerum ironSerum ironSerum iron binding capacity Serum iron binding capacity Serum ferritin Serum ferritin
Microcytic Anemia (IDA)Microcytic Anemia (IDA)
Symptoms of AnemiaSymptoms of Anemia
Nonspecific and reflect tissue hypoxia:Nonspecific and reflect tissue hypoxia:
FatigueFatigueDyspnea on exertionDyspnea on exertionPalpatationsPalpatationsHeadache Headache Confusion, decreased mental acuityConfusion, decreased mental acuitySkin pallorSkin pallor
Clinical Features:Clinical Features: General features of AnemiaGeneral features of Anemia
Pallor, Weakness, Lethargy, Pallor, Weakness, Lethargy, Breathlessness on exertionBreathlessness on exertionPalpitations Palpitations heart failure heart failure pedal pedal
edemaedema
Special features in IDA:Special features in IDA:Angular cheilitis, atrophic glossitis, Angular cheilitis, atrophic glossitis, Oesophageal atrophy/web Oesophageal atrophy/web dysphagia, dysphagia, Koilonychia, brittle nails, gastric atrophy.Koilonychia, brittle nails, gastric atrophy.
Angular cheilitisAngular cheilitis
Angular cheilitis & GlossitisAngular cheilitis & Glossitis
Koilonychia in Iron def.Koilonychia in Iron def.
Koilonychia in Iron def.Koilonychia in Iron def.
Iron Deficiency Anemia:Iron Deficiency Anemia:
IDA on Treatment :IDA on Treatment :
Megaloblastic anemia:Megaloblastic anemia:
Vitamin B12/Folic acid deficiencyVitamin B12/Folic acid deficiency Second most common type of Second most common type of
anemia.anemia. Macrocytic anemia, pancytopenia.Macrocytic anemia, pancytopenia. Pernicious anaemia:Pernicious anaemia:
autoimmune, Gastric atrophy, Vit autoimmune, Gastric atrophy, Vit B12 def.B12 def.
Megaloblastic anemia - EtiologyMegaloblastic anemia - Etiology
MalnutritionMalnutrition Intrinsic factor Ab - Pernicious Intrinsic factor Ab - Pernicious
anemiaanemiaGastrectomy, Ileal resectionGastrectomy, Ileal resection Inflammatory bowel diseaseInflammatory bowel diseaseMalabsorption syndromes - Malabsorption syndromes -
SprueSprueBlind loop syndromeBlind loop syndrome
Megalobl - Pathogenesis:Megalobl - Pathogenesis:
Decreased Vit B12 / FolateDecreased Vit B12 / Folate Decreased DNA SynthesisDecreased DNA Synthesis Delayed maturation of erythroblasts Delayed maturation of erythroblasts
(Nucleus)(Nucleus) Increased cell size (macrocytes)Increased cell size (macrocytes) Normal hb content (Normochromia)Normal hb content (Normochromia) Decreased RBC number Decreased RBC number Decreased WBC number (pancytopenia)Decreased WBC number (pancytopenia) Anemia & Pancytopenia.Anemia & Pancytopenia.
Vitamin BVitamin B1212 Absorption Absorption
Parietal cells - produce IF
IF
B12
B12
B12+IFIF
Stomach
IF
Ileum -IF receptorsB12
B12
Macrocytic Anemia (Meg.):Macrocytic Anemia (Meg.):
Macroovalocytes & MacropolyMacroovalocytes & Macropoly
Megaloblastic Anemia : Megaloblastic Anemia :
Differential diagnosis of Differential diagnosis of AnemiaAnemia
Low Hb=AnemiaLow Hb=AnemiaMCVMCV
LowLowmicrocyticmicrocytic
Normalnormocytic
HighHighmacrocyticmacrocytic
Measure Ferritin
Low Normal/high
Iron def Anemia
Anemia ofchronic disease/Congenital Hb dis.
Reticulocyte count
high low Anemia of chronic diseaseRenal failureMarrow failure
Hemolytic anemia or blood loss
Measure B12 + folate
LowMegaloblasticanemia
Normal
Causes of High Hct/polycythemiaCauses of High Hct/polycythemia
Relative or spurious erythrocytosisRelative or spurious erythrocytosis Hemoconcentration secondary to dehydrationHemoconcentration secondary to dehydration (diarrhea, diaphoresis, diuretics, deprivation of(diarrhea, diaphoresis, diuretics, deprivation of
water, emesis, ethanol, etc.)water, emesis, ethanol, etc.)
Absolute erythrocytosis (True ):Absolute erythrocytosis (True ): Tissue hypoxiaTissue hypoxia – Smoking (Co), – Smoking (Co), High altitude, High altitude,
Pumonary disease, respiratory def. Cardiac Pumonary disease, respiratory def. Cardiac shunts, High oxygen-affinity Hb.shunts, High oxygen-affinity Hb.
High EPO - Tumors eg. Renal cell Ca, hepatomaHigh EPO - Tumors eg. Renal cell Ca, hepatoma Androgen therapyAndrogen therapy Primary - Polycythemia vera Primary - Polycythemia vera
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