RED CELL DISORDERSRED CELL DISORDERS

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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