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RED CELL DISORDERS

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Sel darah merah / Red Blood Cell, RBC
(10
6
/ul)
5,4 4,8
Hematokrit (Hct) %
Vol RBC
-------------- X 100
vol darah
47 42
Hemoglobin/ Hb (g/dL) 16 14
Volume eritrosit rata-rata/
Mean Corpuscular Volume/MCV (fL)
Hct X10
= ----------------
RBC(10
6
/uL)
87 87
Hemoglobin eritrosit rata-rata/
Mean Corpuscular Hemoglobin/MCH
(pg)
Hb X10
=----------------
RBC(10
6
/uL)
29 29
Konsentrasi hemoglobin eritrosit rata-
rata/ Mean Corpuscular Hemoglobin
Concentration /MCHC (g/dL)
Hb X10
=----------------
Hct
34 34
Diameter sel rata-rata/ mean cell
diameter, MCD (um)
=diameter rata-rata 500
sel pada sediaan hapus
7,5 7,5
Tabel Karakteristik RBC Manusia (indikator kapasitas
pembawaan O2)
Normal Blood Cells:
Haemopoiesis:
Pembentukan eritrosit, sebagian besar lekosit,
dan trombosit dewasa di sumsum tulang belakang
(sel multipoten);

Janin: di hati dan limpa
Anak-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)
Basophilic
Normoblast
Polychromatophilic
Normoblast
Orthochromatophilic
Normoblast
Reticulocyte
Erythrocyte
Early Intermediate Late
Steps in Erythropoisis
RED CELL DISORDERS
ANEMIA
ERYTHROCYTOSIS
Anemia is decreased red
cell mass affecting tissue
oxygenation
Practical - Low Hb* or Low Hematocrit*
Anemia
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)
CAUSA

Acquired disorders:
Decreased production
Increased loss

Congenital disorders:
Membrane, Hb & enzyme disorders.

Mechanism of Anemia :
Decreased Production:
Nutrient Deficiency.
Iron, B12/Folate
Hemopoietic cell damage:
Aplastic, Hypoplastic Neoplasms, radiation,
drugs
Lack of erythropoiesis: Kidney disease

Increased loss / destruction:
Blood loss anemias : parasites, bleeding
Hemolytic anemias : Autoimmune, mechanical,
drugs, parasites.
Congenital RBC Disorders:
Membrane Disorders:
Spherocytosis, Elliptocytosis

Hemoglobin Disorders:
Hemoglobinopathies : Sickle cell, HbC
etc.
Thalassemia Syndromes: , ,

Enzyme disorders:
G6PD deficiency
Anemia with Low MCV
(Microcytic Hypochromic)
Differential diagnosis
Iron deficiency
Thalassemia

Laboratory evaluation
Iron, iron-binding capacity, and ferritin
Hb electrophoresis for Thalassemia
Anemia with High MCV
(Macrocytic anemia)
Differential diagnosis
Megaloblastic anemia
Nonmegaloblastic anemia

Laboratory evaluation
Serum vitamin B12, RBC folate
Examination of peripheral smear
Anemia with Normal MCV
(Normocytic anemia)
Differential diagnosis
Primary bone marrow failure
Aplastic anemia
Secondary bone marrow failure
Uremia, HIV infection etc.

Laboratory evaluation
Blood smear for morphology
Erythropoietin level
Bone marrow aspirate and biopsy
Anemia aplastik
Pansitopenia (DT)
Deplesi BM cell & replacement BM with fat
Occur at any age, more frequent in age 50
Etiologi :
1, Primer : congenital, idiopathic
2, sekunder : chemical, drugs, radiation.
Infectius,imunologic

Drugs associated with AA
dose dependent
- benzene
- alkohol
- chemotherapeutic agents
Idiosyncratic :
-cholamphenicol
- fenilbutazon
-Carbonic anhidrase inhibitor

Iron Deficiency Anemia
(IDA)
Katabolisme iron
Eritrosit (100-120 hari)

Difagositosis oleh sistem makrofag

Globin heme

Porfirin iron

Biliverdin utk sistesis Hb baru


bilirubin
Ekskresi (empedu)
IDA - Etiology
Blood loss
Bleeding Parasites, Gynecologic,
ulcers
Increased need
Pregnancy, children
Poor diet / poor absorption
Malnutrition (greens & meat),
malabsorption, intestinal surgery,
gastric atrophy.
IDA - Pathogenesis:
Decreased Iron stores
Decreased Hb Synthesis
Delayed maturation of erythroblasts
(cytoplasmic)
Decreased cytoplasm, more division
(microcytes)
Decreased hb content (hypochromia)
Anemia.
Laboratory tests:

Hematologi rutin
Serum iron
Serum iron binding capacity
Serum ferritin

Microcytic Anemia (IDA)
Symptoms of Anemia
Nonspecific and reflect tissue hypoxia:

Fatigue
Dyspnea on exertion
Palpatations
Headache
Confusion, decreased mental acuity
Skin pallor
Clinical Features:
General features of Anemia
Pallor, Weakness, Lethargy,
Breathlessness on exertion
Palpitations heart failure pedal edema

Special features in IDA:
Angular cheilitis, atrophic glossitis,
Oesophageal atrophy/web dysphagia,
Koilonychia, brittle nails, gastric atrophy.
Angular cheilitis
Angular cheilitis & Glossitis
Koilonychia in Iron def.
Koilonychia in Iron def.
Iron Deficiency Anemia:
IDA on Treatment :
Megaloblastic anemia:
Vitamin B12/Folic acid deficiency
Second most common type of anemia.
Macrocytic anemia, pancytopenia.
Pernicious anaemia:
autoimmune, Gastric atrophy, Vit B12
def.
Megaloblastic anemia - Etiology
Malnutrition
Intrinsic factor Ab - Pernicious
anemia
Gastrectomy, Ileal resection
Inflammatory bowel disease
Malabsorption syndromes - Sprue
Blind loop syndrome

Megalobl - Pathogenesis:
Decreased Vit B12 / Folate
Decreased DNA Synthesis
Delayed maturation of erythroblasts
(Nucleus)
Increased cell size (macrocytes)
Normal hb content (Normochromia)
Decreased RBC number
Decreased WBC number (pancytopenia)
Anemia & Pancytopenia.
Vitamin B
12
Absorption
Parietal cells -
produce IF
IF
B
12

B
12

B
12+IF
IF
Stomach
IF


Ileum -
IF receptors
B
12

B
12
Macrocytic Anemia (Meg.):
Macroovalocytes & Macropoly
Megaloblastic Anemia :
Differential diagnosis of
Anemia
Low Hb=Anemia
MCV
Low
microcytic
Normal
normocytic
High
macrocytic
Measure Ferritin
Low
Normal/high
Iron def
Anemia
Anemia of
chronic disease/
Congenital Hb dis.
Reticulocyte count
high
low Anemia of chronic disease
Renal failure
Marrow failure
Hemolytic anemia or
blood loss
Measure B
12
+ folate

Low
Megaloblastic
anemia
Normal
Causes of High Hct/polycythemia
Relative or spurious erythrocytosis
Hemoconcentration secondary to dehydration
(diarrhea, diaphoresis, diuretics, deprivation of
water, emesis, ethanol, etc.)

Absolute erythrocytosis (True ):
Tissue hypoxia Smoking (Co), High altitude,
Pumonary disease, respiratory def. Cardiac shunts,
High oxygen-affinity Hb.
High EPO - Tumors eg. Renal cell Ca, hepatoma
Androgen therapy
Primary - Polycythemia vera

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