Uploaded by ROSYID

ANEMIA ROSYID RIDHO EDIT

advertisement
ANEMIA
Your company information
anemia
•
•
•
Anemia adalah suatu sindroma klnik yang disebabkan oleh penurunan
kapasits angkut oksigen darah
Keadaan klinik yng ditandai oleh penuruna kadar hemoglogin , hmatokrit
atau hitung eritrosit . Angka normal ketiga parameter itu ditentukan oleh
umur jenis kelamin dan keadaan fisiologi
Pada dasarnya anemia terjadi karena :
–
–
Proses eritropoesis yang tidak normal
Masa hidup eritrosit memendek , karena ;
•
•
1. hemolisis
2 kehilangan darah (bleeding ) baik secara akut maupun krinik
eritropoesis
• Untuk terjadi eritropoisis yang baik maka
diperlukan
– Sel induk
– Bahan pembentuk eritrosiit
– Lingkungan mikro sumsum tulang yang baik
– Mekanisme regulasi
Sel
induk
limfoid
Sel
induk
Mieloid
Sel induk
Pluripoten
Sel induk
mieloid
BFU - E
CFU – E
Pronormoblast
Normoblast
Basofilik
Normoblast
polikromatofilik
Retikulosit
eritrosit
• 2 Proses utam dalam eritropoiesis:
– Pembentukan Inti ( DNA)
– Pembentukan hemoglobin
Pembentukan Inti
• Mula mula terjadi pembentukan DNA
sebagai bhan penyusun yang berguna
dalam mitosis SEL. Bahan Pembentukan
utama DNA (asam folat atau vitamin B12).
Apabila terjadi defisiensi asam folat maka
akan menghambat mitosis
Pembentukan Hemoglobin
• Pembentukan hemoglobin merupakan
proses terpenting dalam sitoplasma
eritrosit (Heme dan Globin).
• Pembentukan heme terjadi dalam
mitokondria yang memerlukan
protoporfirin , Gangguan pembentukan
Protoporfirin menimbulkan anemia
sideroblastic
Definition of anemia:
Anaemia is defined as a reduced haemoglobin level in the
peripheral blood.
The principal effect is decreased oxygen-carrying capacity
of the blood.
Mild
Moderate
Severe
Normal Hb:
Hb 10-12 g/dl (M)
Hb 9-11 g/dl (F)
Hb 8-10 g/dl (M)
Hb 7-9g/dl (F)
Hb concentrations are below this
level
13.5-17.5 g/dl (M)
11.5-15.5 g/dl (F)
Normal Blood Count: adult values
Hb
13.5-17.5 g/dl (M); 11.5-15.5 g/dl (F)
RBC
4.5-6.5 x 1012/l (M); 3.9-5.6 x 1012/l (F)
PCV (haematocrit)
40-52% (M); 36-48% (F)
MCV
80-95 fl
MCH
27-34 pg
MCHC
30-35g/dl
Reticulocytes
0.5-2.0%
Platelets
150-400x109/l
WBC (total)
4.0-11.0 x109/l
Neutrophils
2.5-7.5 x109/l
Lymphocytes
1.5-3.5 x109/l
Monocytes
0.2-0.8 x109/l
Eosinophils
0.04-0.44 x109/l
Basophils
0.01-0.1 x109/l
• Hct, Hb, RBC: determine degree of anemia
• Red cell indices (MCV, MCH, MCHC): determine
normocytic, macrocytic, microcytic, normochromic,
hypochromic.
• RDW: measure aniscytosis.
• Reticulocyte count or index: estimate marrow
response
• Blood film: determine red cell shape, Hb content, red
cell inclusion, abnormalities white cells and platelet.
Hct, hematocrit; Hb, hemoglobin, RBC, red blood cell; RDW, red distribution width
Cardiovascular  Exertional dyspnea, tachycardia,
and
palpitations, orthopnea, angina.
Respiratory
 cardiomegaly, murmurs, vascular bruits,
↑respiratory rate.
Neurologic
Skin
 Headaches, tinnitus, dizziness, faintness,
loss of concentration, fatigue, cold sensitivity.
 Pallor of skin, mucous membranes,
nailbeds, and palms.
Gastrointestina  Anorexia, nausea, constipation, diarrhea.
l
Genitourinary
 Menstrual irregularity, amenorrhea,
menorrhagia, loss of libido or potency.
• Anemia Hipokromik mikrositer (MCV < 80,
MCH <27 pg)
– Anemia defisiensi besi
– Thalasemia
– Anemia oleh Penyakit Kronis
– Anemia Sideroblastik
• Anemia Normokromik normositer ( MCV
80-95, MCH 27 – 34 )
– Anemia pasca perdarahan akut
– Anemia aplastic
– Anemia hemolitik tertentu
– Anemia mieloplastik
– Anemia gagal ginjal kronik
– Anemia leukemia akut
• Anemia Makrositer ( MCV > 95 fl):
– Anemia Megaloblastik
• Anemia defisiiensi asam folat
• Anemia defisiensi vitamin B12
– Anemia non megaloblastic
• anemia pada penyakit hati kronik
• Anemia pada hipotiroidi
• Anemia pada sindroma mielodislastik
ANEMIA MAKROSITIK
HIPOKROMIK
Included :
1. Iron deficiency anaemia
2. Thalassaemic syndromes
3. Sideroblastic anaemia
4. Anaemia of chronic disease (some
cases)
The causes of a hypochromic, microcytic anaemia. These include lack of
iron (iron deficiency) or of iron release from macrophages to serum (anemia
of chronic inflammation or malignancy), failure of protoporphyrin synthesis
(sideroblastic anaemia) or of globin synthesis (α- or β- thalassemia)
Developmental Stages of Iron
Deficiency
Iron depletion
 storage iron decreased or absent
Iron deficiency
 storage iron decreased or absent
 low serum iron
 low transferrin saturation
Iron-deficiency anemia  storage iron decreased or absent
 serum iron
 low transferrin saturation
 low hemoglobin level
The development of iron deficiency anaemia. Reticuloendothelial (macrophage) stores are lost
completely before anaemia develops. MCH, mean corpuscular haemoglobin; MCV, mean
corpuscular volume
Causes of Iron Deficiency
 Chronic blood loss
 Pregnancy, lactation
 Inadequate dietary intake of iron
 Malabsorption of iron
Red blood cells:
• Anisocytosis,  RDW
• Mild ovalocytosis, target cells
• Hypochromia (low MCHC), microcytosis (low MCV)
• Reticulocyte N or 
Leukocytes:
• Differential WBC normal, a small number leukopenia
Platelets:
• Thrombocytosis or thrombocytopenia
Profil iron:
• SI concentration: , in mild IDA low-normal
• TIBC: , in mild IDA high-normal
• Saturation transferin (iron/TIBC) is often <15%
 not specific, may occur in inflamation (e.g.
arthritis)
• Serum transferrin receptor (sTfR): when serum
ferritin levels are borderline low, its elevation 
early stage IDA
SI, serum iron; TIBC, total iron binding capacity; IDA, iron deficiency anemia
sTfR: to evaluate suspected IDA in patients who have inflammation, infection or chronic d
Serum Ferritin:
• Levels < 10 μg/L, characteristic of IDA
• Levels 10 to 20 μg/L: presumptive (not diagnostic)
• May be ↑with concomitant inflammatory disease
(e.g., rheumatoid arthritis), chronic renal
disease, malignancy, hepatitis, or iron
administration.
• IDA can be suspected in RA or other severe
inflammatory states if the ferritin < 60 μg/L.
IDA, iron deficiency anemia; RA, rheumatoid arthritis
Blood Count in Iron Deficiency: example
Hb
RBC
PCV
7.5 g/dl
4.05 x 1012/l
26%
MCV
MCH
Reticulocytes
64 fl
18.5 pg
2.6%
WBC
differential
Platelets
7.5x109/l
Normal
530x109/l
• Diagnosis: Hb, MCV, MCH, Blood film (cell
pencil), Ferritin, sTfR
• If the diagnosis is established, search for a
source of blood loss if unapparent
• GI loss is the most prevalent of blood loss in
men, uterine/menstrual loss in women.
GI, gastrointestinal
Serum Fe
TIBC
Saturatio
n
Ferritin
BM
Stores
Sideroblast
s
Iron
deficiency
↓
↑
↓
↓↓
absent
↓
Chronic
disease
↓
N or ↓
↓
N or ↑
N or ↑
↓
Sideroblasti
c anaemia
N or ↑
N
N or ↑
↑
↑
↑
ring
sideroblasts
Thalassaem
ia
N or ↑
N
N or ↑
↑
↑
↑
Haemochro
matosis
↑
N
↑
↑↑↑
↑↑↑
N or ↑
• 150-200 mg elemental iron in 3 to 4 doses, 1 hour
before meals (65 mg of elemental iron is contained
in 325mg of ferrous sulfate USP, or in 200 mg dried
ferrous sulfate)
• Do not give with meals or antacids or with inhibitors
of acid production.
• A few patients may complain of GI intolerance to
pills, pyrosis, constipation, diarrhea, and/or metalic
taste and require  daily dose, change of oral iron
preparation.
Macrocytic Anemia
• Macrocytic anemia: RBC abnormally large
(MCV>95fL).
• Macrocytic anemia:
1. Anemia megaloblastic
2. Anemia non-megaloblastic
• A group of anemia in which erythroblast in BM
show abnormality-maturation of the nucleus
(delayed relative to cytoplasma), due to
defective DNA synthesis, caused by deficiency
of vit. B12 or folate.
BM, bone marrow
• Found in food of animal origin: liver, meat, fish
• Absorbtion: distal ileum
• Vit B12 – glycoprotein IF (synthesized by
gastric parietal cells)  bind to cubilin
(receptor for IF)  direct endocytosis the cubilin
IF-B12 complex in the distal ileum, where B12 is
absorbed and IF destroyed.
• Transport: transcobalamins, which delivers B12
to BM
BM, bone marrow, IF, intrinsic factor
Causes of severe vitamin B12 deficiency
Nutritional
 Vegan
 Malabsorption
 Congenital lack or abnormality of intrinsic factor
(IF)
 Total or partial gastrectomy
Intestinal causes
 Ileal resection
 Crohn’s disease
Causes of Folate Deficiency
Nutritional
Old age
 Proverty
 Malabsorbtion
Pregnancy, lactation,
 Hemolytic anemias
 Myelofibrosis
 Liver disease
 Inflammatory disease:
 Crohn’s disease
 TBC
 Rheumatoid arthritis
 Psoriasis
 Exfoliative dermatitis
 Malignant disease:
Carcinoma
 myeloma
 lymphoma
The absorption of dietary vitamin B12
after combination with intrinsic factor
(IF), through the ileum.
Folate absorption occurs through the
duodenum and jejunum after
conversion of all dietary forms to
methyltethrahydrofolate (methyl THF).
TC, transcobalamin.
•
•
•
•
•
•
Signs of anemia
Jaundice (mild)  the excess breakdown Hb
(ineffective erythropoiesis in BM)
Glossitis, angular stomatitis
Mild symptoms of malabsorbtion (loss of
weight)
Purpura (thrombocytopenia)
Many asymptomatic patients are diagnosed
when a blood count has been perormed for
another reason (reveal macrocytosis).
Vit. B12 deficiency
Folate deficiency
Compound
Hydroxycobalamin
Folic acid
Route
Intramuscular
Oral
Dose
1.000 ug
5mg
Initial
6x 1.000ug over 2-3
weeks
Daily for 4 month
Maintenance
1.000 ug every 3 months
Life long therapy in:
chronic inherited
hemolytic anemias,
myelofibrosis, renal
dialysis
Prophylactic
Total gastrectomy
Ileal resection
Pregnancy, severe
hemolytic anemias,
dialysis, prematurity.
Hemolytic anemia
•
•
•
Anemia that results from an increase in the rate
of RBC destruction.
The normal adult marrow, is able to produce
red cells at 6-8 times the normal rate.
It leads to a marked reticulocytosis
Warm type
Autoimmune
Idiopathic
Secondary
SLE, other autoimmune
disease
CLL, lymphomas
Drugs (e.g. Methyldopa)
Alloimmune
Induced by red cell antigens
Hemolytic transfusion reaction
Cold type
Idiopathic
Secondary
Infections: Mycoplasma
pneumonia, infectious
mononucleosis
Lymphoma
• AIHA cause by antibody production by the body
against its own red cells.
• Characterized by a positive DAT (Coombs’ test)
• Antibody reacts more strongly with RBC at 370C
(warm type) or 40C (cold type)
AIHA, autoimmune hemolytic anemia; DAT, direct antiglobulin test
• The RBC coated with IgG alone or with
complement,  taken up by macrophages
(have receptor for IgFc fragment)
• Part of the coated membrane is loss the cell
becomes spherical (sperocyte)  prematurely
destroyed, predominantly in the spleen.
Some of the more frequent variations in size (anisocytosis) and shape (poikilocytosis) that may
be found in different anaemias. DIC, disseminated intravascular coagulopathy; G6PD, glucose6-phosphate dehydrogenase; HUS, haemolytic uraemic syndrome; TTP, thrombotic
thrombocytopenic purpura.
•
•
•
•
•
•
Pallor
Jaundice
Splenomegaly
Urine dark (excess urobilinogen)
The disease tends to remit and relapse
When associated with ITP: Evan’s syndrome
ITP, immune thrombocytopenic purpura
• Remove the underlying cause
• Corticosteroids
• Immunosuppressive: azathioprine,
cyclophosphamide, ciclosporin, mycophenolate
mofetyl
• Folic acid
• High-dose immunoglobulin
• The autoantibody: monoclonal or polyclonal
attach to RBC where the blood temperature is
cooled.
• Antibody (IgM), binds to RBC best on 40C.
• IgM Abs are highly efficient fixing complement 
extra- and intra-vascular hemolysis.
• A chronic hemolytic anemia aggravated by the
cold
• Often associated with intravascular hemolysis
• Mild jaundice
• Splenomegaly
• Acrocyanosis (purplish skin discoloration at the
tip of nose, ears, fingers and toes, caused by
agglutination of RBC in small vessels
• Keeping the patient warm
• Treating the underlying cause
• Chlorambucyl
Aplastic Anemia
Aplastic Anemia (AA)
(Panmyelopathy, Panmyelophtysis)
 Definition: a group of pathogenitically heterogenous
bone marrow (BM) failure. Bi- or tricytopenia (anemia,
granulocytopenie, thrombocytopenia) due to hypoplasia
or aplasia of the BM.
 Incidence: in Europe 2-3/1.000.000/year. Age
distribution between 10-25 years and over 60 years
 Classification:
o Moderate AA or non severe AA (MAA or nSAA)
o Severe AA (SAA)
o Very severe AA (vSAA)
Classification of aplastic Anemia
(two out of three criteria must be fullfilled)
nSAA
Neutrophils
<1.0
Platelets
<50
Reticulocytes <2.0
SAA
<0.5
<20
<2.0
vSAA
<0.2
<20
<2.0
Classification based on the presumed etiology:
 Idiopathic: > 80%
 Drug-induced: <20%
 Post-infectious (after hepatitis): <5%
 Hereditary forms : <1%
Clinical presentation


o
o
o
o

Anemia
Neutropenic infection:
oral cavity and pharyngeal ulcers)
necrotizing gingivitis or tonsilitis
Pneumonia
phlegmon
Bleeding due to thrombocytopenia
Diagnosis
Parameter
Discription
Comments
Differential
blood count
Bi- or tri-cytopenia
• Anemia N,N/ moderately macrocytic
• Leukocytopenia resulting from
granulocytopenia and
monocytopenia.
• Absence giant platelet in blood
smear
Bone Marrow
•
• BMA and BMB are mandatory
• Biopsy length at least 15mm
•
•
Aplasia or
hypoplasia,
cellularity <25%
No infiltration of
neoplastic cells
Without fibrosis
N,N, normochrom-normocyter; BMA, bone marrow aspiration,
BMB, bone marrow biopsy
Differential diagnostics and diagnosis by
exclusion






Hypoplastic acute leukemia
Myelodisplatic syndrome
Hairy cell leukemia and other lymphoma
BM infiltration by solid tumors
Hypersplenism
Aplasia after chemotherapy or radiation therapy
Therapy
Object of therapy:
 Induction of “remission”
 Prevention bleeding and neutropenic infections
 Prevention of chronic transfusion requirement
(iron overload, allosensitization)
Therapy AA
Age < 40-50 years:
 HLA Identical sibling donor: BMT
 No HLA Identical sibling donor
o ATG/CsA
Age ≥50 years:
o ATG/CsA or CSA
ATG, anti thymocyte globulin
CsA, Cyclosporin A
BMT, bone marrow transplant
Supportive Therapy

•
•
•

•
•
•
Infection prophylaxis:
Reverse isolation
Air filtration
Prophylactic antibiotics and antimycotics
Bleeding prophylaxis:
Avoid platelet aggregation inhibitor
Tranexamic acid
Platelet transfusion
Transfusion
 Leukocyte-depleted blood products: mandatory in AA
patients
 Erythrocyte concentrates: in case of signs hypoxic
anemia.
 Platelet-transfused prophylactically:
1. Stable out-patients without risks (fever, infections):
<5.000/μl with regular blood count at least once a week
2. Fever (>380C), infections, signs of bleeding, history
severe hemorrhages (WHO grade 3 or 4) transfusion
trigger <20.000/μl
Iron chelation:
Patients with regular transfusion become a persistent
requirement when serum ferritin >1.000ng/ml
Download
Random flashcards
hardi

0 Cards oauth2_google_0810629b-edb6-401f-b28c-674c45d34d87

Rekening Agen Resmi De Nature Indonesia

9 Cards denaturerumahsehat

Rekening Agen Resmi De Nature Indonesia

9 Cards denaturerumahsehat

Nomor Rekening Asli Agen De Nature Indonesia

2 Cards denaturerumahsehat

Create flashcards