PERUBATAN 1

SISTEM KARDIOVASKULAR

GANGGUAN
HAEMOPOIETIK
 GANGGUAN
HEMOPOIETIK

DEFINISI:

Gangguan proses penghasilan sel-sel

darah dan platelet yang berterusan
sepanjang hayat kehidupan.
 Penjelasan
• Proses ini berterusan bagi
menggantikan sel-sel yang berumur
yang dikeluarkan dari sirkulasi darah.
• Dewasa – berlaku di sum-sum tulang
• Embrio & awal infant –
ekstramedullari (medula oblongata)
Medula Oblongata
 Topik
1. Anemia
2. Leukemia
3. Thrombositopenia & Hemofilia
4. Thalasemia
5. Jaundis Hemolitik
 Anemia
Keadaan di mana terdapat
kekurangan atau kadar rendah
konsentrasi hemoglobin (sel darah
merah) dalam darah periferi
bawah paras normal mengikut
umur dan jantina.

Male Female
< 130 g/L < 115 g/L
(13.0 g/dL) (11.5 g/dL)
 Anemia
Male Female

Kurang dpd Kurang dpd

130 g/L 115 g/L
(13.0 g/dL) (11.5 g/dL)
Hemoglobin
 Klasifikasi
1. Mengikut Punca
2. Mengikut morfologi sel darah merah
 Klasifikasi
1. Mengikut punca :
a. Kehilangan darah akut atau
kronik (perdarahan, infestasi
cacing kerawit)
b. Anemia Hemolitik
c. Ketidakseimbangan hasilan sel
darah merah – kecacatan
proliferasi dan pembahagian sel
“stem”. Cth : anemia aplastik
atau kegagalan ginjal kronik.
d. kecacatan proliferasi dan pematangan
pembahagian eritrosit. Cth :
kekurangan vit B12, asid folik, zat
besi dan talasemia.
e. Anemia gangguan sum-sum tulang
f. Keperluan meningkat seperti
pramatang, semasa tumbesaran dan
mengandung
Red blood cells
Infestasi cacing
Sum-sum tulang
Sel-sel darah merah
Sickle cell Anaemia
 Eliptocytosis
(sel bentuk bujur)
2. Mengikut morfologi sel darah merah
a. Anemia normositik (MCV 76-96 fl.
MCHC 30-35g/dl)
- kehilangan darah akut : pendarahan,
kecederaan
- Penyakit hepar
b. Anemia makrositik (MCV lebihi 96 fl.
MCHC 30-35g/dl
- kekurangan vit B12 dan asid folik
- halangan ubat keatas sintesis DNA
c. Anemia mikrositik (MCV kurang 76 fl.
MCHC 30g/dl)
- kekurangan zat besi
- Penyakit thalassemia
Makrosit
Mikrositosis
 Patofisiologi
• Pluripotential stem cell – Erythroid
Progenitor – Pronormoblast – Normoblast –
Reticulocytes (immature red cell).
• Proses ini akan distimulasikan oleh tensi
oksigen di dalam tisu ginjal. Untuk
pembentukan bahan hemoglobin seperti zat
besi, manganese or cobalt; vitamin spt B12,
folate, C, E, B6, thiamine dan riboflavine;
asid amino dan hormon spt erythropoietin,
androgens dan thyroxine diperlukan.
 Manifestasi Klinikal
1. Boleh bermula secara akut atau kronik
2. Persembahan am spt:
- Kelemahan, letih lesu, edema, pucat,
kulitkering
3. Mk sistem kardiovaskular spt:
- Palpitasi, dispnea, sakit angina,
takikardia sinus, “fungtional systolic
murmur”, kardiomegali
4. Mk sistem alimentari spt:
-Anoreksia, heart burn, lidah glossitis
atau kudis,
splenomegali, hepatomegali
5. Mk. Sistem saraf pusat spt:
- Dizziness, Giddiness, Tingling
sensation, kebas, insomnias, dimness
of vision, pelupa, kurang konsentrasi.
6. Mk Sistem Pembiakan spt:
- Amenorrhea, Menorrhagia, Abortion
7. Kuku – Koilonychias
8. Rambut - nipis
 Investigasi
1. Full blood count- mengesan anemia,
thrombositopenia dan jangkitan
2. Full blood picture- kesan morfologi
sel darah
3. MCV- kesan jumlah sel darah merah
4. MCHC- kesan isipadu sel darah merah
5. Serum vit B12- kesan vit B12 dlm
darah
6. Bone marrow aspiration – for FBC &
FBP
7. Gastric analysis – Mengesan
ahidrokloria
8. Serum Bilirubin – Mengesan
jaundis
9. Renal Profile – Mengesan fungsi
ginjal
 Nilai normal
sel darah merah
Lelaki Wanita

Red cell count 4.4-6.1 4.2-5.4

Hg (g/dl) 13.0-18.0 11.5-16.5

Haematocrit
0.40-0.54 0.37-0.47
(PCV)
 Dalam keadaan anemia

Kekurangan
Anemia - Makrositik
Iron
Red cell
2.7 4.9
count
Hg (g/dl) 10.4 10.4
Haematocrit
0.32 0.35
(PCV)
Diagnosis Perbezaan

1. Thalassemia
2. Leukemia
3. Hemofilia
4. Penyakit Hodgkin
5. Mieloma Multipel
6. Barah
 Pengendalian kes
1. Rawatan spesifik:
- rawatan punca
- terapi zat besi- Sulfat ferrous 200mg
BD/TDS – I.V dextran-zat besi 100-200
mBD- suntikan imferon 2-5 ml harian –
Transfusi darah bagi kes teruk.
- terapi vit B12, asid folik, B6, Thiamine
dan Riboflavine
- Steroids
2. Rawatan simptomatik
- sedation utk rehat dan tenangkan
pesakit
- Vitamin B Kompleks
- Losyen atau Cream utk kulit kering
3. Penjagaan kejururawatan
- Rehat atas katil
- Mengawal tekanan darah, nadi,
kadar pernafasan dan suhu badan
- Diet – High Protein dan high
calorie
- Kebersihan diri.
 Komplikasi
1. Kegagalan Jantung
2. Tumbesaran Terbantut
3. Pertahanan badan rendah (mudah
dijangkiti)
 Pendidikan Kesihatan
1. Mengambil ubat mengikut arahan
doktor
2. Datang untuk rawatan susulan
ditetapkan
3. Makanan berzat – terutama zat besi
4. Rehat secukupnya
5. Amalkan cara hidup sihat
 Prognosis
• Bergantung kepada punca
- Baik jika puncanya kehilangan
darah- transfusi darah
- Baik jika puncanya ialah infestasi
cacing
- Tidak baik jika puncanya-
barah/leukemia/thalassemia
 ANAEMIA

PENGURANGAN PENINGKATAN
PENGHASILAN KEHILANGAN
SDM /PEMUSNAHAN SDM

Bone marrow failure Ineffective

or replacement erythropoiesis

•Aplastic anaemia •Iron deficiency anaemia • Haemorrhagic

•Sideroblastic anaemia
•Anaemia of chronic anaemias
disorders •Vitamin B12 deficiency
• Haemolytic
anaemia
•Anaemia due to bone •Folate deficiency
anaemias
marrow infiltration anaemia
Sekian
Terimakasih
 ANAEMIA

Decreased red Increased red cell

cell production loss/destruction

Bone marrow failure Ineffective

or replacement erythropoiesis
Aplastic anaemia
Iron deficiency anaemia
Anaemia of Haemorrhagic
Sideroblastic anaemia
chronic disorders anaemias
Vitamin B12 deficiency
Anaemia due to anaemia Haemolytic
bone marrow Folate deficiency anaemias
infiltration anaemia
Morphological classification
Normocytic normochromic
MCV 76-96 fl MCH 27-32 pg

Increased retic Reticulocyte

count count +/-
•Acute bleeding •Anaemia of
chronic disease
•Haemolytic
e.g. chronic
anaemias osteomyelitis,
renal failure,
rheumatoid
arthritis
Morphological classification
Microcytic hypochromic
MCV <76 fl MCH <27 pg

Iron stores Iron store

absent (Low present
ferritin) •Sideroblastic
•Iron anaemia
deficiency •Thalassaemia
•Chronic
diseases (some
 Morphological classification
Macrocytic
MCV >96 fl

Megaloblastic Normoblastic
•Vitamin B12 •Alcoholism
deficiency •Liver disease (retic
+/-)
•Folic acid
deficiency •Hypothyroidism
•Haemolytic anaemia
(retic )
•Acute blood loss
(retic )
 Patofisiologi
• Pluripotential stem cell berubah menjadi
erythroid progenitor kemudian ke
pronormoblast kemudian ke normoblast
dan reticulocytes matang.Process
dirangsang oleh oxygen tension dalam
ginjal.Untuk menghasilkan hemoglobin
iron,manganese or cobalt,vitamins such
as B 12,folate,vit C,E,B 6,thiamine and
riboflavine,amino acids and hormones
such as eryhtropoietin,androgens and
thyroxine diperlukan.
 Clinical Manifestations
• Boleh bermula secara akut atau kronik
• Persembahan am seperti
• Kelemahan
• Letih lesu
• Headache
• Edema buku lali-fungsi jantung terjejas
• Pucat-conjunctivae, oral mucosa, palmar
creases, nail beds
• Kulit kering
• Manifestasi klinikal CVS seperti
• Palpitasi
• Exertional dyspnea
• Sakit angina
• Takikardia sinus (bounding pulses)
• Functional systolic murmur
• Kardiomegali
• Manifestasi klinikal alimentari
• Anoreksia
• Heart burn
• Lidah-glossitis atau kudis/shiny and
smooth, loss of papillae-folate
deficiency
• Splenomegali
• Hepatomegali
• Jaundice-haemolytic /megaloblastic
anaemia
• Manifestasi klinikal CNS
• Dizziness
• Giddiness
• Tingling sensation
• Kebas
• Dimness of vision
• Pelupa
• Kurang konsentrasi
• Manifestasi sistem pembiakan
• Amenorrhoea
• Menorrhagia
• Abortion
• Kuku-Koilonychia (spoon nails)iron
deficiency
• Rambut-nipis
• Leg ulcer-sickle cell anaemia
Splenomegaly
 Laboratory Investigations
• FBC-mengesan anemia,thrombositopenia
dan jangkitan,eosinophilia-cacing
hookworm
• Full Blood picture-mengesan
morfologi darah
• Reticulocyte count-mengesan jumlah sel
darah merah
• MCV-low
• MCHC-low
• Serum vitamin B 12-mengesan
vitamin B 12 dalam darah
• Gastric analysis-mengesan
anhidrokloria
• Serum bilirubin
• Renal profile
 Diagnosis perbezaan
• Thalassemia
• Leukemia
• Hemophilia
• Penyakit Hodgkin
• Myeloma multiple
• Barah
 Pengendalian
• Rawatan spesifik
• Rawat punca
• Terapi zat besi-Hb 40-80%
• Sulfat ferrous 200mg bd/tds
• Iv dextran-zat besi 100-200 mg bd(5xWxG)
• Suntikan imferon 2-5 ml harian
• Transfusi darah bagi keadaan yang teruk Hb
< 40%
• Terapi vitamin B 12 1000mcg(20 mcg/kg
bw),asid folik 4 mg,B 6,thiamine dan
riboflavine
• Rawatan simtomatik
• Berikan sedation untuk rehat dan
tenangkan pesakit
• Vitamin B Co
• Losyen atau krim untuk kulit kering
• Penjagaan kejururawatan
• Rehat atas katil
• Mengawal tekanan darah,nadi,kadar
pernafasan dan suhu badan
• Diet-high protein dan high calori
• Kebersihan diri
 Komplikasi
• Kegagalan jantung
• Tumbesaran terbantut
• Pertahanan badan rendah
 Pendidikan kesihatan
• Mengambil ubat ikut arahan doktor
• Datang rawatan susulan ikut tarikh
dan waktu yang ditetapkan
• Makan makanan berzat terutama
zat besi
• Rehat secukupnya
• Amalkan cara hidup sihat
 Prognosis
• Bergantung kepada punca
• Baik jika puncanya kehilangan
darah-tambah balik darah
• Baik jika puncanya infestasi cacaing
• Tidak baik jika puncanya adalah
barah/leukemia/thalassemia
 Iron deficiency anaemia

• A decrease in the red cells of the

blood caused by too little iron.

• Most common form of anaemia

– 20% of women
– 50% of pregnant women
– 3% of men
Causes
• Poor dietary iron
• Poor absorption of iron
• Loss of blood
– Gastrointestinal blood loss (due to
ulcers or aspirin or NSAIDS).
– Colon cancer
– Uterine cancer or heavy menstrual
bleedings.
In the condition known as koilonychia (spoon nails),
the nails are flattened and have concavities. This
condition may be associated with iron deficiency
 Ix
• Hb
• MCHC <3%
• RBC-microcytic dan hypochromic
• MCV-decreased
• MCH < 30%
• Se iron < 30 mcg/dl
• Stool ova & cyst-ankylostoma
• Stool occult blood
• Barium meal X ray-GIT bleed
Target cells
• These abnormal red
blood cells (RBCs)
resemble targets.
These cells are seen
in association with
some forms of
anaemia e.g. iron
deficiency anaemia,
and following the
removal of the
spleen
(splenectomy).
Management:
• Identify underlying disease-e.g.
colon cancer, bleeding
esophageal varices, menorrhagia
• Iron supplement-ferrous fumarate
300 mg dly/ferrous sulfate. 1.5-
2mg/kg bw tds
– Vitamin C can increase absorption
and is essential in the production
of Hb.
• Intravenous or intramuscular iron
Imferon 50mg in D5%
• Iron-rich foods -raisins, meats
(liver is the highest source), fish,
poultry, eggs (yolk), legumes (peas
and beans), and whole grain bread.
 Megaloblastic anaemia
Definition
• A blood disorder characterized by
red blood cells that are larger than
normal, low white blood count, and
low platelet count resulting from a
deficiency of folic acid or vitamin
B12.
• Most common causes:
– Deficiency of vitamin B12
– Deficiency of folic acid
• Other causes:
– Leukemia
– Myelofibrosis
– Multiple myeloma
– Certain hereditary disorders
– Drugs that affect nucleic acid
metabolism such as chemotherapy
agents (methotrexate)
 Deficiency of vitamin B12
• Pernicious anemia
– Lack of intrinsic factor HCL/pepsin
untuk penyerapan vit B12)
– Associated with
• Autoimmune endocrine diseases
such as type I diabetes,
hypoparathyroidism, Addison’s
disease, hypopituitarism,
testicular dysfunction, Graves’
disease, chronic thyroiditis,
Manifestasi
Onset insidious
Tidak ambil rawatan sehingga serius
Demam
Pallor/jaundice
Abdominal pain
Splenomegali
• tongue -- smooth and beefy red
because of atrophic glossitis.
• diarrhoea and malabsorption.
 Ix
• Gastric analysis-achlorhydria
• RBC kurang
• Hb moderately low
• MCH-meningkat
• MCV- meningkat
• Pbf- normoblast kurang megablat
meningkat
• TW 3000-4000
• Bone marrow large cells
 Rx
• Inj B 12 1000 microgram dly seumur
hidup
 Complication
• Ca stomach
• Degeneration of spinal cord
 Anaemia of folate deficiency
• A decrease in the red cells in the
blood caused by folate (folic acid)
deficiency.

Causes
• poor dietary intake and overcooked
food
• chronic alcoholism
• malabsorption diseases such as
Sickle cell anaemia

This
photomicrograp
h of red blood
cells (RBCs)
shows sickle-
shaped RBC.
Spherocytosis
Spherocytosis is a
hereditary disorder
of the red blood
cells (RBCs), which
may be associated
with a mild anaemia.
Typically, the
affected RBCs are
small, spherically
shaped, and lack
the light centers
Eliptocytosis
Eliptocytosis is a
hereditary
disorder of the
red blood cells
(RBCs). In this
condition, the
RBCs assume an
elliptical shape,
rather than the