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RBC
Anemia?
Thalassemia
N
Sideroblastik
Mikrositik
Hipokromik
Besi Serum
MCV
Anemia
Normositik
normokromik
Retikulosit
MCV Normal
Defisiensi
folat
Makrositik
MCV
N/
Defisiensi
Besi
Penyakit
Kronik
Anemia
hemolitik
Perdarahan
Akut
Anemia
Aplastik
Leukemia, etc
Defisiensi
B12
Anisositosis
Mikrositik (<7 mikron)
MCV rendah
Anemia megaloblastik
Anemia pernisiosa
Poikilositosis
Sferosit
Sferositosis
herediter
Elliptosis (Ovalosit)
Sel pensil
Thalassemia
Poikilositosis
Sel Target (Mexican Hat cell,
bulls eye cell)
Thalassemia
Penyakit hati kronik
Stomatosit
Stomatosis herediter
Keracunan timah
Alkoholisme akut
Poikilositosis
Sel Sabit (sickle cell;
drepanocyte; cresent cell;
menyscocyte)
Hemoglobinopati
Anemia hemolitik
Penyakit keganasan
Poikilositosis
Sel Spikel
Akantosit (Spurr cell)
Anemia Mi-Hi
Anemia Mi-Hi
ADB
Normal
Terapi ADB
Lini Pertama Terapi Besi Oral
Ferro sulfat mengandung 20 % besi elemental
Sediaan 200 mg, 325 mg (65 mg besi elemental)
Ferro fumarat mengandung 33 % besi elemental
Sediaan 325 mg (107 mg besi elemental)
Ferro glukonat mengandung 12 % besi elemental
Sediaan 325 mg (39 mg besi elemental)
Antasida
Fitat (pada sereal)
Tanin (pada teh)
Fosfat
Daging
Senyawa sitrat
Fruktosa
Asam askorbat
Anemia Mi-Hi
Anemia Sideroblastik
Genetic (X-linked
or AD) or
acquired
(myelodysplasia
syndrome)
Sideroblast:
nucleated
erythroblast
Ring: iron in
perinuclear
mithocondria
Iron (+) but
cannot corporate
it to Hb
Thalassemia
N
Sideroblastik
Mikrositik
Hipokromik
Besi Serum
MCV
Anemia
Normositik
normokromik
Retikulosit
MCV Normal
Defisiensi
folat
Makrositik
MCV
N/
Defisiensi
Besi
Penyakit
Kronik
Anemia
hemolitik
Perdarahan
Akut
Anemia
Aplastik
Leukemia, etc
Defisiensi
B12
Anemia Hemolitik
Curiga anemia hemolitik:
Klinis: Anemia, Jaundice, Splenomegali
Lab: Retikulosit , Bilirubin indirek
Hemolisis
Letak
Extravascular
(90%)
Intravascular
(10%)
Reticuloendothelial (RE)
system
Penyebab
Intrinsik
Extrinsik
Membran
Autoimun
Enzim
Infeksi
Hemoglobin
Microangiopathy
Intrinsik
Membran
Hereditary
spherocyte
Osmotic fragility
test
Enzim
G6PD deficiency
G6PD assay
Thalassemia
Hb
elektroforesis
Hemoglobin
Sickle cell
Intrinsik
Membran
Hereditary
spherocyte
Osmotic fragility
test
Enzim
G6PD deficiency
G6PD assay
Thalassemia
Hb
elektroforesis
Hemoglobin
Sickle cell
Membranopathy
Hereditary Spherocytosis
MDT Spherocytes
Osmotic fragility test
Splenectomy often very effective
Intrinsik
Membran
Hereditary
spherocyte
Osmotic fragility
test
Enzim
G6PD deficiency
G6PD assay
Thalassemia
Hb
elektroforesis
Hemoglobin
Sickle cell
Enzymopathy
G6PD Deficiency
G6PD Deficiency
Harrisons Principles of Internal Medicine 17 Edition, Part 7 Oncology & Hematology, Section 2 Hematopietic Disorders
Intrinsik
Membran
Hereditary
spherocyte
Osmotic fragility
test
Enzim
G6PD deficiency
G6PD assay
Thalassemia
Hb
elektroforesis
Hemoglobin
Sickle cell
Hemoglobinopathy
Hemoglobin Deffect
Thalassemia
Hb elektroforesis
What is Thalassemia?
Inherited disorders
Defective hemoglobin chains
The two main types:
Alpha
Beta more severe
a Thalassemia minor: often no target cells, but an increase in the number of small erythrocytes
(shown here in comparison with a lymphocyte), so that sometimes there is no anemia.
b More advanced thalassemia minor: strong anisocytosis and poikilocytosis (1), basophilic
stippling (2), and sporadic target cells (3).
Thalassemia
Splenomegaly
Thalassemia
Chronic
hemolysis
Iron overload
Tissue damage
Mechanism
Excess iron free hydroxyl radicals ROS
Insoluble iron complexes deposited in
body tissues
Clinical sequelae of iron overload
Pituitary impaired growth
Heart
cardiomyopathy, heart failure
Liver
hepatic cirrhosis
Pancreas diabetes mellitus
Gonads hypogonadism, infertility
+
IRON CHELATING
Deferiprox = 75
mg/kg/hari, dibagi 3
dosis, per oral
Extrinsik
Microangiopathy
Infeksi
Prosthetic
valves etc
Malaria, etc
Cold (30 %)
Maximally bind
RBCs at
37C
0 to 4C
Clinical
Mediated by
autoandibodies
IgG
Mechanism
Treatment
Corticosteroid
Splenectomy (if steroid isnt
adequate)
Avoidance of cold
Corticosteroid and splenectomy
wasnt effective
Coombs Test
Microangiopathic Anemia
Cause:
Microvascular disease (DIC, TTP etc)
Heart valve prostheses
Trauma / implanted devices
Anemia Aplastik
Pansitopenia yang disertai
hiposelularitas sumsum
tulang
Etiologi =
idiopatik (sebagian besar),
Radiasi
Obat & bahan kimia
kemoterapi, benzena, gold,
kloramfenikol,hidantoin,
carbamazepine
Infeksi virus hepatitis,
virus Ebstein-Barr, CMV,
parvovirus, HIV-1
Penyakit imun
Hemoglobinuria
paroksismal nokturnal
Kehamilan
Perdarahan mukosa
Trombositopenia
Perdarahan bawah kulit (memar)
Leukopenia
Anemia Aplastik
Thalassemia
N
Sideroblastik
Mikrositik
Hipokromik
Besi Serum
MCV
Anemia
Normositik
normokromik
Retikulosit
MCV Normal
Defisiensi
folat
Makrositik
MCV
N/
Defisiensi
Besi
Penyakit
Kronik
Anemia
hemolitik
Perdarahan
Akut
Anemia
Aplastik
Leukemia, etc
Defisiensi
B12
Megaloblastic Anemia
18
Komponen Darah
Whole
Blood
Komponen lengkap
Perdarahan masif
atau hipovolemik
Packed Red
Cell (PRC)
Washed
Red Cell
Eritrosit yang
dipisahkan dari
plasma
Anemia tanpa
penurunan
komponen darah lain
Penderita transfusi
berulang atau
mengalami demam
pada transfusi
sebelumnya
18
Komponen Darah
Platelet
Concentrate
Fresh Frozen
Plasma
Cryoprecipitate
Trombosit
All coagulation
factor
Fibrinogen, von
Willebrand factor,
factor VIII, factor XIII
and fibronectin.
Trombositopenia,
profilaksis (operasi),
Multiple coagulation
factor deficiency,
DIC
Haemophilia A, Von
Willebrands
disease,
Hipofibrinogenemia
Stab
Neutrophil
coagulation
cascade
waterfall
PT
APTT
TT
Fibrinolysis system
20
ITP
Terapi ITP
ITP: antiplatelet antibody platelet destruction
Kortikosteroid
Indikasi : AT < 20.000 atau perdarahan
Prednisone 1 mg/kg/hari (2 minggu)
IvIg
Indikasi : severe, life-threatening bleeding, atau anak dengan AT
<20.000 dengan perdarahan minor
Platelet
Not indicated unless there is significant bleeding. In ITP transfusion
increments are usually poor and platelet survival is short
Evans Syndrome
Definisi
AIHA (direct Coombs test +) dan immune-mediated
thrombocytopenia tanpa adanya penyebab yang mendasari
Manifestasi Klinis
Trombositopenia (76%), anemia (67%), neutropenia (24%),
pansitopenia (14%), hemolitik
Manajemen
Stabilisasi fungsi kardiovaskular dan respirasi
Transfusi PRC
Prednison (first line), IvIg
Henoch-Schonlein Purpura
Definisi
Gangguan imun akut yang diperantarai oleh IgA vaskulitis general yang melibatkan
pembuluh darah kulit, GI tract, ginjal, sendi, dan paru / CNS (jarang)
Manifestasi Klinis
Gejala prodromal : sakit kepala, anorexia, demam
Ruam (95-100% kasus), terutama melibatkan tungkai. Lesi urtikaria atau macula
eritematosa, berkembang menjadi papul pucat lalu purpura
Nyeri perut dan muntah
Nyeri sendi, terutama di tungkai bawah dan ankle
Edema subkutan, edema skrotum
Feses berdarah
Manajemen
Suportif, analgetik, corticosteroid
Henoch-Schonlein Purpura
Polisitemia
Polisitemia Vera
Kelainan neoplastik yang ditandai dengan peningkatan eritorosit (disertai
dengan leukositosis dan trombositosis)
Sering tidak bergejala (asimptomatik)
Peningkatan volume dan viskositas eritrosit sakit kepala, malaise,
dyspnea, fatigue, gangguan penglihatan
Peningkatan turnover sel darah hiperurisemia risiko gout dan batu
saluran kemih
Hiperviskositas trombosis stroke, TIA, DVT, infark miokard, oklusi
arteri vena retina
Polisitemia
KATEGORI B
1. Trombositosis >400.000/mm3
2. Leukositosis >12.000/mm3 (tanpa
demam/infeksi)
3. Leukocyte alkaline phosphatase score
>100
4. Serum vitamin B12 >900 pg/ml atau
serum UB12BC >2.200 pg/ml
KRITERIA DIAGNOSIS PV
KEGANASAN HEMATOLOGI
Leukemia
CBC
Hb
AL
AT
Acute
Chronic
(anemia
(anemia)
(leukositosis)
(leukositosis)
(trombositopenia)
- N/
- in CML blast crisis
Myeloid (AML)
Lymphoid (ALL)
Myeloid
(CML)
80-90% case
Adult & children
Myeloblast >20%
Auer rod (+)
Adults
Children>>
Philadelphia
Limfoblast >20% chromosom
Lymphoid CLL
>55 yo
Limfositosis
>50rb
AML M1:
AML without
maturation
AML-M3
promyelocytes
Chronic
phase
several years
Blast
transformation
Accelerated
phase
triphasic
biphasic
several years
Fase:
Kronik: blast <5%
Accelerated: blast >15%
Acute/Blast crisis: blast >30% (mirip AML)
Lymphoma Non-Hodgkin
Sering ekstranodal
Hodgkin Lymphoma
Owls Eyes
Reed Stenberg cell (+)
Hodgkin Lymphoma
TERIMA KASIH