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TATALAKSANA TERKINI

ITP PADA ANAK

Amirah Zatil Izzah

Divisi Hematologi-Onkologi Anak


FK Unad/RSUP Dr. M. Djamil Padang

1
OUTLINE
Definisi

Diagnosis

Tatalaksana newly diagnosed ITP

Tatalaksana ITP Persisten/Kronik

Ilustrasi Kasus

2
Imun TrombositoPenia (ITP )
_________________________________________________________________________

Definisi

q Kelainan autoimun yang ditandai dengan hitung trombosit yang rendah


(< 100.000/mm3) à “trombositopenia terisolasi”
q Perdarahan mukokutaneus
q Dapat terjadi sendiri (primer) atau berhubungan dengan penyakit lain
(sekunder)

Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30


3
Fase Penyakit
_________________________________________________________________________

Newly Diagnosed

• Dalam 3 bulan sejak diagnosis

Persisten

• Antara 3-12 bulan sejak diagnosis

Kronik

• Berlangsung lebih dari 12 bulan

Provan D, et al. Blood.2010;115:168-86


4
Manifestasi Klinis
_________________________________________________________________________

q Bervariasi:
q Ptekie atau purpura (86%)
q Nasal (20%)
q Oral (9%)
q Tanpa perdarahan/asimptomatis (9%)
q Menstruasi, saluran cerna, saluran kemih (3%)
q Perdarahan intrakranial (<0,8 %)

Kuhne T, et al. haematologica. 2011; 96:1831-7


5
Pemeriksaan Penunjang
_________________________________________________________________________

Pemeriksaan Hasil

Darah perifer lengkap Trombositopenia terisolasi


hitung jenis dalam rentang normal
Gambaran darah tepi Trombosit sedikit, large platelet ( > ½ diameter eritrosit)
Tidak ada blast
Tidak ada kelainan pada morfologi eritrosit

Hitung retikulosit Dalam rentang normal (0,5 – 1,5%)


PT-aPTT Dalam rentang normal

6
Diagnosis
__________________________________________________________________

q Diagnosis Klinis

q Terlihat “sehat”

q Perdarahan mukokutaneus tanpa gejala sistemik

q Isolated thrombocytopenia

q Tidak ada penyebab lain dari trombositopenia, seperti:


§ Drug-induced thrombocytopenia
§ Trombositopenia herediter, dll

Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30


7
Derajad Perdarahan
___________________________________________________________________________

Table 8. Updated bleeding scale for pediatric patients with ITP


Grade Bleeding

Grade 1 (minor) Minor bleeding, few petechiae (#100 total) and/or #5 small bruises (#3 cm in diameter), no mucosal bleeding

Grade 2 (mild) Mild bleeding, many petechiae (.100 total) and/or .5 large bruises (.3 cm in diameter), no mucosal bleeding
Grade 3 (moderate) Moderate bleeding, overt mucosal bleeding, troublesome lifestyle

Grade 4 (severe) Severe bleeding, mucosal bleeding leading to decrease in Hb . 2 g/dL or suspected internal hemorrhage

This bleeding scale is based on the one used in the previous consensus report,1 updated based on the authors’ opinion.
Hb, hemoglobin.

Most children with newly diagnosed ITP do not have significant


bleeding symptoms or other risk factors and may be managed 3. Advances.2019;3:3780-817
Provan D, et al. Blood. Any moderate (grade 3) ble
without treatment at the discretion of the hematologist and the review and consideration for
8
patient’s family (evidence levels II-III).5,245,250,251,254,255 Recent (Grade C recommendation).
Derajad Perdarahan
___________________________________________________________________________
Table 3. Bleeding grades according to the WHO and the NCI Common Terminology Criteria for Adverse Events (CTCAE v4.0) [41–43]

Bleeding grade Definition

0 No signs of bleeding

I Petechiae
Small hematomas, ecchymoses (<10 cm)
Bleeding from mucous membranes (mouth, nose)
Epistaxis (<1 h duration, no medical intervention necessary)
Subconjunctival hemorrhages
Vaginal bleeding (independent of menstruation, no more than 2 bandages/day necessary)

II Hematomas, ecchymoses (>10 cm)


(no transfusion required) Epistaxis (>1 h. duration or tamponade necessary)
Retinal bleeding without visual impairment
Vaginal bleeding (independent of menstruation, more than 2 bandages/day necessary)
Melena, hematemesis, hemoptysis, hematuria, hematochezia
Bleeding from puncture sites
Bleeding in muscles and joints

III Epistaxis
(transfusion required) Bleeding from mucous membranes (mouth, nose)
Vaginal bleeding
Melena, hematemesis, hemoptysis, hematuria, hematochezia
Bleeding from puncture sites
Bleeding in muscles and joints

IV Retinal hemorrhage with visual impairment


(life threatening, potentially permanent CNS bleeding
functional impairment) Hemorrhages in other organs with functional impairment (joints, muscles, kidneys, lungs, etc.)
Fatal bleeding (in the NCI CTCAE graded as °V)

9
Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30
Events (NCI-CTCAE) [41–43] (table 3). They are well established ‘persistent’, or ‘chronic’ should be determined not by the prior du-
Talaksana
___________________________________________________________________________

Tujuan: Pertimbangan:

§ Derajad perdarahan >>


q menghentikan perdarahan aktif
§ Faktor anak
q mengurangi risiko perdarahan ke depan § QoL
§ Aktifitas
§ Faktor keluarga/orangtua
§ Kecemasan
§ Kepatuhan berobat
§ Jarak ke RS
§ Hitung trombosit
10
Working Group. 2008;336(7650):924–926.
Thrombocytopenia. 1 More d
GRADE American
process can be found
Society in American Society
of Hematology
Strength of Recommendation
Thrombocytopenia.1 2019
Strong recommendations - Most individu
_________________________________________
Strength of Recommendation
tion. Formal decision aids are not likely to be
Strong with their values- and
recommendations
consistent Mostpreferences
individuals s
tion. Formal decision aids are
Conditional recommendations - Recogn not likely to be nee
consistent with their values and
vidual patients and that you must help each preferences.
Conditional recommendations
with his or her values and preferences. - Recognize Decisth
vidual
makepatients andconsistent
decisions that you must withhelp theireach patie
individu
with his or her values and preferences. Decision
Neunert C, et al. Blood advanced.2019;3:3829-66
make decisions consistent with their individual ris 11
was performed in July 2018. The following search terms were used:

pdf by Amirah Izzah on 15 November 2021


“immune thrombocytopenic purpura,” “idiopathic thrombocy- Given the rate of development of new treatments for ITP,
topenic purpura,” “autoimmune thrombocytopenic purpura,” the consensus report leadership will review the need for updates
“autoimmune thrombocytopenia,” “idiopathic thrombocytopenia,” each year. In addition to publication in professional journals, implemen-
International Consensus Report
“immune thrombocytopenia,” and “ITP.” Corresponding MeSH tation of the consensus recommendations will be encouraged through
distribution via patient support organizations (eg, Platelet Disorder
terms were used, in addition to searching titles and abstracts.
2019
The search was restricted to articles published from 1 January 2009
Support Association [PDSA], UK ITP Support Association),
presentations at international meetings, and through generation of
to 23 July 2018 to capture articles published since the literature an ITP treatment Web site.
_________________________________________
Table 2. Grading of evidence
Grade of recommendation Definition Level of evidence

A Requires $1 RCT as part of a body of literature of overall good quality and consistency addressing specific Evidence levels Ia, Ib
recommendation
B Requires the availability of well-conducted clinical studies but no randomized clinical trials on the topic of Evidence levels IIa, IIb, III
recommendation
C Requires evidence obtained from expert committee reports or opinions and/or clinical experiences of respected Evidence level IV
authorities. Indicates an absence of directly applicable clinical studies of good quality.

Adapted from the National Guidelines Clearinghouse (www.guideline.gov).

26 NOVEMBER 2019 x VOLUME 3, NUMBER 22 UPDATED INTERNATIONAL PRIMARY ITP CONSENSUS REPORT 3781

Provan D, et al. Blood. Advances.2019;3:3780-817

12
Joint Working Group
(DGHO, OGHO, SGH, GPOH and DGTI)
2018
_________________________________________
Grade of Recommendation
A : Strong recommendation “Must”
B : Recommendation “Should”
C : Recommendation open “Can”
EC : Expert Consensus

Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30


13
Talaksana newly-diagnosed ITP

14
Rawat inap atau rawat jalan?
_________________________________________
Management
American Society of Hematology

no or mild
• Tanpa perdarahan atau perdarahan minimal pd kulit dengan hitung trombosit

Outpatient
<20.000/mm3 atau >20.000/mm3) à Rawat inap tidak disarankan 1
nly
• Rawat inap: bila ada keraguan diagnosis, masalah sosial, tinggal jauh dari RS, follow-
up diragukan

International Consensus Report

no or mild
• Rawat inap hanya untuk perdarahan grade ¾ (Rekomendasi C)

Outpatient
• Pertimbangkan rawat inap bila rumah jauh dari RS, pertimbangan sosial,
ketidakpatuhan orangtua 1
nly Neunert C, et al. Blood advanced.2019;3:3829-66
Provan D, et al. Blood. Advances.2019;3:3780-817
15
ment of Newly Diagnosed ITP
Observasi atau obat?
_________________________________________
Management
American Society of Hematology
109/l and
• Tanpano or mild
perdarahan Outpatient
atau perdarahan minimal à observasi 1
tations) only
International Consensus Report
• Mayoritas anak cukup dengan “watch and wait” (Rekomendasi C)
x10 /l and no or mild
9
Outpatient 1
• Pertimbangkan pemberian obat bila perdarahan grade 3 (Rekomendasi C)
tations) only
• Diobati bila perdarahan grade 4 (Rekomendasi C)

Joint Working Group


• Tidak perlu terapi bila perdarahan tidak ada atau ringan (Rekomendasi B)
bout the• diagnosis,
Dapat diberikanthose with
obat bila social concerns,
ada perdarahan those who
mukosa (Rekomendasi B) live far
from the
ow-up cannot be guaranteed, admission to the
Neunerthospital may be preferable.
C, et al. Blood advanced.2019;3:3829-66
Provan D, et al. Blood. Advances.2019;3:3780-817
16
Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30
ly Diagnosed ITP
Jenis obat yang diberikan?
_________________________________________

American Society of Hematology Management


mild
• Pada perdarahan mukosa yang tidak mengancam jiwa dan atau penurunan QoL,

Outpatient 1
kortikosteroid lebih dianjurkan dibanding IVIG/Anti-D
• Prednison (2-4 mg/kg/hari, maksimum 120 mg/hari, selama 5-7 hari)
dibanding dexametason (o,6 mg/kg/hari, maksimum 40 mg, selama 4 hari)

Neunert C, et al. Blood advanced.2019;3:3829-66


17
Jenis obat yang diberikan?
_________________________________________

International Consensus Report


• Steroid:
• Prednison 4 mg/kg/hari, selama 4 hari, maksimum 200 mg/hari atau dosis 1-2
mg/kg, maksimum 80 mg/hari selama 1-2 minggu (Rekomendasi C)
• Metilprednisolon dosis tinggi 30 mg/kg/hari maksimal 1 g/hari
• Pada perdarahan sedang atau berat, IVIG (0,8-1 g/kg, dosis tunggal) atau
anti-D (75ug/kg, dosis tunggal) dapat meningkatkan hitung trombosit dengan
cepat (Rekomendasi A)

Provan D, et al. Blood. Advances.2019;3:3780-817


18
t of Newly Diagnosed ITP
Pilihan terapi emergensi?
_________________________________________
Management
International Consensus Report
and• Terapi
nokombinasi
or mild
mg/kg/hari), IVIG dengan atau tanpa anti-D Outpatient
: transfusi TC, kortikosteroid IV (metilprednisolon 30 1

ns) only
Joint Working Group
and• Padano or mild
perdarahan
Outpatient
mengancam jiwa, IVIG bisa diberikan bersama steroid 1
dan
ns) only
transfusi trombosit (bila diperlukan) (Rekomendasi A)

Provan D, et al. Blood. Advances.2019;3:3780-817


19
Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30
nt of Newly
Terapi lini ke-2?
Diagnosed ITP
_________________________________________

American Society of Hematology


Management
/l and no or mild
Receptor Agonis (TPO-RA) dibanding rituximab atau
Outpatient
• Thrombopoietin
splenektomi 1
ons) only
Joint Working Group
/l and no or mild
Outpatient
• TPO-RA harus diberikan sebagai terapi lini ke-2 (Rekomendasi A) 1
ons) only
Neunert C, et al. Blood advanced.2019;3:3829-66
20
Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30
Talaksana ITP Persisten/Kronik

21
ITP Persisten/kronik
_________________________________________

International Consensus Report

• “Watch and wait” (Rekomendasi C)


• Terapi steroid, IVIG, dan atau anti-D pada perdarahan akut (Rekomendasi C)
• Perdarahan yang sering atau berat, rujuk ke ahli hematologi (Rekomendasi C)
• TPO-RA terbukti mengurangi perdarahan tanpa ES pada banyak kasus
(Rekomendasi A)

Provan D, et al. Blood. Advances.2019;3:3780-817


22
ITP Persisten/kronik
_________________________________________

Joint Working Group

• Tidak ada terapi standar. Pasien harus dirujuk ke ahli hematologi anak
(Rekomendasi EC)
• TPO-RA efektif untuk ITP kronik

Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30

23
Childhood Immune Thrombocytopenia:
Results From PETIT

Bussel JB, et al. Eltrombopag for the treatment of children with persistent and chronic immune thrombocytopenia
(PETIT): a randomised, multicentre, placebo-controlled study. Lancet Haematol. 2015;2(8):e315-e325.

24
Methods – Study Design
Age Duration of ITP Platelet count Not responsive
Eligibility 1 to < 18 years ≥ 6 months < 30 x 109/L to prior therapy

Open-label, dose- Double-blind, 2:1 Open-label, 2:1 randomization,


finding phasea,c randomization phasec eltrombopag-only phasec,d
(N = 15) (N = 67) (N = 67)

Eltrombopag + Eltrombopag + SoC


SoC (n = 45)
(n = 45) 17 weeks
Eltrombopag 7 weeks
+ Follow-up
SoCb 1, 2, 3, 4, 12, and 24
(n = 15) Placebo weeks
24 weeks + Eltrombopag + SoC
SoC (n = 22)
(n = 22) 24 weeks
7 weeks
ITP, immune thrombocytopenia; SoC, standard of care.
a Patients in dose-finding phase were not included in the following phases.
b Includes previous ITP medication and rescue medication.
c Stratified by age.
d Dose titrated based on platelet response to a maximum of 75 mg/day.

Figure adapted with permission from Bussel JB, et al. Lancet Haematol. 2015;2(8):e315-e325.

25
End Points
Secondary End Points
Primary End Point • Safety and tolerability for 24 weeks
• Proportion of patients • Responsea for ≥ 60% of assessments between
week 2 to 6
achieving a response as
• Responsea at least once any time during 24
defined by a platelet count weeks
≥ 50 x 109/L at least once • Effect of eltrombopag on the reduction or
between week 1 to 6 (double- discontinuation of concomitant ITP therapies
blind phase) in the absence of • Need for rescue ITPb medication
rescue therapy • Incidence and severity of WHO bleeding
• QOL and pharmacokinetics

ITP, immune thrombocytopenia; QOL, Quality of life; WHO, World Health Organization.
aResponse: Proportion of patients achieving platelet counts ≥ 50 10⁹/L.
bRescue treatment was defined as any of the following: new ITP medication, increased dose of a
concomitant ITP medication from baseline, platelet transfusion, and splenectomy.

Bussel JB, et al. Lancet Haematol. 2015;2(8):e315-e325.


26
Baseline
Double-blind phase, all cohorts
Characteristics
Characteristic Placebo Eltrombopag
(n = 22) (n = 45)
Age (years), mean (95% CI) 10 (8-12) 9 (8-10)
Males, n (%) 9 (41) 18 (40)
Weight (kg), mean (95% CI) 43 (33-53) 39 (34-45)
Actual baseline ITP medications, n (%) 2 (9) 5 (11)
Prior ITP medication, n (%) 22 (100) 43 (96)
> 2 prior ITP treatments, n (%) 19 (86) 38 (84)
Duration of ITP, n (%)
6 to < 12 months 2 (9) 8 (18)
≥ 12 months 20 (91) 37 (82)
Platelets ≤ 15 × 109/L, n (%) 11 (50) 23 (51)
Baseline platelet count (× 109/L), mean (SD) 12.4 (8.8) 15.5 (8.0)
Splenectomy, n (%) 0 (0) 5 (11)
Ethnic origin, n (%)
White 20 (91) 40 (89)
Other 2 (9) 5 (11)

CI, confidence interval; ITP, immune thrombocytopenia; SD, standard deviation.


Table adapted with premission from Bussel JB, et al. Lancet Haematol. 2015;2(8):e315-e325.

27
Platelet Response – Primary End Point
Proportion of Responders
Placebo Eltrombopag
90
• A significantly higher proportion 80 OR, 4.3 (95% CI, 1.4-13.3)
80%
of patients in the eltrombopag P = 0.011 n=4

Proportion of responders, %
group respondeda 70
62% 63% 63%
60%
• Responsesa in the eltrombopag 60 n = 28 n = 10 n = 12
n=6
groups were similar in all 3 age 50
cohorts
40
32% 33%
30 n=7 n=3

20

10
0%
0
All cohorts Ages 12-17 Ages 6-11 Ages 1-5
aResponse defined as platelets ≥ 50 x 109/L at least once from week 1 to 6 in the CI, confidence interval; OR, odds ratio.
absence of rescue therapy. Figure adapted with premission from Bussel JB, et al. Lancet Haematol. 2015;2(8):e315-e325.
Bussel JB, et al. Lancet Haematol. 2015;2(8):e315-e325.

28
Platelet Count During the Double-blind Phase
140
Median platelet count, x 109/L 120 Eltrombopag
Placebo
100

80

60

40

20

0
Baseline 1 2 3 4 5 6
Study week
Number of patients
Placebo 20 22 22 22 22 22 22
Eltrombopag 43 43 43 41 42 41 42

Figure adapted with premission from Bussel JB, et al. Haematologica. 2014; 99:263;S733 (Oral presented at EHA 2014).

29
Ilustrasi Kasus

30
KASUS 1
_________________________________________
Anak lelaki, 6 tahun, datang dengan bercak kemerahan dan lebam sejak 1
hari yang lalu. Tidak ada perdarahan tempat lain.
Tidak ada riwayat makan obat-obatan. Tidak ada riwayat trombositopenia
sebelumnya. Tidak ada riwayat keluarga dengan trombositopenia.

Pemeriksaan Fisik:
q KU: baik, tidak tampak sakit; tanda vital dalam batas normal
q petekie dan beberapa ekimosis pada lengan dan tungkai
q tidak ada limfadenopati atau hepatosplenomegali

31
KASUS 1
_________________________________________

Laboratorium:
DPL: Hb; 12 g/dL, Leukosit: 7500/mm3, hitung trombosit 30.000/mm3
GDT: beberapa large platelets, tidak ada kelainan morfologi lain

Diagnosis:
Newly diagnosed ITP dengan perdarahan grade 1/2

32
KASUS 1
_________________________________________
Sebagai dokter anak, apa pilihan terapi terbaik untuk tatalaksana pasien?

A. Rawat inap, berikan transfusi trombosit


B. Rawat inap, berikan IVIG
C. Rawat inap, berikan prednison dosis 2-4 mg/kg/hari
D. Rawat inap, observasi
E. Rawat jalan, observasi

33
Rawat inap atau rawat jalan?
_________________________________________
Management
American Society of Hematology

no or mild
• Tanpa perdarahan atau perdarahan minimal pd kulit dengan hitung trombosit

Outpatient
<20.000/mm3 atau >20.000/mm3) à Rawat inap tidak disarankan 1
nly
• Rawat inap: bila ada keraguan diagnosis, masalah sosial, tinggal jauh dari RS, follow-
up diragukan

International Consensus Report

no or mild
• Rawat inap hanya untuk perdarahan grade ¾ (Rekomendasi C)

Outpatient
• Pertimbangkan rawat inap bila rumah jauh dari RS, pertimbangan sosial,
ketidakpatuhan orangtua 1
nly Neunert C, et al. Blood advanced.2019;3:3829-66
Provan D, et al. Blood. Advances.2019;3:3780-817
34
ment of Newly Diagnosed ITP
Observasi atau obat?
_________________________________________
Management
American Society of Hematology
109/l and
• Tanpano or mild
perdarahan Outpatient
atau perdarahan minimal à observasi 1
tations) only
International Consensus Report
• Mayoritas anak cukup dengan “watch and wait” (Rekomendasi C)
x10 /l and no or mild
9
Outpatient 1
• Pertimbangkan pemberian obat bila perdarahan grade 3 (Rekomendasi C)
tations) only
• Diobati bila perdarahan grade 4 (Rekomendasi C)

Joint Working Group


• Tidak perlu terapi bila perdarahan tidak ada atau ringan (Rekomendasi B)
bout the• diagnosis,
Dapat diberikanthose with
obat bila social concerns,
ada perdarahan those who
mukosa (Rekomendasi B) live far
from the
ow-up cannot be guaranteed, admission to the
Neunerthospital may be preferable.
C, et al. Blood advanced.2019;3:3829-66
Provan D, et al. Blood. Advances.2019;3:3780-817
35
Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30
KASUS
_________________________________________
Sebagai dokter anak, apa pilihan terapi terbaik untuk tatalaksana pasien?

A. Rawat inap, berikan transfusi trombosit


B. Rawat inap, berikan IVIg
C. Rawat inap, berikan prednison dosis 2-4 mg/kg/hari
D. Rawat inap, observasi
E. Rawat jalan, observasi

36
KASUS 2 (lanjutan)
_________________________________________
Ibu menghubungi saudara, dan mengatakan bahwa tampak bintik perdarahan di mulut dan juga
mengalami perdarahan hidung selama 10 menit yang berhenti setelah penekanan
Hasil pemeriksaan darah ulangan: hitung trombosit 6000/mm3

Diagnosis : Newly diagnosed ITP dengan perdarahan grade 3

37
KASUS 2
___________________________________________________________________________________________________________________________________________________________________

Apa tatalaksana selanjutnya?

A. Prednison dosis 2-4mg/kg/day (maximum 120 mg daily) selama 5-7 hari


B. Deksametason o,6 mg/kg/hari, maksimum 40 mg, selama 4 hari
C. Transfusi trombosit
D. Berikan IVIg atau anti-D
E. Transfusi TC dan IVIg dan metiprednisolon IV

38
ly Diagnosed ITP
Jenis obat yang diberikan?
_________________________________________

American Society of Hematology Management


mild
• Pada perdarahan mukosa yang tidak mengancam jiwa dan atau penurunan QoL,

Outpatient 1
kortikosteroid lebih dianjurkan dibanding IVIG/Anti-D
• Prednison (2-4 mg/kg/hari, maksimum 120 mg/hari, selama 5-7 hari)
dibanding dexametason (o,6 mg/kg/hari, maksimum 40 mg, selama 4 hari)

Neunert C, et al. Blood advanced.2019;3:3829-66


39
Jenis obat yang diberikan?
_______________________________________________________________________________________________________________________________________________________

International Consensus Report


• Steroid:
• Prednison 4 mg/kg/hari, selama 4 hari, maksimum 200 mg/hari atau dosis 1-2
mg/kg, maksimum 80 mg/hari selama 1-2 minggu (Rekomendasi C)
• Metilprednisolon dosis tinggi 30 mg/kg/hari maksimal 1 g/hari
• Pada perdarahan sedang atau berat, IVIG (0,8-1 g/kg, dosis tunggal) atau
anti-D (75ug/kg, dosis tunggal) dapat meningkatkan hitung trombosit dengan
cepat (Rekomendasi A)

Provan D, et al. Blood. Advances.2019;3:3780-817


40
KASUS 2
_________________________________________
Apa tatalaksana selanjutnya?

A. Prednison dosis 2-4mg/kg/day (maximum 120 mg daily) selama 5-7 hari


B. Deksametason o,6 mg/kg/hari, maksimum 40 mg, selama 4 hari
C. Transfusi trombosit
D. Berikan IVIg atau anti-D
E. Transfusi TC dan IVIg dan metiprednisolon IV

41
KASUS 3 (lanjutan)
_________________________________________
Enam bulan kemudian anak masih sering mengalami perdarahan dan tidak respon dengan
pemberian steroid
Saat ini anak sering mengalami perdarahan dari hidung dan menyebabkan sering
dipulangkan dari sekolah
Orangtua mengeluhkan bahwa kualitas hidup anaknya cukup menurun

Diagnosis : ITP Persisten dengan perdarahan grade 3

42
KASUS 3 (lanjutan)
_________________________________________
Apa tatalaksana yang akan saudara berikan pada pasien?

A. Splenektomi
B. Transfusi trombosit
C. Berikan IVIg atau anti-D
D. TPO-RA
E. Transfusi TC dan IVIg dan metiprednisolon IV

43
ITP Persisten/kronik
_________________________________________

International Consensus Report

• “Watch and wait” (Rekomendasi C)


• Terapi steroid, IVIG, dan atau anti-D pada perdarahan akut (Rekomendasi C)
• Perdarahan yang sering atau berat, rujuk ke ahli hematologi (Rekomendasi C)
• TPO-RA terbukti mengurangi perdarahan tanpa ES pada banyak kasus
(Rekomendasi A)

Provan D, et al. Blood. Advances.2019;3:3780-817


44
ITP Persisten/kronik
_________________________________________

Joint Working Group

• Tidak ada terapi standar. Pasien harus dirujuk ke ahli hematologi anak
(Rekomendasi EC)
• TPO-RA efektif untuk ITP kronik

Matzdorff H, et al. Oncol Res Treat 2018;41(suppl 5):1–30

45
KASUS 3 (lanjutan)
_________________________________________
Apa tatalaksana yang akan saudara berikan pada pasien?

A. Splenektomi
B. Transfusi trombosit
C. Berikan IVIg atau anti-D
D. TPO-RA
E. Transfusi TC dan IVIg dan metiprednisolon IV

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Kesimpulan
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q Tatalaksana ITP pada umumnya adalah “watch and wait”

q Terapi diberikan pada perdarahan derajad sedang dan berat, dengan pilihan
pertama adalah kortikosteroid

q Pada kasus ITP yang tidak respon dengan steroid atau pada ITP persisten/kronik ,
TPO-RA efektif meningkatkan hitung trombosit

q Pada kasus dengan perdarahan berat/mengancam jiwa diberikan terapi kombinasi,


yakni pemberian steroid, IVIG, dan transfusi trombosit, dengan atau tanpa anti-D

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Terimakasih

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