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Case Report 1
Case Report 1
Case Report 1
ANEMIA APLASTIK
Compiled By:
Sakthy Vicknes A/P Shanmugam
110100400
Supervisor :
dr. Rina Amalina Carolina, Sp.A (K)
CONTENTS
CHAPTER I INTRODUCTION ..........................................................................1
1.1................................................................................................................B
ackground ..............................................................................................1
1.2................................................................................................................
Objective ...............................................................................................1
CHAPTER II LITERATURE REVIEW ............................................................2
2.1. Definition .............................................................................................2
2.2. Etiology.................................................................................................2
2.3. Classification.........................................................................................3
2.4.Pathophysiology....................................................................................4
2.5.Differential Diagnosis...........................................................................5
2.6.Diagnosis...............................................................................................6
2.7. Management.........................................................................................8
CHAPTER III CASE REPORT ........................................................................11
CHAPTER IV DISCUSSION & SUMMARY..................................................19
4.1. Discussion...........................................................................................19
4.2. Summary.............................................................................................20
REFERENCES....................................................................................................21
CHAPTER I
INTRODUCTION
1.1 Background
Aplastic anemia is defined as failure of bone marrow to producing
components of blood cells. Aplastic anemia is anemia accompanied by peripheral
blood pancytopenia caused by a primary disorder of the bone marrow in the form
of aplasia or hypoplasia of the absence of infiltration, bone marrow suppression or
displacement. Pancytopenia itself is a condition characterized by the presence of
anemia, leukopenia, and thrombocytopenia in all its manifestations.1
Aplastic anemia is a rare disease in the world. The incidence in Asia including
China, Japan, Thailand and India is higher compared with Europe and the United
States of America. The incidence of this disease varies between 2 to 6 cases per 1
million population. Research conducted The International Aplastic anemia and
agranulocytosis Study in Europe and Israel early in 1980 to get 2 cases per 1
million populasi.3
Comparison of incidence between men and women is approximately 1: 1,
although of some data showing men slightly more often affected by anemia
aplastik.3
Although aplastic anemia occurs in all age groups, a small peak in the
incidence is observed in childhood because of the inclusion of inherited marrowfailure syndromes. A second peak is observed in people aged 20-25 years
(Medscape)
1.2 Objective
The aim of this study is to explore more about the theoritical aspects on
aplastic anemia, and to integrate the theory and application of aplastic anemia
case in daily life.
CHAPTER II
LITERATURE REVIEW
2.1 Definition
Aplastic anemia is a bone marrow failure syndrome that is characterized
by peripheral pancytopenia and bone marrow hypoplasia. In aplastic anemia
decreased production of blood cells from the bone marrow, causing
retikulositopenia, anemia, granulocytopenia, monositopenia and trombositopenia.3
2.2 Etiology
Aplastic anemia is often caused by exposure to radiation and chemicals.
However, most patients the cause is idiopathic, meaning the cause is unknown.
Aplastic anemia can also be associated with viral infections and other diseases
(Table 2).
Table 2. Classification of aplastic anemia Etiology
Acquired Aplastic Anemia
Secondary aplastic anemia
Chemicals
Benzene
Insecticides
Glue
Solvents
Drugs
Cytotoxic agents
Antibiotics
Nonsteroidal anti-inflammatory drugs
Anticonvulsive agents
Gold salts
Radiation
Viruses
Epstein-Barr virus
Non-A, non-B, non-C hepatitis viral agent (?)
Human immunodeficiency virus
Immune and rheumatologic diseases
Graft-versus-host disease
Rheumatoid arthritis
Systemic lupus erythematosus
Paroxysmal nocturnal hemoglobinuria
Pregnancy
Idiopathic aplastic anemia
Inherited Aplastic Anemia
Fanconis anemia
Dyskeratosis congenital
Schwachman syndrome
2.2.1. Radiation
Marrow aplasia is a major acute sequela of radiation. Radiation damages
DNA; tissues dependent on active mitosis are particularly susceptible. Nuclear
accidents can involve not only power plant workers but also employees of
hospitals, laboratories, and industry (food sterilization, metal radiography, etc.), as
well as innocents exposed to stolen, misplaced, or misused sources. While the
radiation dose can be approximated from the rate and degree of decline in blood
counts, dosimetry by reconstruction of the exposure can help to estimate the
patient's prognosis and also to protect medical personnel from contact with
radioactive tissue and excreta. MDS and leukemia, but probably not aplastic
anemia, are late effects of radiation. (HARRISON)
2.2.2. Chemicals
Benzene is a notorious cause of bone marrow failure. Vast quantities of
epidemiologic, clinical, and laboratory data link benzene to aplastic anemia, acute
leukemia, and blood and marrow abnormalities. The occurrence of leukemia is
roughly correlated with cumulative exposure, but susceptibility must also be
important, as only a minority of even heavily exposed workers develop benzene
myelotoxicity. The employment history is important, especially in industries
where benzene is used for a secondary purpose, usually as a solvent. Benzenerelated blood diseases have declined with regulation of industrial exposure.
Although benzene is no longer generally available as a household solvent,
exposure to its metabolites occurs in the normal diet and in the environment. The
association between marrow failure and other chemicals is much less well
substantiated.
2.2.3 Drugs
In general, all drugs can cause aplastic anemia in someone with a genetic
predisposition. Drugs that commonly cause aplastic anemia is chloramphenicol.
This is caused by idiosyncratic reaction. Idiosyncratic drug reactions that arise are
not related to the pharmacological properties of the drug, with the proportion
varying dala population with no known cause. For example, doses of
chloramphenicol which more than 50 mg / kg / day with long-term use causes
idiosyncratic
reactions.
Other
drugs
are
also
frequently reported
are
after
platelets <20x109 / l
reticulocyte <20x109 / l
same as severe aplastic anemia except
neutrophils <0,2x109 / l
2.4 Pathopysiology
Penyebabanemiaaplastiksulitditentukan,
terutamakarenabanyakkemungkinan
yang
harusdisingkirkan.
Jikatidakditemukanpenyebab
yang
pastimakadigolongkankedalampenyebabidiopatik.Pendapatlainmenyatakanbahwa
penyebabterbanyakdarikegagalansumsumtulangadalahiatrogenikkarenakemoterapi
sitostatikatauterapiradiasi.
Kerusakan
yang
terjadipadaanemiaaplastikterdapatpadaselindukdanketidakmampuanjaringansums
umtulanguntukmemberikesempatanselindukuntuktumbuhdanberkembangdenganb
aik.
Hal
iniberkaitaneratdenganmekanisme
yang
yang
terjadipadaanemiaaplastikberhubungandenganinfeksi
virus
normal
merupakaninteraksiantara
denganlingkunganmikro
yang
progenitor
terjadi
di
dalamsumsumtulang,
hematopoetik
(microenvironment)
stem
cell
padasumsumtulang.
Lingkunganmikrotersebutmengaturhematopoesismelaluireaksistimulasiolehfaktor
pertumbuhanhematopoetik.
Sel-
selhematologikimaturdapatterlihatdenganpemeriksaanflouresent
activate
flow
normal.
Anemiaaplastikdapatterjadisecaraheterogenmelaluibeberapamekanismeyaitukerus
akanpadalingkunganmikro,
gangguanproduksiataufungsidanfaktor-
faktorpertumbuhanhematopoetik,
dankerusakansumsumtulangmelaluimekanismeimunologis.
Limfosit
sitotoksikaktif,
memegangperan
yang
besardalamkerusakanjaringansumsumtulangmelaluipelepasanlimfokinseperti
interferon-(IFN-) dantumor necrosis factor (TNF-). Peningkatanproduksi
interleukin-2
mengawaliterjadinyaekspansipoliklonalsel
T.
interferon
adalahmenghambattranskripsi
regulatory
gen
factor
danmasukkedalamsiklus
(IRF-1),
sel.
IFN-
Diagnosa Banding3
a. MyelodisplasiaHiposeluler
Untukmembedakan
anemia
aplastikdarisindrommielodisplastikhipoplastikdapatdilakukanaspirasisumsumtulan
guntukmendapatkanproporsisel-sel
CD34+.
Padasindrommielodisplastik,
CD34+merupakan
proporsisel
CD34+pada
target
anemia
sedangkanpadasindrommielodisplastik,
ataulebihtinggi.
Selainitu,
seranganautoimun.
aplastik
proporsinya
Dengandemikian,
0,3%
ataukurang
normal
(0,5%
untukmembedakan
aplastikdengansindrommyelodisplastikdapatmelaluimorfologidarah
,
1%)
anemia
yang
yang
anemia
prekursorgranulositdapatberkurangatauterlihatgranulasi
danmegakariositdapatmenunjukkanlobulasinukleus
(misalnyamikromegakariositunilobuler).
aplastik.
abnormal
abnormal
diagnosis
untuksumsumtulang
yang
cytometry,
yaitupolapulasansel-
danketidakteraturanreseptorsel
yang
membuktikanadanyaekspansimonoklonalpopulasisel T.
c. Anemia aplastikdanHemoglobinuriaNokturnalParoksisimal (PNH)
Pada
PNH,
selasalhematopoetik
menurunkanpopulasiseldarahmerah,
granulosit,
abnormal
dantrombosit
yang
gen
PIG-A
di
kromosom
yang
yang
mengakibatkanketidakmampuaneritrosituntukmenginaktivasikomplemenpermuka
an.
2.6.
Diagnosis
2.6.1. Anamnesa
Anemia
atauperlahan-lahan
aplastikmungkinmunculmendadak
(dalambeberapahari)
(berminggu-mingguatauberbulan-bulan).
Keluhanpasien
nafsumakanberkurang,
pucat,
sesaknafas,
penglihatankabur,
bahkantelingaberdengung.
Trombositopeniamenyebabkanmudahmemardanpendarahanmukosa. Neutropenia
meningkatkankerentananterhadap infeksi.3
2.6.2. PemeriksaanFisik
Hasilpemeriksaanfisikpadapasien
anemia
aplastikbervariasi.
c. Reactions during ATG transfusion are common and rarely anaphylaxis can
occur
d. Platelet support is essential, as ATG can cause a fall in platelet counts
e. Serum sickness occurs in about 40% patients in the second and third week
Cyclosporine is usually started after steroids are tapered off, usually after 3
weeks of ATG. The response with ATG/cyclosporine is seen after 3-4 months.
Cyclosporine should be tapered off very gradually after a minimum of 6 months.
If there is no response to one course of ATG, orthere is a relapse, a second or third
course may be given. Patients who show no response to twocourse of ATG are
unlikely to respond to a third course. If cyclosporine is used alone, the response
rates are about 30%.10
CHAPTER III
CASE REPORT
Name
: RZ
Age
: 1 years 4 months
Sex
: Boy
RM
: 00.62.43.99
Date of Admission
: 20 April 2015
Chief Complain
: Pale
History
The patient was reference from RSUP Blangkegeren with diagnosis of congestive
heart failure ec severe anemia
Birth history: RZ was the third boy. Birth spontaneously, helped by tocologist,
cried hard. Cyanosis (-), birth weight: 3,2 kg.
Pregnancy history: mother age 34 years old, medical history (-), drug exposure (-),
herb exposure (-).
Feeding History:
From birth to 6 months: Breast feeding only,
From 6 months to 9 months : Breast milk with rice porridge
From 9 months to 1,5 years
: Family food
: 5 months
Crawling
: 8 months
Standing
: 10 months
Walking
: 18 months
Presence status:
Consciousness: Compos Mentis
Heart Rate: 116 x/i regular
Respiratory Rate: 30x/i regular
Body Temperature: 37,4C
Anemic (+), icteric (-), cyanosis (-), edema (-), dyspnea (-),
Localized Status
Head: light reflexes (+/+), pupil isochoric, inferior conjuctiva pale (+/+),
fontanella open flat, icteric sclera (-/-), face edema (-), inferior and
superior palpebrae edema (-), Ear/ Nose/ Mouth: within normal limit/ O2
not palpable
Extremities: pulse 116 x/i , adequate pressure and volume, warm, CRT < 3
Laboratory finding
Complete blood analysis
Test
Hemoglobin
Erythrocyte
Leucocyte
Thrombocyte
Hematocrite
Eosinophil
Result
1,80
0,55
11,48
277
5,70
0,20
Unit
G%
106/mm3
103/mm3
103/mm3
%
%
Referral
11,3-14,1
4,40-4,48
6,0-17,5
217-497
37-41
1-6
Basophil
Neutrophil
Lymphocyte
Monocyte
Neutrophil absolute
Lymphocyte
0,600
43,90
47,40
7,90
5,04
5,44
%
%
%
%
103/L
103/L
0-1
37-80
20-40
2-8
1,9-5,4
3,7-10,7
absolute
Monocyte absolute
Eosinophyl absolute
Basophyl absolute
MCV
MCH
MCHC
0,91
0,02
0,07
103,60
32,70
31,60
103/L
103/L
103/L
fL
pg
g%
0,3-0,8
0,20-0,50
0-0,1
81-95
25-29
29-31
Clinical Chemistry
Test
Carbohydrate Metabolism
Blood Glucose
Electrolite
Natrium
Kalium
Cloride
Differential Diagnosis
Result
Unit
Referral
176
mg/dL
< 200
134
2,2
104
mEq/L
mEq/L
mEq/L
135-155
3,6-5,5
96-106
Working Diagnosis
: Aplastic anaemia
Management
O2 1-2 L/i
IVFD D5% NaCl 0,225% 20 gtt/i micro
Paracetamol 100 mg (as needed)
Oral prednisone 1-1-1
Folic acid1x1 mg
Follow Up
April, 21th 2015
S Swollen eyes (+), fever (-),epigastruim pain (+)
O Cons: alert, Temp: 37,4oC.
Body weight: 7 kg, Body length: 78 cm.
Head
Eye: light reflex (+/+), isochoric pupil, pale inferior conjunctiva palpebra
Neck
Thorax
:
:
Abdomen :
mucosa is pale .
Lymph node enlargement (-).
Symmetrical fusiformis. Chest retraction (-). HR: 120x/i, regular,
murmur (-). RR: 30x/i, reguler, ronchi (-).
Soepel (+),peristaltic (+) normal, liver : palpable for 2 cm, Spleen :not
palpable
Extremities: Pulse 120x/i, regular, adequate pressure and volume, warm, CRT < 3.
Genital
Male. Scrotum (+). Edema (-).
A
P
:
Aplastic Anemia
Management:
-
O2 1-2 L/i
Folic acid1x1 mg
Eye: light reflex (+/+), isochoric pupil, pale inferior conjunctiva palpebra
(-/-).Ear/ Nose/ Mouth: within normal limit/ within normal limit /within
Neck
Thorax
normal limit.
Lymph node enlargement (-).
Symmetrical fusiformis. Chest retraction (-). HR: 116x/i, regular, murmur
A
P
Abdomen
Extremitie
s
Genital
Male. Scrotum (+). Edema (-).
- Leukemia
- Anemia hemolytic
Management:
-
prednisone 1-1-1
Folic acid1x1 mg
Paracetamol drip 100 mg / 6 hour / iv
Blood transfusion PRC III 75 cc ( enter 100 cc , needed 260 cc )
BMP ( 24/4/15 )
Diet normal eating 700 kkal with 14 gram protein .
Consult to nutrition and metabolic .
Neck
Thorax
Abdomen
Extremitie
Genital
A- Leukemia
prednisone 1-1-1
Folic acid1x1 mg
Paracetamol drip 100 mg / 6 hour / iv
Diet normal eating 700 kkal with 14 gram protein .
BMP + Check DL post transfusi
Blood transfusion PRC IV 75 cc ( enter 145 cc , needed 260 cc )
Blood transfusion PRC V 60 cc ( enter 200 cc , needed 260 cc )
Neck
Thorax
Abdomen
Extremitie
s
Genital
Male. Scrotum (+). Edema (-).
A- Aplastic Anemia
P Management:
- prednisone 1-1-1
- Folic acid1x1 mg
- Paracetamol drip 100 mg / 6 hour / iv
Neck
Thorax
Abdomen
Extremitie
s
Genital
Male. Scrotum (+). Edema (-).
A- Aplastic Anemia
P Management:
-
prednisone 1-1-1
Folic acid1x1 mg
Paracetamol 3 100 mg
Diet normal eating 700 kkal with 14 gram protein
April, 26th 2015
S
O
Neck
Thorax
Abdomen
Extremitie
s
Genital
Male. Scrotum (+). Edema (-).
A- Aplastic Anemia
Management:
- prednisone 1-1-1
- Folic acid1x1 mg
- Paracetamol 3 100 mg
April, 27th 2015
S
O
Neck
Thorax
Abdomen
Extremitie
s
Genital
Male. Scrotum (+). Edema (-).
A- Aplastic Anemia
P Management:
-
prednisone 1-1-1
Folic acid1x1 mg
Paracetamol 3 100 mg
Diet normal eating 700 kkal with 14 gram protein
CHAPTER IV
DISCUSSION AND SUMMARY
4.1. Discussion
The
clinical
findings
of
aplastic
anemia
relate
to
anemia,
complete
blood
counts,
reticulocyte
count,
peripheral
smear
of
infection,
hematopoiesis
stem
cell
transplant,
and
4.2. Summary
It has been reported, an adolescent boy with the main complain of pale and was
diagnosed with anemia aplastic. The diagnose was established based on history
taking, clinical manifestation, laboratory finding, and bone marrow puncture.The
patient got PRC transfusion and still need to be followed up.