Você está na página 1de 35

INTRODUCTION

Leukemia is one of the malignancy from bone marrow, with white


blood cell proliferation, with manifestation by abnormal peripheral blood
cell. Acute leukemia is the most common form of cancer in children,
comprising approximately 30 percent of all childhood malignancies
Childhood ALL was the first disseminated cancer shown to be curable and
consequently has represented the model malignancy for the principles of
cancer diagnosis, prognosis, and treatment. It actually is a heterogeneous
group of malignancies with a number of distinctive genetic abnormalities
that result in varying clinical behaviors and responses to therapy. 1,2

T-cell acute lymphoblastic leukemia (T-ALL) in childhood is an


aggressive malignancy characterized by high white blood cell count
(WBC), mediastinal tumor and a high rate of relapses in the central
nervous system, bone marrow (BM) or testicle infiltration. T-ALL accounts
for approximately 15% of all childhood acute lymphoblastic leukemia
(ALL), and in contrast to B-cell ALL, the prognostic significance of
molecular-cytogenetic abnormalities in pediatric T-ALL has not been yet
elucidated . Therefore, exploring the immunophenotype and molecular
connections to define some T-ALL settings is one of the potential issues
for clinical translation. T-ALL can be classified according to specific
subgroups that are associated with the expression of specific
immunophenotypic markers, reflecting maturational arrest at distinct T-cell
developmental stages . 3

Acute leukemia can be classified based on morphologic


characteristics and, cytochemical features , immunologic characteristics ,
and cytogenetic and molecular characteristics 4. A panel of antibodies is
used to establish the diagnosis of leukemia and to distinguish among the
immunologic subclones. The panel should include at least one marker that

1
is highly lineage specific, for example, CD19 for B lineage, CD7 for T
lineage, and CD13 or CD33 for myeloid cells. In addition, the use of
cytoplasmic CD79 for early pre-B-cell lineage, cytoplasmic CD3 for T
lineage, and cytoplasmic myeloperoxidase for myeloid cells can be helpful
in differentiating unclear immunophenotypes4.

Febrile neutropenia (FN) is one of the most common adverse


events associated with myelosuppressive chemotherapy for cancer
treatment. Fever in the setting of neutropenia should be considered
amedical emergency requiring immediate evaluation and administration of
empiric broad-spectrum antibiotics. Fever in neutropenic patients is
defined as a single oral temperature of >38.3°C (101°F) or a temperature
of >38.0°C (100.4°F) sustained for >1 hour. Neutropenia is defined as an
absolute neutrophil count (ANC) <500/mm3.30

The following report is a case of Acute Limphoblastic Leukemia in


a child that at Hematology and Oncology Division Department of Child
Health.Prof. Dr. dr. R.D. Kandou General Hospital Manado.

2
CASE REPORT

CS, 6 years 7 month old boy, Christian, Sangerenes, was admitted to


Outpatient clinic Prof. Dr. R. D. Kandou General Hospital in Manado on 25
Oktober 2016, with chief complained planing for chemotheraphy .

PATIENT IDENTITY

Medical record : 23.96.81

Name : C.S

Date of birth : 8 April 2010

Place of birth : Manado

Gender : Male

Nationality : Indonesia

Tribe : Sangerenes

Religion : Christian

Address : Tikala Baru Lk. 6

PARENTS IDENTITY

FATHER MOTHER

Name: AS AA

Age: 28 Years Old 31 Years Old

Job: Farmer House wife

3
Education: Senior High School Senior High School

Religion: Christian Christian

Tribe: Sangernese Sangernese

History of ilness

(Alloanamnesis, provide by her mother and medical record)

The patient was reffered from Peditrician with chief complained of


having fever from 2 weeks, fever was responded with paracetamol, there
was no gums bleeding, nose bleeding, and any spontaneus bleeding.
Patient looked pale from 2 weeks before hospitalized, and his abdomen
distended. The patient felt more tired than usual, and less activity since 2
weeks before addmited to the hospital.Physical finding was hepatomegaly
(4-4 cm below arcus costae), Spleen was Schufner III. Laboratorium
finding, white blood cell count was 292,000/mm3, Trombocyte
79,000/mm3 after that a few supporting examination has been done. Blood
smear on 11 July 2016 were found blast 84 % with suspect acute
leukemia limphoblastic acute. Then bone marrow pungtion was made on
12 July 2016 and the result was ALL T lineage with marker was CD3,
CD5, and CD7 . Echocardiography was done on 14 july 2016 was normal.
Chest radiograph was normal. Cerebrospinal liquour analysis were not
found blast cell or any of bacteria. Chemotheraphy was strated on 17 July
2016 use "Indonesian Protocol Acute Lymphoblastic Leukemia High Risk
2013". At 2 September 2016 , the 2 nd BMP was done, with result BMP;
showing all speciment can't be evaluated. With eritropoesis cell
domination with various stage and not found limphoid cell proliferation.
And from laboratory finding , the white blood count was decreased to
5745/ mm3, hemoglobin was 8,6 g/dL, trombosit was 181,000/mm3, and
ANC 3734.

4
History of prenatal care and birth

During pregnancy, her mother had regular antenatal care and had
tetanus toxoid immunization twice. There was no exposure with radiation
This patient was born spontaneously by a doctor, immediately cried after
birth, full term baby, birth weight was approximately 3800 grams. He is the
only child at his family.

Developmental milestones

Growth and development of patients according to age.

Social smile : 2 months

Turning in prone position : 2 months

Sitting : 4 months

Crawling : 6 months

Standing : 8 months

Calling mama/papa : 8 months

Walking :10 month

History of Feeding

Breast feeding :-

Formula milk : birth – 2 years

Milk porridge : 6 - 8 months

Soft rice porridge : 10 – 12 months

Rice : 12 months until now

5
Immunizations

BCG : 1 time

Polio : 3 times

DPT : 3 times

Measles : 1 times

Hepatitis B : 3 times

Family History

No history of the other family member suffers the same illness.

Pedigree

6
Social, Economic and Evironmental Conditions

He is the only children in the family. Her father is 28 years old,


graduate from senior high school and work as an employe while her
mother is 31 years old, graduate from senior high school, an employee.

They live in a permanent house, consist of two bedroom, occupied


by 2 adults and 1 children. They had electrical source from the
governmental electric company and water supply from governmental water
company. Bathroom is located inside the house. Garbage management by
dumped and burned. The patient used government social health insurance
2rd class.

Physical examination 30/10/2016

General conditions : Looked ill, compos mentis.

Body weight : 24,5 kg

Ideal body weight : 21,5kg

Body height : 124cm

BMI : 15,9kg/m2
Nutritional status : Body weight / age = 24/21,5 x 100 % = 111 %
Body height / age = 124/118,5 x 100 % = 104 %
Bodyweight/Bodyheight=24,5 / 24x100 %= 102%
CDC 2000 Stature for age and weight for age
2-20 year boys chartz
Normal weight

Vital sign : Blood pressure : 100/70 mmHg

Pulse rate : 104 times/minute

Respiratory rate : 28 times/minute

7
Body temperature: 36 ⁰C

Head : Normocephaly

Eyes : Conjuntiva was not anemic and sclera was no icteric,

pupil was round, isochors, 3-3 mm, light reflex was


normal, palpebra was oedema on both eyes, fascial
edema (-)

Ears : Clear meatus acusticus externus, normal ear drums,

no secrete

Nose : There was no secretes, flare (-)

Mouth : There was no cyanosis, tonsils T1/T1 without

inflammatory sign, pharynx without inflammatory


sign.

Neck : There was no lymph node enlargement

Chest : Symmetrical respiratory movements,

there was no retractions

Heart :

- Inspection : no visualization of ictus cordis

- Palpation : ictus cordis was not palpable

- Percussion : left margin : linea midclavicularis sinistra

right margin : linea parasternalis dextra

upper margin : 2nd – 3th intercostals


spaces (ICS)

8
- Auscultation : No murmurs

Lungs :

- Inspection : symmetrical respiration movement on the both

- Palpation : vocal fremitus right = left

- Percussion : sonor percussion right = left

- Auscultation : bronchovesicular breath sound, rales -/-,

wheezing -/-

Abdomen : Flat, soft, with normal bowel sound, liver and spleen

were not palpable, tympanic percussion`

Extremities : Warm, no cyanotic, capillary refill time less than 2”,

normal muscle tone, edema(-), physiological


reflexes normal, no pathological reflexes.

Genitalia : Male , no abnormality

Diagnosis

Acute Lymphoblastic Leukemia T - cell ( C91.5) HR Consolidatian phase


W11 D7 ( postpone chemotheraphy day 14 )

Treatment

- 6MP 1X1 tab

- 21.00 - 09.00 WITA Hiperhidration ( NaCl 0,45% in D5% + 20 Meq


Nabic 1150ml every 12 hours = 95- 96 ml/ hours

9
BD every 4 hour ; if Balance > 100 ml gave Furocemid injection 15
mg ( dosage 0,5 mg /BW/ time )

Planning

- Continue Chemotheraphy

Nutritional care

1. Nutritional assessment : Good nutrition

2. Nutritional requirement :

Based on Recommended Daily Allowance (RDA)

Calorie needed : 90 kcal/kgBW/day = 2200 kcal/day

Protein needed : 1,2 gr/kgBW/day = 30 gr/day = kcal/day

Fluid needed : 70 – 85 mL/kgBW/day = 2200 – 2700 mL/day

Carbohydrate : 50% = 1100 kcal

Fat : 30% = 660 kcal

3. Adminitration route : oral

4. Food menu :

- 3 portion of full meal (@ 500kcal, 7 gr protein)


- Snack 2 x (@ 200 kcal), cokies, bread
- Fruit / juice 2 x @ 150 kcal
- Clear water 2200-2700 ml

5. Monitoring and evaluation : tolerance, adverse reaction, body weight .

Support care

- The general hygiene care patients

10
- Monitoring of hygiene for parents and medical personel

- Provide mental support to parents

Laboratory Result 27/10/2016

Hemoglobin : 8,5 gr/dL ALT : 90 u/l

Hematocrit : 25,2% AST : - u/l

Thrombocyte : 176,000 /mm3 Ureum : 13 mg/dl

Leukocyte : 1400/mm3 Creatinin : 0,3 mg/dl

DC : 0/14/14/42/0 CRP :48 mg/L

ANC : 392 Malaria :-

Na : 131 mEq/L

Ca : 91,9 mEq/L

K : 3,89 mEq/LCl

Ca : 8,66 mg/dL

Urine examination

PH :6 Bilirubin : (-)

Erithrocyte : 8-10 RBCs/hpf Glucose : (-)

Leukocyte : 3-4 WBCs/hpf Keton : (-)

Nitrit :-

Protein : (-)

11
FOLLOW UP

31 September 2016 – 2 November 2016 (2nd – 4th day )

Complaint : Fever (-), good intake, no vomiting

General conditions : Looked ill, compos mentis

Vital sign : Blood pressure : (90/60-100/60) mmHg

Pulse : 108–112times/minutes,regularly

Respiratory rate : 20-24 times/minutes

Temperature : 36.4 0C

Physical examination
Head : Normocephal
Eyes : Conjuntiva was not anemic and sclera was no icteric,
pupil was round, isochors, 3-3 mm, light reflex was
normal
Ears : Clear meatus acusticus externus, normal ear drums,
no secretes
Nose : There was no secretes, flare (-)
Mouth : There was no cyanosis, tonsils T1/T1 without
inflammatory sign, pharynx without inflammatory
sign.
Neck : There was no lymph node enlargement
Chest : Symmetrical respiratory movements, there was no
retractions
Heart : Heart margin was normal
Lungs : Bronchovesicular breath sound, trales -/-,
wheezing -/-
Abdomen : Flat, soft, with normal bowel sound,
shifting dullness (-),ascites (-),tympanic percussion,
liver and spleen were not palpable.

12
Extremities : Warm, no cyanotic, capillary refill time less than 2”,
normal muscle tone, there was no oedema on both
leg, physiological reflexes normal, no pathological
reflexes.
Genitalia : male, no abnormality

Diagnosis

Acute Lymphoblastic Leukemia T - cell ( C91.5) HR Consolidatian phase


W12D1-3

Treatment 31 September 2016

- 09.00-10.00 MTX IT 12 mg, premedication with Midazolam 2.5 mg


and Ketamin 13 mg
- 10.00-10.00 (1/11/2016) MTX HD 920 mg in Nacl 0,9% 500 ml

Treatment 1 November 2016

- 6MP 1 X 1 TAB
- 10.00(31/11)-10.00 (1/11/2016) MTX HD 920 mg in Nacl 0,9% 500
ml
11.00- 23.00 wita Hiperhidrasi NaCl 0,45% in D5% + Nabic 20 Meq
= 1150/ 12 hour. = 95-96 ml/ hours . BD/ 4 hours if more than 100
ml, give furosemid injecttion 15 mg ( dosage 0,5 mg /BW/ time )
- 23.00 – 23.30 Leucoverine I 13,65 mg in Nacl0,9 % 100 ml
- 23.30-03.30(2/11/2016) Ivfd NaCl0.9% in D5%24 ml / hours
- Ondansentron injection 4 mg (if needed)

Treatment 2 November 2016

- 6MP 1 X 1 TAB
- 03.30-04.00 Leucoverine II 13,65 mg in Nacl0,9 % 100 ml
- 04.00-08.00 Ivfd NaCl0.9% in D5%24 ml / hours
- 08.00-08.30 Leucoverine III 13,65 mg in Nacl0,9 % 100 ml
- Ondansentron injection 4 mg (if needed)

13
Pediatric nutritional care same as before
Nursing care same as before

Laboratorium Finding 2 November 2016

SGOT : 122

SGPT : 143

Planing

- Pro chemotheraphy 7th November, 2016

6 November 2016 – 7 November 2016 (5th – 6th day )

Complaint : fever (-), vomiting (-)

General conditions : Looked ill, compos mentis

Vital sign : Blood pressure : (100/70 - 120/80) mmHg

Pulse : 110-116 times/minutes, regularly

Respiratory rate : 24 times/minutes

Temperature : (36.3-37.1) 0C

Physical examination
Head : Normocephal
Eyes : Conjuntiva was not anemic and sclera was no icteric,
pupil was round, isochors, 3-3 mm, light reflex was
normal
Ears : Clear meatus acusticus externus, normal ear drums,
no secretes
Nose : There was no secretes, flare (-)
Mouth : There was no cyanosis, tonsils T1/T1 without

14
inflammatory sign, pharynx without inflammatory
sign.
Neck : There was no lymph node enlargement
Chest : Symmetrical respiratory movements, there was no
retractions
Heart : Heart margin was normal
Lungs : Bronchovesicular breath sound, trales -/-,
wheezing -/-
Abdomen : Flat, soft, with normal bowel sound,
shifting dullness (-),ascites (-),tympanic percussion,
liver and spleen were not palpable.
Extremities : Warm, no cyanotic, capillary refill time less than 2”
Genitalia : Male, no abnormality

Laboratory 5 November 2016


Hemoglobin : 8,9gr/dL ALT : 77 u/l
Hematocrit : 24,9% AST : 169u/l
Thrombocyte : 86.000 /mm3 Ureum : 15 mg/dl
Leukocyte : 2400 /mm3 Creatinin : 0,3 mg/dl
DC : 1/0/059/36/4 Prot. Total : 7,19g/dL
ANC : 1416 Albumin : 4,38 g/dL
Na : 133 mEq/L Globulin : 2,81 g/dL
K : 3,6 mEq/L
Cl : 89,5 mEq/L
Ca : 9,83 mg/dL

Diagnosis

Acute Lymphoblastic Leukemia T - cell ( C91.5) HR Consolidatian phase


W12D7 – W13 D2

Pediatric nutritional care same as before

15
Nursing care same as before

Treatment 6 November 2016

- Curlive 2 x 10 ml
- 0ralit ad lib
- 20.00 (6/11/2016 ) - 08.00 ( 7/11/ 2016 ) Hiperhidration with NaCl
0,45% in D5% + Nabic 20 mEq = 1125 ml/ 12 hours = 31 gtt/
minute
- 7/11/2016 (08.00-20.00) CPA 920 mg in NaCl 0,9 % 250 ml = 8gtt/
minute
- 20.00 (7/11/2016) – 08.00 (8/11/2016 ) Hiperhidration with NaCl
0,45% in D5% + Nabic 20 mEq = 1125 ml/ 12 hours = 31 gtt/
minute

Treatment 7 November 2016

- Curlive 2 x 10 ml

- 0ralit ad lib

- 20.00 (6/11/2016- 08.00 ( 7/11/2016 ) Hiperhidration with NaCl


0,45% in D5 % + Nabic 20 mEq = 1125 ml/ 12 hours = 31 gtt/
minute
- 7/11/2016 (08.00-20.00) CPA 920 mg in NaCl 0,9 % 250 ml = 8gtt/
minute
- 20.00 (7/11/2016) – 08.00 (8/11/2016 ) Hiperhidration with NaCl
0,45% in D5% + Nabic 20 mEq = 1125 ml/ 12 hours = 31 gtt/
minute

Pediatric nutritional care same as before

Nursing care same as before

16
12 November 2016

BW : 24,1 kg Height : 124 cm BSA : 0,91

Complaint : fever (+) since 1 day before, runny nose (+), intake
(+)

General conditions : Looked ill, compos mentis

Vital sign : Blood pressure : 90/50 mmHg

Pulse : 112times/minutes, regularly

Respiratory rate : 24 times/minutes

Temperature : 38,4 0C

Physical examination
Head : Normocephal
Eyes :Conjuntiva was not anemic and sclera was not icteric,
pupil was round, isochors, 3-3 mm, light reflex was
normal
Ears : Clear meatus acusticus externus, normal ear drums,
no secretes
Nose : There was secrete (+), flare (-)
Mouth : There was no cyanosis, tonsils T1/T1 without
inflammatory sign, pharynx without inflammatory
sign.
Neck : There was no lymph node enlargement
Chest : Symmetrical respiratory movements, there was no
retractions
Heart : Heart margin was normal
Lungs : Bronchovesicular breath sound, rales -/-,
wheezing -/-
Abdomen : Flat, soft, with normal bowel sound,
shifting dullness (-),ascites (-),tympanic percussion,

17
liver and spleen were not palpable.
Extremities : Warm, no cyanotic, capillary refill time less than 2”
Genitalia : Male, no abnormality

Laboratory Finding 12 November 2016


Hemoglobin : 5.9gr/dL Creatinin : 0,4 mg/dl
Hematocrit : 16.6% Ur : 14 mg/L
Thromboyte : 31,000 /mm3 AST : 17 u/l
Leukocyte : 470/mm3 ALT : 29u/l
DC : 3/0/4/6/71/16
ANC : 47
Na : 138 mEq/L
K : 3,16 mEq/L
Cl : 93,1 mEq/L
Ca : 9,37 mg/dL

Diagnosis

Acute Lymphoblastic Leukemia T - cell ( C91.5) HR Consolidatian phase


W13D6 + Febrile Neutropenia (D70.9)

Treatment

- Inj. Ceftriaxone 2 x 1,2 gram iv (1)


- Inj. Gentamicin 1 x 180 mg iv (1)
- GCSF 1 x1 20 mcg sc (1)
- Paracetamol 3 x 250 mg
PRC transfusion 240 ml (PCV post transfution : 20 %)

Pediatric nutritional care same as before

Nursing care same as before

18
13 November 2016 - 17 November 2016
BW : 24,1 kg Height : 124 cm BSA : 0,91
Complaint : fever (+), runny nose (+), intake (+)
General conditions : Looked ill, compos mentis
Vital sign : Blood pressure : (90/50- 100/60) mmHg
Pulse :(110-132)times/minutes
regularly
Respiratory rate : (28-36) times/minutes
Temperature : (37,2-39) 0C
Physical examination
Head : Normocephal
Eyes :Conjuntiva was not anemic and sclera was not
icteric, pupil was round, isochors, 3-3 mm, light reflex was normal
Ears : Clear meatus acusticus externus, normal ear
drums, no secretes
Nose : There was secrete (-), flare (-)
Mouth : There was no cyanosis, tonsils T1/T1 without
inflammatory sign, pharynx without inflammatory sign.
Neck : There was no lymph node enlargement
Chest : Symmetrical respiratory movements, there
was no retractions
Heart : Heart margin was normal
Lungs : Bronchovesicular breath sound, rales -/-,
wheezing -/-
Abdomen : Flat, soft, with normal bowel sound,
shifting dullness (-),ascites (-),tympanic
percussion,liver and spleen were not palpable.
Extremities : Warm, no cyanotic, capillary refill time less
than 2”
Genitalia : Male, no abnormality

Diagnosis

19
Acute Lymphoblastic Leukemia T - cell ( C91.5) HR Consolidatian
phase W13D6 – W13D7 ( postpone chemotheraphy D4 ) + Febrile
Neutropneia (D70.9)

Treatment
- Inj. Ceftriaxone 2 x 1,2 gram iv (2-6)
- Inj. Gentamicin 1 x 180 mg iv (2-6)
- GCSF 1 x1 20 mcg sc (2-6)
- Paracetamol 3 x 250 mg
- PRC transfusion 240 ml
PVC post transfusion 32%
Pediatric nutrition care same as before
Nnursing care same as before

18 November 2016 BW : 23,9 kg Height : 124 cm BSA : 0,90

Complaint : fever (-), Runny nose (+), intake (+)

General conditions : Looked ill, compos mentis

Vital sign : Blood pressure : 100/60 mmHg

Pulse : 116 times/minutes, regularly

Respiratory rate : 28 times/minutes

Temperature : 36,5 0C

Physical examination

Head : Normocephal

Eyes : Conjuntiva was not anemic and sclera was no icteric,

pupil was round, isochors, 3-3 mm, light reflex was


normal

20
Ears : Clear meatus acusticus externus, normal ear drums,

no secretes

Nose : There was no secretes, flare (-)

Mouth : There was no cyanosis, tonsils T1/T1 without

inflammatory sign, pharynx without inflammatory


sign.

Neck : There was no lymph node enlargement

Chest : Symmetrical respiratory movements, there was no

retractions

Heart : Heart margin was normal

Lungs : Bronchovesicular breath sound, rales -/-,

wheezing -/-

Abdomen : Flat, soft, with normal bowel sound,

shifting dullness (-),ascites (-),tympanic percussion,

liver and spleen were not palpable.

Extremities : Warm, no cyanotic, capillary refill time less than 2”

Genitalia : Male, no abnormality

21
Laboratory

16 November 2016 18 November 2016


Hemoglobin : 11.5gr/dL Hemoglobin :11,5gr/dL
Hematocrit : 33.3% Hematocrit :33,2%
Thrombocyte : 46.000 /mm3 Thrombocyte:72.000/mm3
Leukocyte :2753/mm3 Leukocyte :23.870/mm3
DC :0/0/2//33/60 DC : 1/4/15/50/15/15
ANC : 56 ANC : 15.515
Creatinin : 0,4 mg/dl
Ur : 14 mg/L
AST : 31 u/l
ALT : 19u/l
Na : 135 mEq/L
K : 3,07 mEq/L
Cl : 87,1 mEq/L
Ca : 9,46 mg/dL
CRP : 48 mg/L

Diagnosis

Acute Lymphoblastic Leukemia T - cell ( C91.5) HR Consolidatian phase


W13D7 ( Postpone Chemoteraphy day -5)

Treatment

- Paracetamol 3 x 250 mg
- Ambroxol 12,5 mg + CTM 2 mg
- Continue Chemotheraphy ( 19/11/226 )
Pediatric nutritional care same as before
Nursing care same as before

22
Prognosis

Ad vitam : dubia ad malam

Ad functionam : dubia ad malam

Ad sanationam : dubia ad malam

23
DISCUSSION

In this patient at first laboratory finding at 11 July 2016 , the white


bloods count was 292,000/mm2, there was relevant with T cell ALL which
is usualy has higher white blood cell count.

The cause of human leukemia is unknown. There is a 2–4 times


higher incidence of leukemia in siblings than in children in the general
population aged 0-15 years (1:720–1,000). In a monocytic twin there is an
increased risk of leukemia within months after theco-twin develops
leukemia. Higher risk in the following congenital disorders ;Trisomy 21 (14
times higher), Other trisomies, fanconi anemia, radiation, chemical and
drug ( chloramphenicol, benzene, chemical warfare agent).32

In this patient, he is the only child in his family, not founded any
congenital disorder, and never has high radiation exposured nor chemical
agent induced leukemia, and from the others family doesn't has same
illnes like the patieent.

Clinical Features of ALL shows the common clinical and laboratory.


General featrures is Fever (60%), Lassitude (50%), Pallor (40%). The
duration of symtoms is day to several weeks, occasionaly several months.
Hematologic effect tarising from bone marrow invasion :

1. Anemia—causing pallor, fatigability, tachycardia, dyspnea, and


sometimes congestive heart failure

2. Neutropenia—causing fever, ulceration of buccal mucosa, and infection

3. Thrombocytopenia—causing petechiae, purpura, easy bruisability,


bleeding from mucous membrane, and sometimes internal bleeding (e.g.,
intracranial hemorrhage).

4. One to 2% of patients present initially with pancytopenia and may be


erroneously diagnosed as having aplastic anemia or bone marrow failure

24
(represents 5% of acquired aplastic anemia) and ultimately develop acute
leukemia.

Clinical manifestation arising from lymphoid system invasion:

1. 1. Lymphadenopathy—sometimes mediastinal lymphadenopathy causing


superior vena cava syndrome.
2. 2. Splenomegaly
3. 3. Hepatomegaly

Central nervous system; At time of diagnosis less than 5% of


patients have CNS leukemia with meningeal signs and symptoms:
morning headache, vomiting, papilla edema, or focal neurological signs
such as cranial nerve palsies, hemiparesis, convulsion. Diagnosis by
analysis of cerebrospinal fluid: CNS I: no lymphoblasts; CNS II: less than 5
cells/cm3, but with leukemic blasts on centrifugation; CNS III: at least 5
cells/cm3, with leukemic blasts on centrifugation or cranial nerve palsy.4,31

In this patient at the first time came to the hospital with chief
complained of having fever since two weeks, looked pale from 2 weeks,
but didn't have symtoms of bleeding. On physycal examination was
founded hepatomegaly ( 4-4 cm bellow arcus costae), spleenomegaly
(Schuffner III). At this patient was not found symtoms of CNS involvement,
and cerebrospinal liquour analysis were not found blast cell. Laboratory
was WBC 292.000, and trombocytopenia 79.000/mm3.

The diagnosis of ALL was based on morphologic and cytochemical


evaluation of bone marrow smears as well as immunophenotyping and
cytogenetic and molecular genetic analysis of leukemic blast cells.
Depending on the pattern of blast cell reactivity to a panel of monoclonal
antibodies, cases were classified as T-cell or B-cell precursor8.

25
From Immunophenotyping 12 July 2016 the result was T Lineage
ALL, with marker was CD3, CD5, CD7.

Precursor T cell ALL and mature B cell ALL have historically been
associated with poor prognosis. Whether the outcomes in T cell ALL are
directly related to immunophenotype or due to the increased incidence of
elevated initial WBC counts and occurrence in older patients is not clear.
In a series of 125 children with T cell ALL who were treated the same as
high risk Precursor B cell ALL, there were no significant differences based
on age, presenting white blood cell (WBC) count, sex, central nervous
system (CNS) involvement, or presence of a mediastinal mass, which
could prospectively identify those children with T cell ALL who experienced
either induction failure or early relapse5.

Certain clinical and laboratory features have been historically


correlated with prognosis . Features that continue to be used in risk
stratification include:

●Initial white blood cell (WBC) count


●Age
●Cytogenetics and ploidy
●Immunologic subtype
●Rapidity of cytoreduction

White blood cell (WBC) count and age at the time of diagnosis
remain independent predictors of prognosis. They have been established
by the National Cancer Institute (NCI) as the standard criteria for risk
assignment at diagnosis in precursor B cell ALL , but are less predictive of
outcome in T cell ALL . Several studies have demonstrated a linear
relationship between initial WBC and outcome . Children with initial
WBC >50,000/microL have a more guarded prognosis and are stratified
into a higher risk group . Patients older than 10 years, or younger than one
year, also have less favorable outcomes and are assigned to higher risk
groups 5.

26
At this patient we put in High Risk category based on white blood
cell count more than 50.000/mm2, immunophenotyping where is found T
cell lineage. Annd we use "Indonesian Protocol Acute Lymphoblastic
leukemia (ALL) 2013 High Risk ".

Current treatment protocols for ALL in children emphasize risk-


based therapy in order to reduce toxicity in low risk patients while ensuring
appropriate, more aggressive therapy for those with a high risk of relapse .
Successful treatment of children with ALL involves administration of a
multidrug regimen that is divided into several phases (ie, induction,
consolidation, and maintenance) and includes therapy directed to the
central nervous system (CNS) 5,6.

Induction therapy is the initial phase of treatment. The primary goal


of induction is achievement of an initial complete remission (CR), defined
as the eradication of all detectable leukemia cells (less than 5 percent
blasts) from the bone marrow and blood and the restoration of normal
hematopoiesis (>25 percent). 6

At "Indonesian Protocol Acute Lymphoblastic leukemia (ALL) 2013


High Risk ", consolidation phase involves weekly administration of
vincristine for six weeks, daily corticosteroid (dexamethasone), L-
asparginase, and Dounorubicine8. After finish induction phase (
September 2nd, 2016 ) BMP was done wiht result BMP WITH all T-
Lineage HR Recovery phase 1st week; showing all speciment can't be
evaluated. With eritropoesis cell domination with various stage and not
found limphoid cell proliferation. From laboratory finding September 1st,
2016 white blood count was normal ( 5745/mm 3), Hb was 8,6 g/dL,
Trombosit was 181.000/mm3, with ANC 3734. There was improve from the
first laboratory finding. From clinical finding was at good condtion, and we
assest this patient was Complete remition.

Consolidation or intensification therapy is the second phase of ALL


treatment and is initiated soon after attainment of complete remission

27
(CR). Ongoing treatment is required because small numbers of leukemic
lymphoblasts remain in the bone marrow despite histologic and molecular
evidence of CR after induction therapyt cellularity and normal peripheral
blood counts). Consolidation therapy usually lasts from four to eight
months. It commonly involves the use of several different drug
combinations and drugs with mechanisms of action that differ from those
used during the induction phase. Regimens often include the following
drugs( Cytarabine, Methotrexate, Antharcyclines, Alkylating agent,
Epipodophyllotoxin) administered according to a variety of schedules to
maximize drug synergy and minimize the development of drug resistance6.

In protocol have used for this patien consolidation phase is 6


weeks with MTX IT 3 times, High Dose MTX 3 times eith leocovorin
rescue, cyclosphamid, and daily 6-MP. When this patient was assaignes
as case report he was at consolidation phase.

Improved survival also has been gained with the addition of more
intensive therapy following consolidation, an approach known as
intensification. Iintensification involves the administration of a five- to
eight-week "pulse" of intensive, multi-agent chemotherapy similar to that
administered during induction and consolidation. 6

Chemotherapy-induced febrile neutropenia remains a life-


threatening complication for patients with cancer and causes prolonged
hospitalisation. Fever is defined as a single oral temperature of 38.3◦C
(101◦F) or a temperature of 38.0◦C (100.4◦F) for 1 h. Neutropenia is
defined as a neutrophil count of 500 cells/mm3, or count of 1000
cells/mm3 with a predicted decrease to 500 cells/mm3. Currently, Gram
positive organisms are the predominant bacterial pathogens in this
setting,Staphylococcus aureus, Enterococcus spp., and viridans group
streptococci being isolated most often. Among Gram-negative bacilli,
Escherichia coli, Klebsiella spp., and Pseudomonas aeruginosa remain the
predominant species, although other Enterobacteriaceae,
Stenotrophomonas maltophilia, and Acinetobacter spp. are isolated

28
frequently. Approximately 10% to 15% of bacteremias are polymicrobial,
with Gram-negative bacilli being isolated from more than 80% ofhese
infections.

Since the 1980s, several studies have shown that the frequency of
febrile episodes and infectious diseases can be reduced with the
administration of antibiotics during the early afebrile period of neutropenia.
Empiric, broad-spectrum antibiotic therapy should be administered
promptly to all patients with febrile neutropenia. Early protocols used
combination therapy, typically a b-lactam antibiotic (third or fourth
generation cephalosporin) plus an aminoglycoside.27,30

The current treatment is supportive care plus antibiotics. Colony-


stimulating factors (CSFs), such as granulocyte-CSF (G-CSF) and
granulocyte-macrophage CSF (GM-CSF), are cytokines that stimulate and
accelerate the production of one or more cell lines in the bone marrow.28

Inthis patient we give broadspectrum antimicrobial with ceftriaxone


and gentamicin, and GCSF .

T –ALL patient were at higher risk for induction failure, induction


death,early relapse, and isolated CNS relapse, compared with B-
progenitor patients. Higher rates of induction failure and early relapse in T-
ALL suggest that this subtype may be more inherently resistant to
conventional ALL chemotherapeutic agents. Since last decade, the
outcome of pediatric B-ALL patients has been progressively improved in
China, with an overall 5-year EFS rate of approximately 85 %. However,
the prognosis of T-ALL patients was still not optimistic, with a 5-year EFS
rate of around 65 %29.

Patient in this case has prognostic dubia ad malam, where he was


entire at High Risk with white blood cell count more than 50.000/mm 3, with
T Lineage ALL, which know has a worse prognostic.

29
REFERENCES

1. Poermono Bambang,Sutaryo, Ugrasena IDG, Widiastuti Endang,


Abdusalam Maria. ed. Buku Ajar Hemato- Onkologi anak.
Jakarta:Balai penerbit IDAI, 2010; p : 236-47

2. Klingeman Robert,Stanton, St.Geme Joseph, Schor.f. Nina, Behrman


.E. Richard . Nelson Textbook of Pediatric 19th Edition.2011. Elsevier.
p:1732-49.3.

3. Elda Noronha, Andrade Gomes, etc. Immunophenotyping with CD135


and CD117 Pediatrics and gene mutation in chilhood T-cell acute
Leukemia.Blood cells,moleculs,and disease. J.bcmd.2015;12.003.

4. Lanzkowsky P. Leukemias. Manual of pediatric hematology and


oncology 4th edition. New York. Elsevier; 2005. p. 415-45.

5. Tezrah M Horton, Steuber Philip. Risk gropup stratification and


prognosis for acute Lymphoblastic Leukemia in Children. 2016.

6. Tezrah M Horton, Steuber Philip. Overview of the reatment of acute


lymphoblastic leukemia in children and adolescents. 2016.

7. Schultz KR, Pullen DJ, Sather HN, et al. Risk- and response-based
classification of childhood B-precursor acute lymphoblastic leukemia: a
combined analysis of prognostic markers from the Pediatric Oncology
Group (POG) and Children's Cancer Group (CCG). Blood 2007;
109:926.
8. Pui CH, Sandlund JT, Pei D, et al. Improved outcome for children with
acute lymphoblastic leukemia: results of Total Therapy Study XIIIB at St
Jude Children's Research Hospital. Blood 2004; 104:2690.

30
9. Smith M, Arthur D, Camitta B, et al. Uniform approach to risk
classification and treatment assignment for children with acute
lymphoblastic leukemia. J Clin Oncol 1996; 14:18.

10. Maloney KW, Shuster JJ, Murphy S, et al. Long-term results of


treatment studies for childhood acute lymphoblastic leukemia: Pediatric
Oncology Group studies from 1986-1994. Leukemia 2000; 14:2276.

11. Simone JV, Verzosa MS, Rudy JA. Initial features and prognosis in
363 children with acute lymphocytic leukemia. Cancer 1975; 36:2099.

12. Sather HN. Age at diagnosis in childhood acute lymphoblastic


leukemia. Med Pediatr Oncol 1986; 14:166.

13. Silverman LB, Gelber RD, Dalton VK, et al. Improved outcome for
children with acute lymphoblastic leukemia: results of Dana-Farber
Consortium Protocol 91-01. Blood 2001; 97:1211.

14. Reaman G, Zeltzer P, Bleyer WA, et al. Acute lymphoblastic leukemia


in infants less than one year of age: a cumulative experience of the
Children's Cancer Study Group. J Clin Oncol 1985; 3:1513.

15. Pui CH, Kane JR, Crist WM. Biology and treatment of infant
leukemias. Leukemia 1995; 9:762.

16. Chessells JM, Eden OB, Bailey CC, et al. Acute lymphoblastic
leukaemia in infancy: experience in MRC UKALL trials. Report from the
Medical Research Council Working Party on Childhood Leukaemia.
Leukemia 1994; 8:1275.

17. Pui CH, Campana D, Pei D, et al. Treating childhood acute


lymphoblastic leukemia without cranial irradiation. N Engl J Med 2009;
360:2730.

18. Vora A, Goulden N, Wade R, et al. Treatment reduction for children


and young adults with low-risk acute lymphoblastic leukaemia defined

31
by minimal residual disease (UKALL 2003): a randomised controlled
trial. Lancet Oncol 2013; 14:199.

19. Rubnitz JE, Lensing S, Zhou Y, et al. Death during induction therapy
and first remission of acute leukemia in childhood: the St. Jude
experience. Cancer 2004; 101:1677.

20. Seif AE, Fisher BT, Li Y, et al. Patient and hospital factors associated
with induction mortality in acute lymphoblastic leukemia. Pediatr Blood
Cancer 2014; 61:846.

21. Tsuchida M, Ohara A, Manabe A, et al. Long-term results of Tokyo


Children's Cancer Study Group trials for childhood acute lymphoblastic
leukemia, 1984-1999. Leukemia 2010; 24:383.

22. Tsurusawa M, Shimomura Y, Asami K, et al. Long-term results of the


Japanese Childhood Cancer and Leukemia Study Group studies 811,
841, 874 and 911 on childhood acute lymphoblastic leukemia.
Leukemia 2010; 24:335.

23. Wilson FP, Berns JS. Tumor lysis syndrome: new challenges and
recent advances. Adv Chronic Kidney Dis 2014; 21:18.

24. Truong TH, Beyene J, Hitzler J, et al. Features at presentation predict


children with acute lymphoblastic leukemia at low risk for tumor lysis
syndrome. Cancer 2007; 110:1832.

25. Goldman SC, Holcenberg JS, et al. A randomized comparison between


rasburicase and allopurinol in children with lymphoma or leukemia at
high risk for tumor lysis. Blood 2001; 97:2998.

26. Coiffier B, Altman A, Pui CH, et al. Guidelines for the management of
pediatric and adult tumor lysis syndrome: an evidence-based review. J
Clin Oncol 2008; 26:2767.

32
27. Hughes WT, Armstrong D, Bodey GP, et al. 1997 guidelines for the use
of antimicrobial agents in neutropenic patients with unexplained fever.
Infectious Diseases Society of America. Clin Infect Dis 1997;25:551–73.

28. Mhaskar R, Clark OAC et all. Colony-stimulating factors for


chemotherapy-induced febrile neutropenia (Review). The Cochrane
Librar 2014, Issue 10.
29. Wei Wei, Xiaojuan Chen, Yao Zou, Lixian Chang, Wenbin An, et all.
Prediction of outcomes by early treatment responses in childhood T-cell
acute lymphoblastic leukemia: a retrospective study in China. Wei et al.
BMC Pediatrics (2015) 15:80.
30. Garth Meckler, Susan Lindemulder. Fever and Neutropeniain Pediatric
Patients with Cancer.Emerg Med ClinN Am27.2009 p:525-544.
31. P. Imbach · T. Kühne · R. Arceci, ect. Pediatric oncology " a
Comprehensive Guide".2004.Springer.p: 6 – 27.

33
ATTACHMENTS

Patient’s Photo

34
Nutritional status

BW/A : 24/21,5 x 100% = 111%


BH /A : 124 / 118,5 x 100% = 104%
BW/BH : 24,5 /24 x 100% = 102%

35

Você também pode gostar