SEVER

E
APLAS
TIC
ANAEMI
JT KAVITHA A/P RAJA RATNAM

A
WAD STEM CELL TRANSPLAN,HOSPITAL TUNKU AZIZAH,KL.
 DEFINITON
Is a disorder of the hematopoietic stem cell that results in a loss
of blood cell precursors, hypoplasia or aplasia of bone marrow,
and cytopenias in two or more cell lines (red blood cells, white
blood cells, and/or platelets). Symptoms result from anemia,
thrombocytopenia (petechiae, bleeding), or leukopenia
(infections).

The body needs:

❖ Red blood cells – to carry oxygen.
❖ White blood cells – to fight infection.
❖ Platelets – to control bleeding.
 EPIDEMIOLOGY

The incidence of aplasic anaemia in Europe and

North America is approximately 2 per million
population and is about 2-3 times higher in East
Asia.

There is no significant difference in incidence

between men and women.
 ETILOGY
Chemicals (eg, benzene, inorganic arsenic).

Medications (eg, antineoplastic drugs, antibiotics, nonsteroidal anti-inflammatory drugs, antiseizure

medications, acetazolamide, gold salts, penicillamine, quinacrine) or toxins.

Hepatitis (seronegative for hepatitis viruses)

Pregnancy

Radiation

Viruses (Epstein-Barr virus and cytomegalovirus)

Inherited disorders of bone marrow failure due to genetic mutations (eg, Fanconi anemia, Shwachman-
Diamond syndrome, dyskeratosis congenita)

The precise mechanism remains unclear, but in the majority of acquired cases, the mechanism involves an
immune attack on the hematopoietic stem cell. Clonal hematopoiesis is frequently present, and there is a risk
of progression to a myeloid malignancy.
 SYPTOM
❖ Fatigue
❖ Shortness of breath
❖ Petechiae
❖ Ecchymosis
❖ Pale
❖ Frequent severe infection
❖ Easy bleeding
 CAUSES

Aplastic anaemia occurs when there is damage to

stem cells.As a result,bone marrow begin to slow or
shut down the production of new blood cells.

Stem cell in the bone marrow produces blood cells,

such as RBC,WBC and platelets.

The term Aplastic in Aplastic anaemia means empty

or Hypoplastic means contains very few blood cells.
 RISK FACTORS

❖ Treatment with high dose radiation or

chemotheraphy for cancer.

❖ Exposure to toxic chemicals.

❖ Certain blood diseases,autoimmune

disorders and serious infections.
 TEST AND DIAGNOSTIC PROCEDURE
❖ Complete blood count (CBC) and reticulocyte count.

❖ Bone marrow examination with cytogenetic and molecular testing.

❖ Severe aplastic anemia is defined by a bone marrow with < 25%

cellularity (hypocellularity) and the presence of ≥ 2 of the following:

Absolute neutrophil count < 500/microL (< 0.5 × 109/L)

Absolute reticulocyte count < 60,000/microL (< 60 × 10 9/L)
Platelet count < 20,000/microL (< 20 × 109/L)
Very severe aplastic anemia is defined as absolute neutrophil count <
200/microL (< 0.2 x 109/L).
 TREATMENTS
Hematopoietic stem cell transplantation.

If transplantation is not an option, immunosuppression with equine antithymocyte

globulin and cyclosporine and sometimes eltrombopag.

In aplastic anemia, hematopoietic stem cell transplantation can be curative and is the
treatment of choice, particularly in younger patients with a matched donor. At diagnosis,
siblings are evaluated for HLA (human leukocyte antigen) compatibility.

In those patients unfit for transplant or lacking a donor, immunosuppressive treatment

with equine antithymocyte globulin (ATG) combined with cyclosporine produces overall
response rates of approximately 60 to 80%. Allergic reactions and serum sickness may
occur. Long-term relapse or clonal evolution to myeloid malignancy occurs in up to half of
patients.
 TREATMENTS
Blood transfusion.

-Tranfusions of red blood cells raises red blood cell count,

which helps relieve anaemia and fatigue.

-Transfusion of platelet raises platelets counts that aids

in preventing bleeding.
 TREATMENTS
Stem Cell Transplant.

A stem cell transplant to rebuild the bone marrow with

stem cells from a donor.

Bone marrow transplant is generally the treatment of

choice for patient who have a matching donor.(most
often a sibling).
 TREATMENTS

Immunosuppressants.

For those who can’t undergo a bone marrow transplant

may be due to an autoimmune disorder,treatment may
involve drugs that suppress the immune system.
For example: cyclosporine,thymoglobulin.
 TREATMENTS
Bone marrow stimulants.

Certain drugs including colony-stimulating factors,such

as sargramostim(leukine),filgrastim (neopogen) and
pegfilgrastim (neulasta).

May help stimulate the bone marrow to produce new

blood cells.
 CASE STUDY
Patient LJY, a 8 years old male,was admitted to SCT ward for matched sibling bone marrow transplant.Innitialy before admit to us:
-Presented with multiple bruises post trauma + hematoma in March 2023
- Brought to Columbia Asia Hospital Seremban on 2/4/23 due to presence of multiple bruises over the
extremities
- examination - small haematoma on his forehead, multiple ecchymoses and petechiae on his extremities,
along with a resolving ecchymosis on his left flank
- FBC (11/4/23) - WBC 2 (ANC 240, ALC 1660), Hb 6.9 and Plt 2.
- Transferred to Sunway medical Centre for further investigations and for leukodepeleted blood products.
- FBP : pancytopenia with no blast cells
- BMT (3/4/23) - aplastic anemia
- BMA (3/4/23) - severe aplastic anemia TRO causes
- HLA 1+3 sent on 20/4/2023 - 1 fully matched siblings.

Past Medical History : H/O transfusion reaction to blood products

- in Sunway medical centre (11/04/23) - during packed red blood cell transfusion, after 100ml of transfusion,
he passed dark red urine and transfusion was immediately halted.
 CASE STUDY
April 2023 June 2023

Transferred to BMT ward on 23/6/23.

-Counselling given by BMT specialist.

-25/6/23 – developed gross hematuria with Platelet refractoriness.

-Was started onhyperhydration and IV.Methylprednisolone.

Transfused BD platelets.

-Conditioning commenced on 27/6/23 (Day -8).

Conditioned with Fludarabine,Cyclophosphamide/ATG.
 CASE STUDY
4th July
April 2023 June 2023
D -1

Transferred to PICU on 5/7/23.

-Developed multiple episodes of blood stained vomiting with malenic stool.

-HR ranging 175-185bpm,Spo2 ranging 90-94%,BP-119/80mmHg.

-Diagnosed to have Compensated Shock Secondary to UGIB.

-Transferred to PICU.Inittialy on BiPAP but needed intubation due to patient having

di culty in breathing and desaturated to 60%.Ventilated with high settings and needed
inotropic support.

-Transfused platelets BD-TDS.

 CASE STUDY
4th July 5th July
April 2023 June 2023
D -1 D0

PICU STAY FROM 5/7/2023 (D0) – 11/7/23 (D+7)

-Received bone marrow infusion and cord blood infusion

in PICU on 5/7/23.

-Bone marrow CD34 – 7.2 x 106/kg

-Patient engrafted on 17/7/23 (D+13)

CASE STUDY
CASE STUDY
 CASE STUDY
th 5th
th
4 July 5 July
April 2023 June 2023 August
D -1 D0
D +31

Discharged home on 5/8/23 (+31)

-Last review in BMT Clinic on

15/9/23(D+72).

-Patient well with no evidence of GVHD.

NURSIN
G CARE
PLAN
 NURSING CARE
PLAN
NURSING EVALUATION
GOAL INTERVENTION
PROBLEM
- Nursing
1. Observe for any intervention
bleeding tendencies no.1- no.5 is
such as petichiae, done.
ecchymosis or - No bleeding
subconjunctival bleed. seen.
Potential 2. Advise on safety - Please refer
bleeding related No bleeding measure to avoid nursing report
to low platelet seen (within bleeding. for current
count. 2 months) 3. Inform doctor if any evaluation.
bleeding.
4. Transfuse platelet
when order.
5. Send daily FBC to
monitor platelet count.
 NURSING CARE PLAN
NURSING EVALUATION
GOAL INTERVENTION
PROBLEM

1. Monitor vital sign and signs of

infection.
2. Monitor laboratory results.
3. Perform aseptic technique upon - Nursing intervention
No infection doing procedures on patient. no.1- no.6 is done.
Potential infection related
(until patient 4. Advise good hygiene: - No infection seen.
to disease.
discharge) handwashing. - Please refer nursing
5. Teach patient and families about report for current
how to prevent infection and evaluation.
signs of infection.
6. Administer IV antibiotic as
ordered by doctor.
 NURSING CARE PLAN
NURSING EVALUATION
GOAL INTERVENTION
PROBLEM
- Nursing
intervention
1. Observe colour of
no.1- no.5 is
patient.
Hb > 9g/dl 2. done.
Rest patient in bed.
or patient is - Patient is pink.
Pale related to 3. Encourage to take
pink - Please refer
low Hb balance diet.
(within 2 nursing report
4. Monitor FBC.
weeks) for current
5. Transfuse packed
evaluation.
cells when required.
THANK YOU