Scenario 2 SGD IBlock X : Immunology

Skmarina Hanna Larose S.P. Vesper J. N. ZamiliAdolf Finsensius SarumahaAgnes Meinulo DuhaAlhoi Lesley DavidsonAnnisa Maichie KurniasihEvie Clarensia LaseLouis Mapolo Sutian

Maruli Freddy SimamoraNikko Fernando VenesiaNita Fatmasari BangunRosnilam MohoSiti RamadhaniSri Wahyu Ratnasari NasutionTiorasi Pakpahan

Scenario

Adi, a six-year-old-boy, can’t take school activities in his school for six days because of having severe fever, myalgia and anorexia, but lately, Adi’s face comes paler, his heart beats faster, malaise, lose appetite, bleeding around his gums and petechiae, ecchymosis in superficial. Adi has history of eating Chlorampenicol often and antipiretic which are bought by his mother. Hence Adi’s condition is worse, Adi is taken to pediatrician. Doctor suggests to do a complete blood examination and Bone Marrow Punction. What has happened to Adi and how is the management?

Classification of Terms• Myalgia : pain on muscle• Ecchymosis : patch of bleeding under skin with

diameter > 2 mm• Petechiae : patch of bleeding under skin with

diameter < 2 mm• Anorexia : appetite disruption• Bone Marrow Punction : an examination by doing

aspiration of bone marrow liquid

Problems Determination

1. Adi, a six-year-old-boy, can’t take school activities in his school for six days because of having severe fever, myalgia and anorexia, but lately, Adi’s face comes paler, his heart beats faster, malaise, lose appetite, bleeding around his gums and petechiae, ecchymosis in superficial.

2. Adi has history of eating Chlorampenicol often and antipiretic which are bought by his mother.

3. Doctor suggests to do a complete blood examination and Bone Marrow Punction.

Analysis of Problems

1. - Probably caused by infection- Probably caused by aplastic anemia- Patient has hemostatic disfunction- Probably having thrombosis disfunction- Probably having pancytopenia

2. - Probably caused by toxin of medicines - Lack of knowledge of patient’s mom

3. To uphold the final diagnose

Temporary conclusion

Adi, a-six-year-old-boy, probably is having anemia aplastic

Learning Objectives1. Classification of anemia2. Definition of aplastic anemia3. Etiology of aplastic anemia4. Epidemiology of aplastic anemia5. Classification of aplastic anemia6. Pathophysiology of aplastic anemia7. Clinical symptoms of aplastic anemia8. Anamnesis of aplastic anemia9. Physical examination of aplastic anemia10.Supporting examination of aplastic anemia11.Management of aplastic anemia12.Prevention of aplastic anemia13.Complication of aplastic anemia14.Prognosis of aplastic anemia

1. Classification of anemia

A. Based on etiopathogenesisI. Disturbance in erythrocytes formation and

marrow bonea. Deficiency of essential

a. Iron Deficiency b. Folic acid deficiencyc. Vitamin B12 deficiency

b. Disturbance in Iron utilizationa. Chronic diseaseb. Sideroblastic anemia

c. Marrow bone destructiona. Aplastic anemiab. Mieloptisica anemiac. Hematologic anemiad. Dyserythropoiesis anemiae. Myelodisplastic syndrome

d. Deficiency of erithropoiesisII. Hemorrhagic causeIII. Hemolitic causeIV. Unknown and complex pathogenesis

B. Based on morphologyI. Hypochromic microcyticII. Normochromic normocyticIII. Macrocytic

a. Megaloblasticb. Non-megaloblastic

2. Definition of aplastic anemiaAplastic anemia is a condition caused by decreasing of peripheral blood hematopoietic cells such as erythrocytes, leukocytes and platelets as a result of the cessation of cell production in bone marrow hemopoetic.

3. Etiology of aplastic anemiaa. Congenital Factor : Fanconi syndromeb. Acquired factors :• Chemicals : Benzene, insecticides, compound As, Au, Pb.• Drugs : Chloramphenicol, mesantoin (anticonvulsants),

piribenzamin (antihistamines ), santonin - calomel, a drug sitostatica (myleran, methrotrexate, TEM, vincristine, rubidomycine etc. ), anti- tumor drugs (nitrogen mustard), anti- microbial.

• Radiation : Roentgen rays, radioactive.• Infection : Miliary tuberculosis, hepatitis and others -

others.• Malignancy : Kidney disease, endocrine disorders, and

idiopathic.

4. Epidemiology of aplastic anemia

The incidence of acquired aplastic anemia varies around the world and range from 2 to 6 cases per 1 million population per year with geographic variation. Acquired aplastic anemia generally appears at the age of 15 to 25 years; peak incidence appears after the age of 60 years.

5. Classification of aplastic anemia I. Primary 1 . Congenital abnormalities 2 . Idiopathic II . Secondary : 1 . Due to radiation, chemicals, or drugs 2 . Due to other causes : a. Virus Infection : Hepatitis Viruses / other

viruses b . Due to pregnancy c . Radiation

6. Pathophysiology of aplastic anemia

7. Clinical symptoms of aplastic anemia

1. Tachycardia2. Increasing of breathing frequency3. Pale4. Weakness5. Dizzy6. Nausea7. Decreasing quality of skin and hair

8. Anamnesis of aplastic anemia

a. Complaints eritroblastopeniab. Complaints thrombocytopeniac. Complaints eukopeniad. History of treatment :• Consume drugs in a long period of time• Chemotherapy• Often exposed to radiation like x-rays and

radioactive rontgene. previous Illness Historyd. Family history of sitopenia

9. Physical examination of aplastic anemiaa. Inspection :

1. Pale2. There are bruises, petechiae, or ecchymosis3. bleeding gums

b. Palpation :1. body temperature increase2. cold extremities3. tachicardy4. Hepatomegaly (found in a several patients)

c. Percussion :Hepatomegaly (found in a fraction of patients)

d. Auscultation :gallop rhytm and wheezing

10. Supporting examination of aplastic anemia1. Laboratory Examinationa. Peripheral Blood

According to The International Agranulocytosis and Aplastic Anaemia Study (IAAS) is called anemia aplastik when:

i. the levels of hemoglobin ≤ 10 g/dl or Hematokrit ≤ 30%ii. Count of platelets ≤ 50,000/mm3iii. Leukocyte Count ≤ 3500/mm3 or Granulocytes ≤ 1.5 × 109/l

2. bone marrow Biopsy3. Radiological Examination

1. Nuclear Magnetic Resonance Imaging2. Radionuclide Bone Marrow Imaging (Scanning Bone Marrow)

Diagnosis differential1. Idiopathic Thrombocytopenic Purpura (ITP) and Thrombocytopenic Purpura Amegakaryocytic (ATP)2. Type of aleukemik acute Leukemia, especially ALL (Acute Lymphocytic Leukemia)3. Preleukemik Stage of acute leukemia

11. Management of aplastic anemiaManagement1. Supportive therapy

1. Leukocyte-poor red cells Transfusion (overcome anemia)

2. Platelet suspension transfusion (Overcome bleeding)3. Antibiotics ( for infections, recommended antibiotics are

b-lactam and aminoglycosides)4. Blood, urine, faeces, sputum, CSF culture5. Avoiding the chemical and physical toxic and drugs.

2. Medication Therapy1. Androgen

Oksimetolon is better instead of testosteron, with dose 1-2 mg/kg/day2. Imunosuppressive

a. Methylprednisolon ( 5 mg/kg IV 8 days then tappering)b. Antilymphocyte-Globulin (40mg/kg IV for 12hours

+methylprednisolon 1mg/kg/hours IV for 4 days)c. Antithymocyte-Globulin (100-2000mg/kg IV)d. Cyclosporin A (8mg/kg/day PO for 14 day continue

with 15mg/kg/day)e. Cyclophosphamide (50mg/kg/day for 4 days, still

controversy)3. Growth factor ( G-CSF with dose 5 ig/kg/day)

4. Combination therapycombination of ALG/ATG, methylprednisolon and CsA.

3. Blood Transfusion4. Chelation Therapy

given when there is accumulation of iron due to blood transfusion

5. bone marrow transplant6. Food7. Rest

Relapsin case of relaps then can be given CsA and if unsuccessful then can be given repeat ATG

12. Prevention of aplastic anemia

Prevention1. Avoiding toxic materials2. Avoiding drugs consumption which can trigger

aplastic anemia3. Avoiding radiation4. Good hygiene5. Management of bleeding and infection

13. Complication of aplastic anemia

1. Infection2. Bleeding3. GVHD after transplantation4. Death

14. Prognosis of aplastic anemia

Based on :1. Bone Marrow representation2. HbF > 200 mg% give a better prognosis3. Granulocyte > 2000/mm3 give better prognosis

4. Secondary infection prevention

Final Conclusion

Diagnose : Adi, 6-year-old-boy, suffers Aplastic anemiaSupporting examination :• Peripheral blood count to see pancytopenia,

morfology of erythrocyte, leucocyte and thrombocyte and count the sums.

• Bone Marrow Punction to see bone marrow representation.

Management :• Stop using Chlorampenicol• Give supporting therapy, such as :• Make sure the etiology of Adi’s fever, if it is caused by

bactery infection, give antibiotic, such as B-Lactam and Aminoglicoside• Take care of self-hygiene

• Result of supporting examination :• If Hb , 7 gr% then do transfusion of red-packed cell• If thrombocyte < 20.000/uL then do thrombocyte

transfusion

• Observation patient, wether Adi’s condition comes better. If not do combination therapy, consist of :• Methylprednisolon 5 mg/KgBW IV for 8 days, then

tappering until 1 mg/KgBB/day for 9-14 days.• ALG is given 40 mg/KgBW for 12 hours, continued with

infuse that combinated with Methylprednisolon.• Siklosporin A is given 8 mg/KgBB

• Last option is Transplation of Bone Marrow

Thank you..