paket 1: hematologi
TRANSCRIPT
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Paket 1: Hematologi dr. Fauqa Arinil Aulia, Sp.PK
Sabtu, 8 Mei 2021
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Sub-topik
• Instrumentasi di bidang Hematologi
• Pem. Lab. Hemopoiesis dan Anemia
• Pem. Lab. Kelainan Sistem Lekosit dan Trombosit Non-Neoplastik
• Pem. Lab. Disfungsi Endotel, Mikroangiopati dan Makroangiopati (Cardiac Marker)
• Pem. Lab. Kelainan Hemostasis dan Fibrinolisis
• Pem. Lab. Diatesis Hemoragik dan Trombotik
• Pem. Lab. Sindroma Mielo Displastik, Leukemia Mieloblastik Akut dan Kelainan Mieloproliferatif
• Pem. Lab. Kelainan Limfoproliferatif dan Kelainan Sel Plasma
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• Penghitungan trombosit manual menggunakan reagen
• a. Hayem
• b. Turk
• c. Rees Ecker
• d. Brilliant Cresyl Blue
• E. New Methylene Blue
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Hitung Trombosit :
• Fungsi trombosit : - menghentikan perdarahan - menjaga keutuhan dinding kapiler
• Jumlah normal : 150-400 x 109/l
• Trombositosis : - Polisitemia Vera, Trombositemia idiopatik, Lekemia mielositik kronis, pasca splenektomi .
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• Trombositopenia :
- Purpura trombositopenia, anemia aplastik, lekemia akut, anemia pernisiosa, pascaradiasi/kemoterapi
- Bila trombositopenia berat → waktu perdarahan memanjang dan retraksi bekuan abnormal .
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- Kendala dalam Hitung Trombosit :
• Ukuran trombosit kecil (sulit dibedakan dari partikel2 debu/pecahan sel2 lain) dan mudah alami disintegrasi .
• Trombosit mudah beragregasi dan adakan adesi dgn permukaan asing (EDTA mencegah timbulnya agregasi)
• Jumlah trombosit dari darah kapiler lebih rendah daripada darah vena
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Cara Hitung Trombosit :
• Cara langsung : - Metode Rees-Ecker → dgn mikroskop sinar . - Metode Brecker Cronkite → dgn mikroskop fase-kontras dan lar.pengencer ammonium oxalat 1% . - Menggunakan alat hitung sel elektronik (Blood cell counter)
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Metode Rees Ecker :
• Lar.Pengencer : R/ Sodium citrate 3.8 g Brilliant Cresyl Blue 0.1 g Formaldehyde 40% 2.2 ml Aquadest ad 100 ml
• saring sebelum dipakai .
• Trombosit tercat kebiruan .
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- Prosedur penghitungan :
• Hisap darah-EDTA dgn pipet eritrosit Thoma sampai tanda ‘0.5’ dilanjutkan dgn lar.pengencer sampai tanda ‘101’ (dilusi 200 x → ?) → campur baik2 3 menit .
• Buang 4 tetes pertama lalu isi kamar hitung yg telah diberi cover-glass , biarkan 15 menit pd suasana lembab
• Hitung jumlah trombosit pada 4 kotak-W
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- Kalkulasi :
• Jumlah trombosit dlm 4 kotak-W = N
• Dilusi = 200x , luas 4 W = 0.4 mm3
• Juml.Trombo/mm3 = 1/0.4 x 200 x N = 2.5 x 200 x N = 500 N
• Catatan : - Rees Ecker tdk melisiskan eritrosit - angka kesalahan = 16-25% - pemeriksaan dilakukan < 5 jam setelah pengambilan darah .
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Hitung Trombosit cara Tidak Langsung :
• Buat HDT dgn Wright / Giemsa
• FN→menggambarkan Ø lp, tercantum di lensa okuler mikroskop
Juml.Trombo/lp
x 1000
FN-22 11 lp
FN-18 22 lp
FN<18
40 lp
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• Tehnik pada hematologi analyzer untuk memfokuskan aliran sel disebut dengan
• A. isoelectric point
• B. hydrodynamic focusing method
• C. stopped flow kinetic
• D. electromechanical clot detection
• E. photo-optical clot detection
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• Untuk menghitung indeks eritrosit membutuhkan data parameter
• A. Hemoglobin
• B. Hematokrit
• C. Eritrosit
• D. Jawaban a dan b benar
• E. Jawaban a, b, dan c benar
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Indeks Sel Darah Merah
• Indeks SDM digunakan untuk menentukan ukuran & kadar Hb dlm eritrosit ; di kalkulasi menggunakan RBC, Hb dan PCV
• Termasuk Indeks Sel Darah Merah :
1. MCV (Mean Cell Volume)
2. MCH (Mean Cell Hb)
3. MCHC (Mean Cell Hb Concentr)
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- Rumus :
MCV :
MCV = PCV / ∑ Eri (juta/cmm) x 10 (fl)
Nilai normal : * Dewasa : 76-96 fl
* Neonatus : 120 fl
* Bayi, 3 bln- 1 thn : 95 fl
* Anak, 3->6 thn : 76-92 fl
Normositik = MCV normal Mikrositik = MCV < normal Makrositik = MCV > normal
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MCH :
• MCH menunjukkan kandungan Hb rata-2 dalam 1 eritrosit .
• MCH = Hb(g/dl) / Σ Eri (juta/cmm) x 10
( dalam satuan pg)
* Nilai normal : Dewasa : 27 – 32 pg
Anak,3 bln-2 thn :24 – 30 pg
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MCHC
• MCHC menunjukkan kadar Hb rata-2 dlm 1 eritrosit . MCHC = Hb (g/dl) / PCV(%) x 100% (satuan dalam % atau g/dl)
• Nilai normal :
Dewasa & anak = 30-35 g/dl Bayi = 27.3-32.7 g/dl
Hipokrom = MCHC < normal
Normokrom = MCHC normal
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• Untuk mengetahui adanya penyakit HbH disease, pemeriksaan yang dilakukan?
• A. Cyan metHb
• B. Pemeriksaan myoglobin
• C. Hb elektroforesis dan HbH Inclusion bodies staining
• D. Laurell sulphate
• E. Serum protein elektroforesis
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• HB elektroforesis : HbA 94%, HbA2 5%, HbF 1%, diagnosisnya?
• A. HbH disease
• B. Beta talasemia trait
• C. HbE disease
• D. Alfa talasemia
• E. Heterozigositas ganda HbE Talasemia beta
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• Pemeriksaan yang khas pada talasemia beta trait adalah?
• A. HbF>4%
• B. HbH>10%
• C. HbE>10%
• D. HbS>5%
• E. HbA2>3,5%
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• Limfosit yang memproduksi immunoglobulin sebagai respon stimulasi antigenic adalah
• A. sel T limfosit
• B. sel B limfosit
• C. sel plasma
• D. timosit
• E. prolimfosit
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The third line of defence against infectious disease is the adaptive immune system, which is specific in its response It can differentiate between particular pathogens and target a response that is specific to a given pathogen It can respond rapidly upon re-exposure to a specific pathogen, preventing symptoms from developing (immunological memory)
• Lymphocytes
• The adaptive immune system is coordinated by lymphocytes (a class of leukocyte) and results in the production of antibodies
• B lymphocytes (B cells) are antibody-producing cells that recognise and target a particular pathogen fragment (antigen)
• Helper T lymphocytes (TH cells) are regulator cells that release chemicals (cytokines) to activate specific B lymphocytes
• When phagocytic leukocytes engulf a pathogen, some will present the digested fragments (antigens) on their surface
• These antigen-presenting cells (dendritic cells) migrate to the lymph nodes and activate specific helper T lymphocytes
• The helper T cells then release cytokines to activate the particular B cell capable of producing antibodies specific to the antigen
• The activated B cell will divide and differentiate to form short-lived plasma cells that produce high amounts of specific antibody
• Antibodies will target their specific antigen, enhancing the capacity of the immune system to recognise and destroy the pathogen
• A small proportion of activated B cell (and activated TH cell) will develop into memory cells to provide long-lasting immunity
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• Granula toksik adalah komponen sitoplasmik yang terdapat pada
• A. monosit
• B. limfosit
• C. eosinophil
• D. neutrofil
• E. basofil
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• Di antara kondisi berikut ini, kadar trombopoietin didapatkan paling tinggi pada
• A. ITP
• B. gangguan liver
• C. anemia aplastik
• D. trombositosis esensial
• E. trombositosis reaktif
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• Parameter yang diperiksa pada keadaan DIC
• A. Hapusan darah tepi
• B. Bone marrow aspiration
• C. D-dimer
• D. Laju endap darah
• E. Osmotic fragility test
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• Yang merupakan prekursor sel darah merah adalah
• A. Cyanonormoblast
• B. Pronormoblast
• C. Retikulosit
• D. Basophilic normoblast
• E. Eosinophilic normoblast
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• Seorang anak laki-laki didapatkan hasil hitung trombosit 21,000/mm3 pada saat pemeriksaan pra-bedah. Keadaan umum baik dan tidak ada riwayat perdarahan sebelumnya. Hapusan darah tepi menunjukkan adanya platelet clumps. Yang disarankan
• A. transfusi TC sebelum tindakan bedah
• B. menunda tindakan bedah hingga trombosit normal
• C. mengulang pemeriksaan hitung trombosit dengan sampel baru pada tabung sitrat
• D. tidak diperlukan evaluasi lanjutan pra-bedah
• E. mengulang pemeriksaan hitung trombosit dengan sampel baru pada tabung EDT
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• Monitoring penggunaan terapi warfarin adalah?
• A. aPTT
• B. Bleeding Time
• C. PT
• D. D-Dimer
• E. Fibrinogen
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Mechanism of action of warfarin
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• Monitoring penggunaan terapi heparin intravena adalah?
• A. aPTT
• B. Bleeding Time
• C. PT
• D. D-Dimer
• E. Fibrinogen
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• Kelainan berikut ini memiliki manifestasi klinik pemanjangan PPT, kecuali
• A. defisiensi FVII
• B. sirosis hepatis
• C. defisiensi FVIII
• D. terapi warfarin
• E. defisiensi vitamin K
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• Parameter berikut ini yang menggambarkan kadar fibrin degradation product adalah
• A. d-dimer
• B. plasmin
• C. protein C
• D. alpha-2 antiplasmin
• E. fibrinogen
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• Pasien dengan DIC akan mengalami peningkatan parameter berikut ini, kecuali
• A. PT
• B. APTT
• C. fibrinogen
• D. d-dimer
• E. TT
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• Pernyataan yang benar tentang rumple leed, kecuali
• A. lokasi di folar lengan atas bawah
• B. menilai fungsi trombosit
• C. menilai fragilitas vaskuler
• D. tekanan manset = (systole + diastole)/2
• E. menilai fungsi faktor pembekuan darah
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• Pemeriksaaan onkogen pada Chronic Myelocytic leukemia (CML)?
• A. JAK2
• B. TNM
• C. APL/RARa
• D. BCR-Abl
• E. MMP22
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• Pemeriksaan onkogen untuk Polisitemia rubra vera (PRV)?
• A. BCR-Abl P210
• B. APL/RARa
• C. JAK 2
• D. BCR-Abl P230
• E. MMP22
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• Pada pemeriksaan bone marrow ditemukan gambaran leukosit agranular (glassy appearance) atau pseudo pelger huet. Gambaran tersebut dapat ditemukan pada
• a. MDS
• b. CML
• c. CLL
• d. ALL
• e. AML
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MDS
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• Pemeriksaan yang termasuk dalam kriteria Durie-Salmon pada multiple myeloma adalah?
• A. Bilirubin, AFP, Transaminase
• B. Kadar gula darah, mikroalbumin, total protein
• C. Jumlah trombosit, MAP, PO2/FIO2
• D. Kalsium darah, hemoglobin, bence jones protein, beta2 mikroglobulin
• E. eGFR, Kreatinin serum
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• Anak laki-laki, 10 tahun, didapatkan demam, malaise, hepatosplenomegali. Hb 11.2 mg/dL, PLT 247,000/mikroliter, WBC 85,000/mikroliter (90% eosinofil). Aspirasi sumsum tulang didapatkan 23% limfoblas dan peningkatan precursor eosinophil. Hasil pemeriksaan sitogenetik didapatkan metaphase pada t(5;14)(q31;q32). Diagnosis pada pasien ini adalah
• A. Acute lymphoblastic leukemia
• B. Acute eosinophilic leukemia
• C. Idiopathic hypereosinophila
• D. Myelodysplasia
• E. Bi – lineage leukemia
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• Laki-laki, 72 tahun, demam hilang timbul sejak 3 minggu yang lalu, badan terasa lemah, dan sering berkeringat pada malam hari. Hb 11.8 mg/dL, WBC 9480/mikroliter (limfosit 7530/mikroliter), PLT 220.000/mikroliter. Hapusan darah tepi didapatkan dominasi limfosit. Immunophenotyping didapatkan CD5 (+), CD23 (+). Diagnosis pasien adalah
• A. Waldenstorm macroglobulinemia
• B. Multiple myeloma
• C. Limfoma Hodgkin
• D. Limfoma Non-Hodgkin
• E. CLL
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• Modalitas untuk menegakkan diagnosis leukemia adalah
• A. hapusan darah tepi
• B. aspirasi sumsum tulang
• C. immunophenotyping
• D. pemeriksaan sitogenetik
• E. semua benar
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