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Dr. Lucia Sincu Gunawan AAK NASIONAL SURAKARTA.

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Presentasi berjudul: "Dr. Lucia Sincu Gunawan AAK NASIONAL SURAKARTA."— Transcript presentasi:

1 Dr. Lucia Sincu Gunawan AAK NASIONAL SURAKARTA

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3  Protos : yang paling utama (bahasa Yunani)  Senyawa organik kompleks berbobot molekul tinggi yang terdiri dari asam amino yang diikat dengan ikatan peptida  Molekul protein mengandung karbon, hidrogen, oksida, nitrogen, sulfur& fosfor (kadang)  Ditemukan oleh : Jons Jacob Berzelius (1838)

4  Dalam tubuh protein disintesa dari ◦ asam amino yang terdapat dalam makanan ◦ dari penguraian protein jaringan ◦ dari asam amino yang dibentuk oleh aminasi fragmen carbon dari metabolisme KH dan Lipid

5  Disebut juga protein serum  Protein darah adalah protein yang ditemukan dalam plasma darah.  Total protein serum dalam darah : 7g/dl, 7 % BB tubuh

6 ◦ Transport / pembawa molekul seperti lipids (lipoprotein), hormones, vitamins and logam ◦ Enzymes, komponen komplemen, protease inhibitors, and kinin precursors protease inhibitorskinin precursors ◦ Mengatur aktivitas sel ◦ Berfungsi dalam sistem imun

7  Ratusan jenis protein beredar dalam darah ◦ Protein pembawa (seperti serum albumin, transferrin, and haptoglobinsserum albumin transferrinhaptoglobins ◦ Fibrinogen dan faktor koagulan lainnya ◦ Komponen komplemen ◦ Imunoglobulin ◦ Inhibitor enzim ◦ Prekusor zat seperti angiotensin dan bradykinin bradykinin

8  Semua protein dalam plasma diproduksi dari hati / liver kecuali gamma globulin.

9 Blood protein Normal level %Function Albumins g/dl60% create osmotic pressure and transports other molecules Immunoglobul insmmunoglobul ins g/dl participate in immune system Fibrinogens g/dl4%blood coagulation alpha 1- antitrypsin neutralize trypsin that has leaked from the digestive system Regulatory proteins <1% Regulation of gene expression

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11  Suspek Multiple myeloma  Suspek Primary amyloidosis  Neuropathy yang tidak jelas  Nyeri punggung yyang tidak jelas  Hypercalcemia dengan kecurigaan malignancy  Insufisiensi renal dengan peningkatan serum protein  Adanya fraktur patologis  Bence jones proteinuria

12  Diproduksi oleh hati  Memainkan peranan penting dalam mengatur tekanan osmotik koloid plasma  Harga normal : 3,5 – 5 gr/ dL  Penurunan kadar dalam serum dapat dijumpai pada kegagalan hati dan malnutrisi

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14  Memelihara tekanan osmotik  Transport thyroid hormoneshormones  Transports hormon lain yang larut dalam lemak  Transports asam lemak ("free" fatty acids) ke liver  Transports bilirubin tak terkonjugasi  Transports obat  Pengikat calsium secra kompetitif (Ca2+)  Buffers pHpH

15  Sirosis hepatis (paling sering)  Penurunan produksi seperti pada kelaparan  Pengeluaran melalui urin (nephrotic syndrome)nephrotic syndrome  Kehilangan melalui usus (protein losing enteropathy)  Luka bakar luas  Mutasi  analbuminemia  Penyakit akut (referred to as a negative acute phase proteinacute phase protein

16 ALBUMIN DRAINASE ASCITES

17  Berfungsi sebagai antibodi, dalam bentuk glikoprotein.  Terbagi dalam 5 kelas : Ig A, Ig D, Ig E, Ig G, Ig M, berdasarkan struktur dan aktivitas biologinya

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19  peningkatan bersama alpha 1 dan alpha 2 biasanya signifikan terjadi  peningkatan sering terjadi pada penyakit radang akut

20 KADARALPHA 1 GLOBULINALPHA 2 GLOBULIN PENINGKATANKehamilanAdrenal insufficiency Nephrotic syndrome Adrenocorticosteroid therapy Advanced DM PENURUNANAlpha 1 antytripsin defficiency Malnutrisi Anemia megaloblastik Penyakit liver berat

21 ◦ Non -specific ◦ Active liver disease, ◦ Suppurative dermatopathy ◦ Nephrotic syndrome ◦ Cushing disease ◦ Hypotyroidism ◦ Anemia deff Fe ◦ Obstructive jaundice ◦ Kehamilan trimester III High, sharp monoclonal spikes may be seen in this region due to multiple myeloma, or lymphosarcoma

22  Beta-Gamma Bridging Beta-gamma bridging is lack of clear separation between the beta and gamma fractions. (biasa terjadi pada penggunaan plasma yang mengandung fibrinogen sebagai sampel menggantikan serum)  Dalam serum beta-gamma bridging terjadi pada ◦ Chronic active hepatitis berhubungan dengan peningkatan IgM dan IgA ◦ Gammopathy ◦ Lymphosarcoma

23 ◦ Kadar rendah gamma globulins diharapkan dari pemeriksaan serum fetus dan precolostral neonates. ◦ Low level mungkin terlihat pada kasus Severe Combined Immunodeficiency (SCID) pada kelainan kongenital

24 ◦ Ada 2 : polyclonal (broad) or monoclonal (sharp) peaks. ◦ ◦ Polyclonal gammopathies  immune stimulation  chronic infections, chronic hepatitis, immune- mediated diseases or tumours. ◦ Monoclonal or oligoclonal (bi-phasic)  multiple myeloma  cutaneous lymphoma or lymphosarcoma  Amyloidosis  Immune -mediated disease.

25  Sebuah protein yang membawa besi dalam darah  Merupakan beta globulin protein  Adanya resetor transferin di permukaan sel, untuk memasukkan besi ke dalam sel.  Persentase pengikatan besi oleh transferin akan meningkat pada overdosis Fe, ataupun hemokromatosis.  Serum transferin menurun pada kekurangan protein

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27  Multiple myeloma  Sindroma nefrotik  Anemia defisiensi besi

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30  Juga dikenal sebagai plasma cell myeloma/ Kahler's disease  Adalah kanker dari sel plasma a type of white blood cell normally responsible for the production of antibodies.  Kumpulan abnormal sel terakumulasi di tulang, yang menyebabkan lesi pada tulang dan mempengaruhi sumsum tulang dalam memproduksi sel darah yang normal  anemia

31  Memproduksi paraprotein (bence jones protein), sebuah antibodi yang abnormal yang menyebabkan kerusakan ginjal  Mengganggu produksi antibodi yang normal sehingga timbul imunodefisiensi.  Hypercalcemia (high calcium levels) sering ditemukan

32  1–4 per 100,000 people per year.  It is more common in men  Twice as common in blacks as it is in whites.  With conventional treatment, the prognosis is 3–4 years, which may be extended to 5–7 years with advanced treatments.  Multiple myeloma is the second most common hematological malignancy (13%) and constitutes 1% of all cancers.[1]

33  Age The risk of multiple myeloma goes up with age. Less than 1% of cases are diagnosed in people younger than 35. Most people diagnosed with this cancer are over 65 years old.  Gender Men are slightly more likely to develop multiple myeloma than women.  Race Multiple myeloma is almost twice as common among black Americans as white Americans. The reason is not known.  Radiation Exposure to radiation may increase the risk of multiple myeloma. At most, this accounts for a very small number of cases.  Family history Multiple myeloma does seem to run in some families. Someone who has a sibling or parent with myeloma is 4 times more likely to get it than would be expected. Still, most patients have no affected relatives, so this accounts for only a small number of cases.  Workplace exposures Some studies have suggested that workers in certain petroleum-related industries may be at a higher risk.  Obesity A study by the American Cancer Society has found that being overweight or obese increases a person's risk of developing myeloma.  Other plasma cell diseases Many people with MGUS or solitary plasmacytoma will eventually develop multiple myeloma.

34  B lymphocytes start in the bone marrow  activated to secrete antibodies, they are known as plasma cells.  The proliferation of B cells and the secretion of antibodies under tight control.  Chromosomes and genes are damaged  stimulates an antibody gene to overproduction.  IN dysregulation of the oncogene  Production of cytokines  osteoporosis, angiogenesis,, produced antibodies are deposited in various organs,  renal failure, polyneuropathy

35  B lymphocytes start in the bone marrow and move to the lymph nodes. As they progress, they mature and display different proteins on their cell surface. When they are activated to secrete antibodies, they are known as plasma cells.  The immune system keeps the proliferation of B cells and the secretion of antibodies under tight control.  When chromosomes and genes are damaged, often through rearrangement, this control is lost. Often, a promoter gene moves (or translocates) to a chromosome where it stimulates an antibody gene to overproduction.  A chromosomal translocation between the immunoglobulin heavy chain gene (on the fourteenth chromosome, locus 14q32) and an oncogene (often 11q13, 4p16.3, 6p21, 16q23 and 20q11[9]) is frequently observed in patients with multiple myeloma. The chromosome 14 abnormality is observed in about 50% of all cases of myeloma. Deletion of (parts of) the thirteenth chromosome is also observed in about 50% of cases.  This mutation results in dysregulation of the oncogene which is thought to be an important initiating event in the pathogenesis of myeloma.  The result is proliferation of a plasma cell clone and genomic instability that leads to further mutations and translocations.  Production of cytokines [10]) (especially IL-6) by the plasma cells causes much of their localised damage, such as osteoporosis, and creates a microenvironment in which the malignant cells thrive. Angiogenesis (the attraction of new blood vessels) is increased.  The produced antibodies are deposited in various organs, leading to renal failure, polyneuropathy and various other myeloma-associated symptoms.

36  Blood tests (protein electrophoresis)  Microscopic examination of the bone marrow  X-rays tulang yang sering terkena.

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38  Nyeri tulang  Patah tulang  Anemia  Infeksi berulang  Lemah, bingung and lelah ( gejala hypercalcemia)  Sakit kepala, visual changes and retinopathy (akibat hyperviskositas darah karena adanya paraprotein)

39  KEMOTERAPI  Autologous stem cell transplantation (ASCT), the transplantation of a patient’s own stem cells after chemotherapy, is the most common type of stem cell transplantation for multiple myelomA

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