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Hematology (Multiple Myeloma (CRAB :red_flag: (HyperCa,Renal involve…

- - - - Factor 12 11 9 8
      - :red_flag:vWF protect&carry Factor 8
    - - Factor 7
    - - Factor 10 5 2(thrombin) 1(Fibrin) 13
    - - Plasmin
    - - Factor 2 7 9 10 ,Protein C S
      - :star:Warfarin inh epoxide reductase
    - - Heparin
      - tPA (Thrombolytic) + plasmin
  - - - 1.injury : vasocon by endothelin reflex
        
        2.Exposure : vWF(จากendothelium&plt) bind collagen
        
        3.Ahesion :plt bind vWF via GpIb, Plt หลั่ง ADP(ช่วยplt adhere to endothelium) ,Ca,TXA2
        
        4.Activation : ADP bind P2Y12 induce GP2b3a express on Plt
        
        Aggregate : fibrinogen bid Gp2b3a and link Plt, Balance Pro-aggregation factor & ANti-agg factor ช่วย tempolary stop bleed
        
        Secondary (Coag cascade)
        
        Pro-agg factor
        
        TXA2 by plt
        
        Anti-agg factor
        
        PGI2 & NO by endothelium
        
        :star:Clopidogrel/Prasugrel/ticlopidine : inh ADP bind P2Y12(Gp2b3a)
        
        :star:Aspirin irreversible inh TXA2 synth(Cyclogenase)
        
        :star:Ristocetin ช่วยกระตุ้นการจับของvWF&GpIb
        Fail in vWD&Bernard
- - - - Abetalipoproteinemia, Liver ds
    - - Lead poison,Sidero anemia,MDS
    - - Myelofibrosis(BM infiltrate)
    - - G6PD def
    - - ESRD
    - - Megaloblastic anemia
    - - MAHA(DIC,TTP,HUS,HELLP),heart valve prosthesis
    - - Hereditary spherocytosis
    - - Thalassemia, Liver disease, Asplenia
    - - Hypoplenia/ Asplenia
  - - - Sepsis/Postinfection, Aplastic anemia, SLE,radiation
    - - HIV, DiGeorge syndrome, SCID, SLE, corticosteroids
    - - Cushing syndrome,corticosteroids
    - - immature cell
  - - - ALA ,porphobilinogen
      - Symptoms 5P
        
        painful abdomen, port wine urine, polyneuropathy, Psycho, precipitate by drug,alcohol ,starv
    - - Uroporphyrin(Tea urine)
      - Blister cutaneous photosens/hyperpig trigger by alcohol
  - - - Factor 1 2 5 7 10
    - - F/U warfarin
    - - Factor ที่ไม่ใช่ 7 13
    - - A : factor 8 :arrow_up:PTT XR
        
        Tx : Desmopressin + factor8 conc.
      - B : factor 9 :arrow_up:PTT XR
        
        Tx : factor9 conc.
      - C : factor 11 :arrow_up:PTT AR severe
        
        Tx : factor11 conc.
    - - :arrow_up:BT intrinsic defect :arrow_down:vWF :arrow_up:PTT defect Plt plug formation , AD Tx : Desmopressin
    - - :arrow_up:PT/PTT/BT :arrow_down:Plt clotting factor defect, schitocytes :arrow_up:D-dimers
      - cause : Gr.neg sepsis, truama,obstetic comp, pancreatitis, Malignancy, Nephrotic ,transfusion
  - - - defect plt plug formation, :arrow_down:GpIb(pt to vWF)
    - - defect Gp2b3a(Plt to Plt)
    - - :arrow_down:Plt,MAHA,ARF มักพบในเด็กมาด้้วยท้องเสียจากEHEC adultจะคล้ายTTP tx : plasmapheresis
    - - :arrow_down:plt, AntiGp2b3a Ab, Splenic macrophage, Megakaryocytes Tx : steroid,IVIG,splenectomy
      - cause : idiopathic, viral infection,AI,CA
    - - :arrow_down:plt , :arrow_up:LDH ,defect ADAMTS13 เกิด Plt agg,thrombosis Tx : plasmaparesis,steroid
      - Symptoms : Pentad of neurologic,renal,fever,thrompocytopenia,MAHA
- - - - LAB : :arrow_down:Fe , :arrow_down:Ferritin , :arrow_down:%transferrin sat, :arrow_up:TIBC,
      - Symp : เพลีย กินของแปลกpica
    - - Hb Barts(ขาด4ตายเรียบ g4)
      - HbH disease(b4 ขาดa3)
      - ขาด2/ขาด1 ยังดี
    - - B thal minor(heterozygote) : HbA2>3.5% ไม่มีอาการ
      - B thal major ต้องได้เลือด Chipmunk face ตับม้ามโต เจอtarget cell
    - - PBS : basophillic stripping
      - L(lead line/Burton lineที่เหงือกและlong bone)E(encep)A(abdominal colic)D(wrist/foot drop)
      - Tx : Deferoxamine
    - - genetic/MDS/Lead B6 Cu def/Isoniazid LABตรงข้ามกับFe def
      - Tx : pyridoxine(B6)
  - - - impared DNA synth เจอ hypersegmented neutrophil
    - - NO neuro symptoms,มักมาจากmalnu/alcoholics/ยา เช่น MTX,TMX,phynytoin
    - - Veganism,Crohn disease,Gastrectomy,Diphyllobothrium latum
      - Neuro symptoms; reversible dementia
  - - - :arrow_down:Heptaglobin, เจอschistocytes,hemoglobinuria,unconj bilirubin
      - Prosthetic valve,PNH,MAHA
        
        PNH
        
        Triad : Coomb-,pancytopenia,venous thrombosis
        
        Tx : Eculizumab
    - - Spherocytes, NO hemoblobinuria
      - Hereditary spherocytosis, AIHA
        
        HS
        
        ขาดโปรตีนRBC memb,skeleton,plasma membrane
        
        ankyrin,band3
        
        LAB : OF+
        
        Tx : splenectomy
        
        AIHA
        
        Warm(IgG)
        
        Chrnoic anemia in SLE,CLL,drug(methyldopa)
        
        Cold(IgM&compliment)
        
        Acute trigger by cold in CLL,M.pneumo,IM
        
        LAB : Coombs+
    - - Anemia of chronic disease
        
        hepcidin inh Fe trasport ได้แลป :arrow_up:ferritin :arrow_down:TIBC
        
        Tx : Cause แต่ถ้า CKD ให้ EPO
      - Aplastic anemia
        
        Failure of Myeloid stem cells
        
        cause
        
        Radiation/Drug : benzene ,Chloram
        
        ParvoB19,EBV,HIV,hep
        
        Fanconi syndrome(DNA repair defecrt) : macrocytosis,short
        
        idiopathic
        
        :arrow_down:reti count, :arrow_up:EPO, pancytopenia
        
        Tx : withdraw agent,immunosuppress;cyclosporine,BM allograft,transfusion,BM stim(GM-CSF)
- - - - เด็ก assoc.Down syndrome ตอบสนองดี
      - T-cell ALLอาจมาด้วย Mediastinal mass;SVC like syndrome
      - PBS : Lymphoblast++ , CD10+
    - - M/C adult>60
      - PBS : smudge cell, AIHA
  - - - 65 assoc.Down syndrome
      - มักมาด้วย DIC
      - Myeloperoxidase+,PBS : myeloblasts++
    - - 45-85
      - Philadelphia chromosome t[9;22],BCR-ABL
      - มักมาด้วยม้ามโต
      - Tx : bcr-abl tyrosine kinase inh : Imatinib
- - - - Burkitt lymphoma
        
        young,t(8;14), Starry sky with tingible body macrophage,assoc.EBV, Jaw lesion
      - DLBCL
        
        adult,Bcl2,6, M/C type
      - Follicular
        
        t(14;18)CD5+, painless waxing&waning lymphadenopathy
      - Mantle cell
        
        aggressive, Male adult
      - Marginal zone
        
        assoc. chronic inflam(Sjogren/MALT)
      - primary CNS
        
        M/C assoc HIV/AIDs,EBV มาด้วยสับสน จำไม่ได้ ชัก
    - - Adult T cell lymphoma
      - Mycosis fungoides/Sezary