Inter Hospital Corrected
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Transcript of Inter Hospital Corrected
INTERHOSPITAL CONFERENCE
รพ.ขอนแก่น 17/6/2554
ผู้ป่วยหญิงไทยคู ่อายุ 33 ปี อาชีพ รับจ้างทั่วไป
ที่อยู่ อ.บ้านฝาง จ.ขอนแก่น
CC: ไข้ หอบเหนื่อยมากขึ้นมา 5 วัน PTA PI: 5 วัน PTA มีอาการไข้สูงหนาวสั่น เจ็บคอ ไอแห้ง ไม่มีเสมหะ ไม่มีน้ํามูก ปัสสาวะปกติ ไม่แสบขัด ถ่ายเหลว 8 ครั้ง ต่อวัน ถ่ายเหลวเป็นน้ํามากกว่าเนื้อ ไม่มีมูกเลือดปน ไป Admit รพ.เวชประสิทธิa์dmit 3วัน ได ้Augmentin,Ceftazidime iv + Pred 2*3 + Bactrim 2*2, Metronidazole 2*3 , CQ 1* hs อาการไม่ดีขึ้น จึงส่งตัวมารพ .ขอนแก่น
• PH: – เคยผ่าตัดต่อมไทรอยด์ที ่รพ.ศรีนครินทร์ เมื่อ 5 ปีก่อน บอกว่าไทรอยด์เป็นพิษ
– 3 เดือน PTA Dx:Possible SLE (ANA positive(nucleolar),polyarthritis, Pancytopenia ) with Lupus hepatitis (เคยมี transaminitis AST 107 ALT 108)
– CBC WBC 2200 N36% L47% M2%Band10% Hct 23.9 MCV90 MCHC33 Plt 99000
– F/U WBC 2200 -> 7000 -> 50000 with Lt. shift – BMA: peripheral destruction,stool occult blood
+ve เคย on pred 8*1tapering off มา 3 เดือน
• PH: – u/s abdomen ,True hyperechoic mass 7.5*8.9
mm, 8.6*6.5 mm Rt lobe liver likely Hemangioma ปฏิเสธ liver biopsy
– anti dsDNA neg ,AMA neg, anti smooth m Ab neg, anti HIV neg ,HbsAg neg, antiHCV neg
– SPEP: polyclonal gammopathy – Hb typing EA Bart
• Family history : Healthy
Physical examination
• A middle age woman,fully conscious ,well co-operate,looked fatigue.
• BT 38.2 0C PR 102/min RR 22/min BP 120/70 mmHg
• HEENT : mild pale, anicteric sclera, pharynx and tonsil not injected,no malar rash, no discoid rash, no oral ulcer,old surgical scar at neck,CLN impalpable,no dry lip,tongue
• HEART: PMI 5 th ICS and midclavicular line , no heaving , no thrill ,normal S1,S2, no murmur
• Lung : trachea midline, fine crepitation at Lt.lower lung
• Abdomen : soft,not tender,liver and spleen impalpable, active bowel sound
• Extremities : no petechiae,no rash, no edema
LAB
CBC Hb 9.4, Hct 28 WBC 42000 PMN 34, Lym 7, Band 14 Metamyelo 27, myelo 17 Promyelo 2 Platelet 28000 MCV 85.6 Retuculocyte 0.30
• PBS: – NCNC, few shistocyte – PMN predominate with left shift, no blast – Plt. decrease
• BUN 5 Cr 0.5 • Na 134 K 3.2 Cl 104 HCO3
23.3 Mg 2. Ca 7.8 • Uric acid 4.0 • UA sp.gr. 1.015 pH 6.0 ,Pro
trace ,Rbc 3-5,Wbc1-2
PT 12.4
INR 1.02
PTT 35.8
• LFT – TP 5.9 Alb 2.0
– Glo 3.9
Chol 138 – TB 0.4 DB 0.1
– AST 37 ALT
53 – ALP 159
LDH 167
D-dimer=negative
CXR
3 เดือน PTA ขณะ admit
• H/C : NG • G/S ,AFB,mAFB: no organism • Rectal S/C : NG • Stool exam : normal • Sputum C/S
• Numerous Kieb. Pneumoniae (ESBL) • Numerous Coagulase Negative Staphylococci
• Sent : Tazocin, Meropenem,Colistin
Abnormal granule
Flow cytometry
Flow cytometry • CD7=0.7%,CD10=2.3%, • CD11b=30.3%, CD13=99.6%, • CD15=5.1%,CD19=2.1%,
CD33=97.4%CD34=0.8%, • CD56=80.9%, CD64=1.2%, • CD117=7.4%,HLADR=1.6%, • MPO=97.1%,TdT=2.9%
Flow cytometry • CD7=0.7%,CD10=2.3%, • CD11b=30.3%, CD13=99.6%, • CD15=5.1%,CD19=2.1%,
CD33=97.4%CD34=0.8%, • CD56=80.9%, CD64=1.2%, • CD117=7.4%,HLADR=1.6%, • MPO=97.1%,TdT=2.9%
Suggestive of Acute Promyelocytic Leukemia(AML-M3)
Chromosome study
• 46,XX,t(15;17)(q22;q21)
Molecular study
• PML-RARA gene in APL: positive (bcr 1)
Progress case
• Am J Hematol. 1980;9(4):413-20.
• Unusual intracytoplasmic inclusions in acute myeloblastic leukemia.
• Wolf DJ, Fialk MA, Mouradian J, Gottfried EL, Pasmantier MW. Abstract
• Unusual intracytoplasmic inclusions within early granulocyte precursor cells from a patient with acute myeloblastic leukemia (AML) are described. Based upon their staining characteristics and electron- and light-microscopic appearance, the inclusions are distinctly different from any previously described. The inclusions display a variety of shapes, including rectangles, squares, circles, ovals, and irregular, globular forms. Most of the inclusions are refractile and crystal-like. The possible composition of these inclusions is discussed. They are compared with inclusions previously described within leukemic and granulocytic cells.
• PMID: 6163354 [PubMed - indexed for MEDLINE]
The bone marrow aspirate shows numerous abnormal promyelocytes with prominent cytoplasmic granules, characteristic of hypergranular acute promyelocytic leukemia.
Tallman M S , Altman J K Blood 2009;114:5126-5135
©2009 by American Society of Hematology
• Microgranular Variant: In the microgranular variant, M3v, the leukemic cells have a monocytic appearance with clefted angel-wing nuclei and abundant cytoplasm having at best indistinct cytoplasmic granulation.
Acute promyelocytic leukemia (M3) Bone marrow aspirate from a patient with the hypergranular promyelocytic variant of AML (FAB classification M3). (Wright-Giemsa stain). The cell in the top center and far left are "faggot" cells, with numerous intertwining Auer rods (arrows).
Acute promyelocytic leukemia (M3V) Blood smear from a patient with the microgranular promyelocytic variant of AML (FAB classification M3V). (Wright-Giemsa stain). The promyelocytes vary in size and degree of cytoplasmic basophilia. The cytoplasm contains abundant, fine, azurophilic granules; nuclei are markedly lobulated and invaginated.
Myeloblasts with Auer rods
Acute promyelocytic leukemia (M3)
Hypergranular variant APL with DIC
APL, microgranular variant
Auer rod morphology in APL
• APL represents a medical emergency with a high rate of early mortality, often due to hemorrhage from a characteristic coagulopathy
APL
• Bone marrow failure syndrome • DIC • 80% leukopenia --à hypergranular • 20% leukocytosis--à microgranular
Flow cytometry
• CD 13 ,33 MPO -à positive • CD11b CD 117 HLA-DR ->negative
Risk stratification
• Low risk ---WBC < 10000, plt >40000
• Intermediate risk --- WBC <10000,plt<40000
• High risk ---WBC>10000
• Without treatment, APL is the most malignant form of AML with a median survival of less than one month
• treatment of APL is distinct from that of other types of AML and is comprised of several stages
• Remission induction • Consolidation • Maintenance