Pediatric hematology oncology inter hospital conference
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PEDIATRIC HEMATOLOGY/ONCOLOGY INTER-HOSPITAL CONFERENCE. King Chulalongkorn Memorial Hospital. 16 NOV 2010. History. 2. ผู้ป่วยเด็กชายไทย อายุ 2 เดือน ภูมิลำเนาเดิมและปัจจุบัน สมุทรปราการ รับไว้ในโรงพยาบาลครั้งที่ 1 เมื่อ 15 มีนาคม 53 ประวัติได้จากใบส่งตัวและมารดา เชื่อถือได้

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Pediatric hematology oncology inter hospital conference

PEDIATRIC HEMATOLOGY/ONCOLOGYINTER-HOSPITAL CONFERENCE

King Chulalongkorn Memorial Hospital

16 NOV 2010


History

History

2

  • ผู้ป่วยเด็กชายไทยอายุ 2 เดือนภูมิลำเนาเดิมและปัจจุบันสมุทรปราการ

  • รับไว้ในโรงพยาบาลครั้งที่1 เมื่อ15 มีนาคม 53 ประวัติได้จากใบส่งตัวและมารดาเชื่อถือได้

  • Chief complaint : ไอ 2 สัปดาห์ก่อนมาร.พ.

  • Present illness : 2 สัปดาห์ก่อนมาร.พ. มีอาการไอแห้งไม่หอบเหนื่อยไม่มีไข้ไม่ซึมปัสสาวะอุจจาระปกติไม่มีจ้ำเลือดตามตัวผิดปกติไม่มีสำลักมีอาการนาน 1 สัปดาห์จึงไปพบแพทย์ที่คลินิกได้ยาแก้ไอยาฆ่าเชื้อไม่ทราบชนิดมารับประทาน

    2 วันก่อนมาร.พ. อาการไม่ดีขึ้นไอมากแม่สังเกตเวลาทานนมไอมากขึ้นจึงไปพบแพทย์ที่โรงพยาบาล


History1

History แรกรับที่โรงพยาบาลจังหวัดสมุทรปราการ

3

Physical exam: pale, tachypnea.

  • Lungs: crepitation both lungs with subcostal retraction both lungs.

  • Abdomen: Liver1 cm. BRCM , spleen not enlarge

    Investigation

  • CBC: Hb. 5.9 g/dL Hct. 18.7% MCV 89 fL

    WBC 15,790/mm3 N50% L37% Mo7% Eo 5% Ba1%

    Platelet 27,000 /mm3

    PBS: anisocytosis 1+, poikilocytosis 1+, microcyte 1+ , tear drop few, decrease platelet.

  • CXR : RUL LUL patchy infiltration

  • Impression : pneumonia and anemia with thrombocytopenia

  • Rx : Ampicillin100 mg/kg/day , erythromycin, PRC10 ml/kg จึงส่งตัวมารักษาต่อที่โรงพยาบาลจุฬา


  • Pediatric hematology oncology inter hospital conference

    14/3/53


    Pediatric hematology oncology inter hospital conference

    5

    • Past history

      • ไม่เคยเจ็บป่วยเข้าโรงพยาบาลก่อนหน้านี้

      • ปฏิเสธประวัติการแต่งงานในเครือญาติ

      • ปฏิเสธสมาชิกในครอบครัวป่วยเป็นโรคเลือด/มะเร็ง

      • ขณะอายุ 4 วันแม่พาไปกวาดลิ้นหลังจากนั้นลิ้นเป็นแผลไปคลินิกได้ยาฆ่าเชื้อมาทานไม่ทราบชนิดหลังจากไม่มีอาการอีก

      • อายุ7วันมีผื่นแดงที่ขาหนีบไปคลินิกบอกว่าเป็นผื่นผ้าอ้อมได้ยามาทา


    Pediatric hematology oncology inter hospital conference

    6

    • Pediatrics history :

      • Prenatal : มารดาผลเลือดปกติ , serology neg. all

        ระหว่างตั้งครรภ์ไม่มีไข้หรือเจ็บป่วย

      • Perinatal : บุตร1/1, birth weight 2,970 gm., term NL ,no complication แรกเกิดมีปัญหา microcephaly head circumference 30 cm (< P3)

      • Postnatal : กินนมแม่ 3 มื้อ/วันร่วมกับนมผสม 2 oz. x 8 feeds

      • Immunization : แรกเกิดได้รับ BCG, HBV

      • G&D : ชันคอได้จ้องมองตา


    Pedigree

    Pedigree

    7

    72 ปี

    63 ปี

    42 ปี

    47 ปี

    พนง.บบริษัทเครื่องดื่ม

    ไม่มีโรคประจำตัว

    อยู่กทม.

    พนง.บริษัทเครื่องดื่ม

    ไม่มีโรคประจำตัว

    อยู่กทม.

    ไม่ได้ทำงาน

    ไม่มีโรคประจำตัว

    อยู่จ.กำแพงเพชร

    ไม่ได้ทำงาน

    ไม่มีโรคประจำตัว

    อยู่จ.กำแพงเพชร

    I

    II

    21ปี

    19ปี

    22ปี

    42 ปี

    40ปี

    38ปี

    36ปี

    35ปี

    34ปี

    32ปี

    ไม่ได้ทำงาน

    ไม่มีโรคประจำตัว

    อยู่กทม.

    ทหาร

    ไม่มีโรคประจำตัว

    III

    ผู้ป่วย 2 mo.


    Physical examination

    Physical examination

    8

    • GA: A Thai male infant active , tachypnea

    • Measurement : Weight 3.22kg (<P3) ,Length 52 cm(<P3), HC 33cm (<P3)

    • V/S: BT 37.2 ◦c PR110/min BP 86/50 mmHg

    • RR 38/min SpO2 97%

    • Skin: no rash, no petechiae, no ecchymosis

      BCG scar positive

    • HEENT: AF: 2x2cm., not bulging, not depress ,PF: FT

      Eye: not pale conjunctiva ,anicteric sclera

      Ears: intact both TM.


    Physical examination1

    Physical examination

    9

    • RS : subcostal retraction, medium crepitation both lungs, occasional rhonchi, inspiratory stridor

    • CVS : normal S1S2 ,no murmur

    • Abd : not distension, soft, liver 1 cm BRCM span 6cm,

      no splenomegaly

    • Musculoskeletal : no edema, capillary refill time<2 sec

    • NS : active, pupil 3 mmRTLBE, equal movement , Brudzinski sign negative, DTR 1+ all extremities.


    Head circumference

    Head circumference

    11


    Problem lists

    Problem lists

    12

    • Thrombocytopenia with anemia

    • Failure to thrive with microcephaly

    • Pneumonia


    Differential diagnosis

    Differential diagnosis

    13

    • Infection

      • Pneumonia

      • Septicemia

      • Congenital infection: ToRCH infection

      • HIV infection

    • Bone marrow disease

      • Congenital bone marrow failure

      • Infiltrative disease


    Initial investigation 15 53

    Initial investigation (15 มีนาคม 53)

    14

    • CBC post PRC transfusion

      • Hb 9.6 g/dL, Hct 30.2%, MCV 89 fL., RDW 16%

        WBC 10,610 /mm3 (N 61% L 23.3% Mo 6.1% Eo 3.9% Ba 0.6%) Platelet 39,000/mm3

      • Reticulocyte count 3.1%

      • Absolute neutrophil count = 6,472/mm3 Absolute lymphocyte count = 2,493/mm3


    Pediatric hematology oncology inter hospital conference

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    Pediatric hematology oncology inter hospital conference

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    Initial investigation 15 531

    Initial investigation (15 มีนาคม 53)

    17

    • BUN 5 g/dl Cr 0.2 d/dl

    • Electrolyte Na 134 mmol/L K 3.1 mmol/L

      Cl 105 mmol/L HCO3 26 mmol/L

    • LFT : TB 0.56 mg/dL DB 0.24 mg/dL

      SGOT 69 U/L, SGPT 49 U/L Alp 117 U/L

      Alb 2.7 g/L globulin 2.6 g/L TP 5.3 g/L

    • Calcium 9.3 mmol/L


    Pediatric hematology oncology inter hospital conference

    CXR

    18

    15/3/53


    Investigation

    Investigation

    19

    • KOH preparation at groin : pseudo hyphae and budding yeast cells

    • H/C: no growth

    • Stool occult blood: negative


    Investigation1

    Investigation

    20

    • TORCH titer (15/3/53)

      • Rubella IgG negative

      • Rubella IgM negative

      • Anti CMV IgG positive (147.12 unit) (cut off 40 unit )

      • Anti CMV IgM negative

      • Anti HSV IgG negative

      • Anti HSV IgM negative

      • CMV viral load: pending


    Investigation2

    Investigation

    21

    • Anti-HIV : negative


    Bone marrow aspiration

    Bone marrow aspiration

    22

    • BMA (16/3/2553)

      The specimen shows hypercellular marrow with mild erythroid and myeloid hyperplasia and normal maturation. Megakaryocytes were increased. Mildly increased mature histiocytes were observed.

      Conclusions : Increase peripheral destruction of platelet and erythroid series.


    Pediatric hematology oncology inter hospital conference

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    Pediatric hematology oncology inter hospital conference

    24


    Pediatric hematology oncology inter hospital conference

    25


    U s abdomen 18 3 53

    U/S abdomen (18/3/53)

    26

    • Mild splenomegaly size ~ 6.2 cm , normal size of liver , unremarkable otherwise.


    Film lateral ap skull

    Film lateral + AP skull

    27


    Diagnosis

    Diagnosis

    29

    • Infection

      • Pneumonia √

      • Candidiasis √

      • CMV infection ?

      • Septicemia x

      • HIV infection x

    • Bone marrow disease x

      • Congenital bone marrow failure

      • Infiltrative disease

    Anemia with thrombocytopenia due to peripheral destruction


    Treatment

    Treatment

    30

    • Antibiotics: ampicillin 100 mg/kg/day

    • Platelet transfusion: 1 unit

    • Topical cotrimazole

    • Oxygen support: canula 0.5 LPM


    Progress note

    Progress note

    31


    Admission day 4 19 3 53

    Admission day 4 (19/3/53)

    32

    ผู้ป่วยไข้สูงหายใจเร็วเหนื่อยกินนมลดลงซึม

    Diagnosis: pneumonia, respiratory failure with septic shock

    • CBC: Hb 7.9 g/dl Hct25.1% WBC15,790/mm3 (N91% ,L 1%, Mo 3%) Plt 50,000/mm3 ALC 1,579

      • H/C: E. coli

      • TSC, stool C/S, urine C/S :candida albicans

    • Treatment

      • ETT 5 days

      • Antibiotics: meropenam + fluconazole

      • PLT and LPRC transfusion


    Cbc 2553

    CBC มีนาคม 2553

    33


    Admission day 10 25 3 53

    Admission day 10 (25/3/53)

    34

    • Extubation ยังมีไข้ต่ำๆหายใจหอบเท่าเดิม

    • CBC : Hb 10.7 g/dl Hct 32.8% WBC 6,240/mm3 (N 62% ,L 14% , Mo 5%) Plt 74,000/mm3

    • CMV viral load 52,800 copies/ml

    • Treatment: O2 Box 10 LPM

      • Gangcyclovir 5 mg/kg/dose q12hr(25/3-12/5)

      • LPRC 10 ml/kg.

      • Plt. Conc. 1unit


    Cbc 25531

    CBC มีนาคม 2553

    35


    Problem lists1

    Problem lists

    36

    • Thrombocytopenia and anemia

    • Failure to thrive

    • Microcephaly

    • Pneumonia

    • Systemic candida infection

    Infection : CMV


    Progress

    progress

    37

    Rota diarrhea with central line infection with hypovolemic and septic shock


    Cbc 25532

    CBC มีนาคม 2553

    38


    Pediatric hematology oncology inter hospital conference

    31 /3/53


    Progress1

    progress

    40


    Pediatric hematology oncology inter hospital conference

    41

    Ganciclovir start 25/3/53


    2 nd bone marrow aspiration 19 4 53

    2nd Bone marrow aspiration 19/4/53

    43

    • Hypercellular marrow ,increase of megakaryocyte  peripheral destruction


    Progress2

    progress

    44

    Thrombocytopenia from immune process


    Progress3

    progress

    45


    Pediatric hematology oncology inter hospital conference

    46


    Progress4

    progress

    47

    Oral candidiasis and skin candidiasis

    Pneumonia


    Pediatric hematology oncology inter hospital conference

    CXR

    48


    Pediatric hematology oncology inter hospital conference

    CBC เดือนพ.ค.-มิ.ย.

    49

    IVIG 2.5 gm. 3/6/53


    Cmv viral load and platelet count

    CMV viral load and platelet count

    50

    Ganciclovir (23/3-12/5)

    Re-start 31/5 


    Cmv viral load and platelet count1

    CMV viral load and platelet count

    51

    Ganciclovir (31/5-25/8)


    Pediatric hematology oncology inter hospital conference

    15/3/5319/3/53 23/3/53

    Pneumonia Respiratory failure Systemic fungal with septic shock infection

    25/3/53 31/3/53 22/4/53

    CMV infection Rota diarrhea +Pneumonia Phlebitis lt.foot

    27/5/53 3/6/53 30/6/53

    CMV infection Oral candidiasis Pneumonia

    Skin candidiasis


    Problem lists2

    Problem lists

    53

    • Multiple infection

      • CMV infection

      • Recurrent pneumonia with respiratory failure with sepsis

      • Systemic candida infection

    • Persistent Thrombocytopenia

    • Eczema


    Immunodeficiency wiskott aldrich syndrome

    IMMUNODEFICIENCY ∞WISKOTT-ALDRICH SYNDROME


    Investigation3

    Investigation

    55

    • Complete blood count

      • Absolute lymphocyte count = 2,493 /mm3

      • Absolute neutrophil count = 6,472/mm3

      • Platelet count =39,000 MPV = 7 fL


    Pediatric hematology oncology inter hospital conference

    56

    • Screening test for B-cell defects

      • IgG 1140 (normal 340 +/- 119)

      • IgM 59.1 (normal 30 +/- 11)

      • IgA 113 (normal 21 +/- 13)

      • Total IgE 3,750 (<15)


    Pediatric hematology oncology inter hospital conference

    57

    • Screening test for T-Cell defect

      • Absolute lymphocyte count = 2,493 /mm3

      • Candida albican intradermal skin test positive

      • PHA : impair T-cell function

    • Screening test for phagocytic cell defects

      • Absolute neutrophil count = 6,472

      • Respiratory burst assay : DHR assay  normal

    • Screening test for complement deficiency

      • CH50 =14.6 (19-40 U/ml)


    Pediatric hematology oncology inter hospital conference

    58

    • Flow cytometry

      • %CD3 (Total T lymphocyte)  79% (55-78%)

      • %CD4 (Helper/inducer T lymphocyte)11%

        (21-64 %)

      • %CD8 (Suppressor/cytotoxic T lymphocyte)  57%

        (16-35%)

      • %CD 19 (B cell)  2% (7.7-25.4)

      • %CD 56 (NK cell)  18% (10.6 +/- 5.4)


    Combine t cell and b cell defect

    Combine T-cell and B-cell defect


    Pediatric hematology oncology inter hospital conference

    60

    • Combine T-cell and B-cell defect

      • Primary immune defect

        • Severe combined immunodeficiency (SCID)

        • Ataxia-Telangiectasia syndrome (AT)

        • Wiskott-Aldrich syndrome (WAS)

        • Reticular dysgenesis

      • Secondary immune defect

        • CMV infection


    Pediatric hematology oncology inter hospital conference

    61

    • Suspected combine T-Cell and B-Cell defect

    • Persistent thrombocytopenia

    • Eczema

      Wiskott-Aldrich syndrome (WAS)


    Pediatric hematology oncology inter hospital conference

    63


    Wiskott aldrich syndrome

    Wiskott-Aldrich syndrome

    64

    • 1st described by Wiskott in 1937 and was further characterized by Aldrich in 1954.

    • 1 in 250,000 live male births

    • X-linked recessive immunodeficiency disorder characterized by the triad of

      • recurrent bacterial sinopulmonary infections

      • eczema (atopic like dermatitis)

      • bleeding diathesis caused by thrombocytopenia and platelet dysfunction.

    Pediatr. Dec 1994;125(6 Pt 1):876-85


    Wiskott aldrich syndrome1

    Wiskott-Aldrich syndrome

    65

    • only 30% of patients with the syndrome have the classic triad

      • 90% of patients have manifestations of thrombocytopenia at presentation

      • 20% have only hematologic abnormalities

      • 5% have only infectious manifestations,

    Mol There. May 2008;16(5):836-44.


    Wiskott aldrich syndrome2

    Wiskott-Aldrich syndrome

    66

    • Wiskott-Aldrich syndrome gene

      • localized to Xp11.22-23

      • consists of 12 exons

      • encode a 502 amino acid (53 kD) protein Wiskott–Aldrich syndrome protein (WASP)

    • WASP

      • signal transduction and regulates actin filament assembly

      • explains the abnormalities in platelet and lymphocyte cytoskeleton and signaling.

    J Leukoc Biol. Jun 2005;77(6):993-8.


    Clinical manifestations

    Clinical manifestations

    67

    • The first clinical signs are petechiae and ecchymosis of the skin and oral mucosa and bloody diarrhea.

    • One series of 154 patients found

      • petechiae or purpura in 78%

      • serious GI bleeding (hematemesis or melena) in 28%

      • epistaxis in 16%

      • intracranial bleeding in 2% of patients.

    J Pediatr. Dec 1994;125(6 Pt 1):876-85.


    Hematologic findings in was

    Hematologic Findings in WAS

    68

    • Thrombocytopenia (platelet count 10,000–100,000/mm3);

    • Microthrombocytes; low mean platelet volume (MPV).(This is not obvious in the newborn and the MPV is unreliable when the platelet count is low.)

    • Platelets have abnormal aggregation in response to agonists such as ADP, epinephrine and collagen.

    • Platelets have reduced platelet survival to half normal.

    • Ineffective megakaryocytopoiesis reflected by a platelet turnover 25% that of normal megakaryocyte mass.


    Hematologic findings in was1

    Hematologic Findings in WAS

    69

    • Anemia (due to blood loss).

    • Leukocytosis (due to infection).

    • Normal or increased megakaryocytes.


    Pediatric hematology oncology inter hospital conference

    70

    Normal platelets

    Small platelets


    Other manifestations

    Other Manifestations

    71

    • Autoimmune manifestations

      • Hemolytic anemia

      • Vasculitis

      • Inflammatory bowel disease

      • Glomerulonephritis

      • Arthritis

    • Malignancies: adolescents, young adults

      • The most common malignancy is non-Hodgkin lymphoma.


    Diagnostic tests immunologic

    Diagnostic tests; immunologic

    72

    • Decrease CD3, CD4, CD8 T-cell

    • Normal B-cell and NK cell

    • Markedly decreased/ Absent isohemagglutinins (PHA)

    • Decreased IgM

    • Normal/ slightly low IgG

    • Normal/ Elevated IgA, IgE

    • Defective production of pneumococcal Ab

    • Depressed lymphocyte response to mitogens


    Treatment1

    Treatment

    73

    • Allogeneic SCT is treatment of choice when matched donor available.

    • If no matched donor is available the patient should be managed as follows:

      • Aggressive treatment of infections

      • Platelet transfusions for hemorrhagic episodes

      • Steroid cream for eczema

      • Splenectomy, reserved only for severe case with usual pre- and post-splenectomy precautions pertain

      • Thrombopoietic agents are being explored.


    Thank you for your attention

    Thank you for your attention

    74


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