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Interhospital Conference 17 November 2009. 10 years old boy Prolong fever, arthritis. Patient data. ผู้ป่วยเด็กชายไทย อายุ 10 ปี ที่อยู่ อำเภอเมือง จังหวัดกาฬสินธุ์ Chief complaint ไข้สูง ปวดข้อ 1 สัปดาห์ ก่อนมาโรงพยาบาล. Present illness.

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Interhospital conference 17 november 2009

Interhospital Conference17 November 2009

10 years old boy

Prolong fever, arthritis


Patient data
Patient data

ผู้ป่วยเด็กชายไทย อายุ 10 ปี

ที่อยู่ อำเภอเมือง จังหวัดกาฬสินธุ์

Chief complaint

  • ไข้สูง ปวดข้อ 1 สัปดาห์ ก่อนมาโรงพยาบาล


Present illness
Present illness

  • 1 เดือนก่อน มาด้วยไข้ 7 วัน ปวดข้อ ข้อบวม

  • SEM at LUPSB, liver 4cm below RCM

  • Lt wrist, Lt knee and ankle then Rt ankle arthritis (migratory polyarthritis)


Investigation 1
Investigation 1 เดือนก่อน

  • CBC: Hct 28.6%, Hb 9 g/dl, corrected WBC 9,600, N 37%, L 43%, M 18%, NRC 153/100 WBC, plt 117,000

    MCV 72.9, MCH 22.9, MCHC 31.4, RDW 20.8

  • ESR 82 mm/hr, ANA-, ASO positive 1:1, RF -, CRP + 1:64,

  • Melioid titer 1:40, H/C no growth, PPD -

  • CXR: mild cardiomegaly

  • EKG: NSR, HR 120/min, QRS axis 750, PR 0.12 sec, QTc 0.38 sec, borderline LVH

  • Echocardiogram: EF 76.8%, mild LV enlargement, trivial MR


  • Dx: acute rheumatic fever: 1 major (poly arthritis), 2 minor ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Rx: cefotaxime x 8 d, amoxy+clavulanic acid x 6d,

    ASA gr V (90mkd)

    Severe joint pain, pain score= 10 : suspected malignancy/ JRA

  • Brufen, tramol, MST, morphine syrup prn

  • Prednisolone 2 MKD x 2 wk

  • LDH 2,476 U/L uric acid 6.3 mg/dl, ESR 135 mm/hr, CRP 1:16,

  • Coomb’s test –, ANA-, RF-, UA normal

  • eye exam: no uveitis


  • BMA: inadequate ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • BM biopsy: fragment of cartilage, no bone marrow tissue seen

  • Film bone survey: multiloculated cystic lesion at distal of Lt femur, no osteolytic lesion, suspected benign condition,

    No malignancy condition

  • Bone scan: ?

  • Ultrasound abdomen: hepatomegaly


Present illness1
Present illness ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • 1 สัปดาห์ก่อนมาโรงพยาบาล มีไข้สูง ส่วนใหญ่เป็นไข้กลางคืน ปวดเมื่อยต้นขา 2 ข้าง ปวดข้อศอก ทั้ง 2 ข้าง ข้อมือ ข้อนิ้วมือ ข้อเข่าขวา ไม่ไอ ไม่หอบ ปวดศีรษะเล็กน้อย ปัสสาวะอุจจาระปกติไม่มีน้ำหนักลด รับประทานอาหารได้

  • 2 วัน ก่อนมา ไข้ตลอดวัน ปวดข้อมากขึ้น ปวดสะโพก 2 ข้าง เดินลำบาก


Past history
Past ( fever, ESR, CRP), + evidence Strep infection (ASO+)history

  • มีเลือดกำเดาไหลเป็นๆหายๆ (ตั้งแต่อายุ 3-4 ปี ปีละ 3-4 ครั้ง)

  • 7 เดือนก่อน มาด้วยไข้ 2 เดือน เบื่ออาหาร อ่อนเพลีย ท้องโตขึ้น ซีดลง น้ำหนักลดลง 2 กิโลกรัม ใน 1 เดือน

  • ตรวจร่างกาย พบซีด ม้ามโต 10 cm below LCM,

  • CBC: pancytopenia

  • BMA: erythroid hyperplasia, no blast

  • BM biopsy: bony tissue with paucity number of

    hematopoietic cells without evidence of leukemia

  • Lymph node biopsy at inguinal: reactive lymphoid hyperplasia


Past history1
Past ( fever, ESR, CRP), + evidence Strep infection (ASO+)history

  • CT abdomen: diffuse splenomegaly

  • Splenectomy:

    diffuse sinusoidal hemangiomatosis with extramedullary hemopoeisis

  • EBV Ig M +, IgG+, CMV IgM-, IgG+

  • Antibiotic 17 days

  • Hb typing: Homozygous Hb E


Past history2
Past ( fever, ESR, CRP), + evidence Strep infection (ASO+)history

  • จากนั้นมา follow up ทุก 1 เดือน มีไข้บางครั้ง เลือดกำเดาไหลเล็กน้อย admit 1 ครั้ง เมื่อ 4 เดือนก่อน x 2 วัน

    Hct 29% WBC, platelet ปกติ

  • ได้รับ folic, MTV, zyrtec

Family history

  • มารดาเป็นโรคหอบหืด

  • มีญาติทางบิดาเป็นโรคเลือด (ไม่ทราบว่าเป็นโรคอะไร)

  • ปฏิเสธประวัติมะเร็งในครอบครัว


Physical examination
Physical Examination ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Vital signs:

    BT 39.70 C BP 110/70 mmHg

    PR 112/min RR 22/min

    BW 23 kg (P3-10) Ht 130 cm (P25)

  • GA: A boy, fully consciousness, well co-operate

  • HEENT: mild pale conjunctiva , anicteric sclera

    pharynx and tonsils: not injected

    Rt cervical lymph node 0.5 cm


Physical examination1
Physical examination ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Heart : systolic ejection murmur gr II at LPSB

  • Lungs : normal and equal breath sound, no adventitious sound

  • Abdomen: soft, liver 3 cm below RCM

  • Extremities: no edema

  • Tender and swelling of bilateral elbow, wrist, 4th, 5th MCP, Lt 2nd, 5th MCP, Rt knee joint


Problem lists
Problem lists ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Prolong fever

  • Polyarthritis

  • Anemia

  • Hepatomegaly

  • SEM gr II

  • S/P splenectomy

  • Homozygous HbE


DDx ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Infection: TB, virus (EBV, CMV), Bacteria, fungus

  • Connective tissue disease: JRA, SLE

  • Malignancy: acute leukemia, lymphoma

  • IAHS

  • LCH


Investigation
investigation ( fever, ESR, CRP), + evidence Strep infection (ASO+)


Investigation1
Investigation ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • CBC: Hb 10.2 g/dL, Hct 31%, WBC corrected 4,900

    N 45%, L 36%, M 18%, E 8%,

    NRC 91/100 WBC, Platelet 131,000

    MCV 85.7 fl, MCH 28.2 pg, MCHC 32.9 g/dL

  • BUN 5 mg/dL, Cr 0.4 mg/dL, Uric acid 3.4 mg/dL

  • Na 139, K 3,8, Cl 97, CO2 29 mmol/L, Ca 8.9, Mg 2, P 4 mg/dL

  • Albumin 2.4 g/dL, AST 54 U/L, ALT 37 U/L. ALP 520 U/L

  • Hemoculture; no growth

  • UA : pH 5.5 ,sp.gr. 1.025, wbc 1-2 , rbc 0-1

  • ESR: 75 mm/hr


Investigation2
Investigation ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • LDH 542 U/L

  • Ferritin 3,840 ng/ ml

  • B-HCG 1.11 mIU/ml, AFP 3.08 IU/ml

  • ASO-, CRP-, Melioid 1:20


Bone marrow aspiration ( fever, ESR, CRP), + evidence Strep infection (ASO+)


Multiple ( fever, ESR, CRP), + evidence Strep infection (ASO+)radiolucency lesions


Ultrasound whole abdomen ( fever, ESR, CRP), + evidence Strep infection (ASO+)

:diffuse hepatomegaly


BMA: inadequate, rare nucleated cell, no blast ( fever, ESR, CRP), + evidence Strep infection (ASO+)

Cytochemistry

: numerous cell debris and very rare blood cell have been observed, no hematologic malignancy cell

:Other cause of bone marrow destruction should be ruled out

Flow cytometry: blast gate 2.63% of total event

No evidence of hematologic malignancy


1 ( fever, ESR, CRP), + evidence Strep infection (ASO+)st admission


Histopathology report
Histopathology report ( fever, ESR, CRP), + evidence Strep infection (ASO+)

1st admission


Splenic ( fever, ESR, CRP), + evidence Strep infection (ASO+)hemagiomatosis

(1st admission)


Splenic ( fever, ESR, CRP), + evidence Strep infection (ASO+)hemagiomatosis

(1st admission)


Bone marrow biopsy: ( fever, ESR, CRP), + evidence Strep infection (ASO+)

Bone marrow necrosis

No malignancy cell


Bone biopsy at Lt humerous: ( fever, ESR, CRP), + evidence Strep infection (ASO+)

Bone marrow necrosis

No malignancy cell


Bone marrow necrosis bmn
Bone Marrow Necrosis (BMN) ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Necrosis of myeloid tissue and medullary stroma in the large areas of hematopoietic bone marrow

  • On BM biopsy: disruption of the normal bone marrow architecture with a considerable loss of fat space

  • Aplastic anemia: only loss of myeloid tissue and no destruction of reticular structure

  • Aseptic necrosis: no destruction of the spicular architecture


  • Hypoxemia after failure of microcirculation: ( fever, ESR, CRP), + evidence Strep infection (ASO+)

    • Inflammatory damage or mechanical obstruction

      : DIC, sickle cell disease or tumor cell plug

    • Immune process

    • Toxicity and the release of toxins, cytokines,

      or vasoactive substances from damaged cells

      : TNF

  • Major complication: pancytopenia, embolic processes

  • Repopulation of the bone marrow cavity

    can occur after BMN resolved


Identified underlying malignancy 90
Identified underlying malignancy 90% ( fever, ESR, CRP), + evidence Strep infection (ASO+)

Extensive search for neoplastic disease is justified whenever BMN is diagnosed


  • 1 ( fever, ESR, CRP), + evidence Strep infection (ASO+)st described by Wade and Stevenson, 1942

    : Sickle cell disease, died of cerebral infarction

  • Nies et al. 1965: define BMN in acute leukemia

    34/ 316 (10.75%) postmortem

  • Kiraly and Wheby.1976: 13/664 (2%) in BM specimens during 12 –year period

  • Norgard, 1979. retrospectively reviewed 120/368 (32.5%), only 4 were diagnosed at initial


Grading
Grading ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Small necrotic lesions: <25% of the dimention of the biopsy (39%)

  • Intermediate BMN < 50% (24%)

  • Large BMN >75% (12%)

  • Extensive: throughout the dimension of the specimen

  • Pennaforte et al. 1986: extensive 4/1149 (0.3%)

  • Maisel etal. 1988: severe BMN > 50% of dimension (3%)


Bmn in children
BMN in children ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Macfarlane and Tauro: 4/379 (1%) in ALL

  • Pui et al: 7/1419 (0.5%) in cancer patients


Bone marrow necrosis bmn ann m janssens et al cancer 2000
Bone Marrow Necrosis (BMN) ( fever, ESR, CRP), + evidence Strep infection (ASO+)Ann M. Janssens et al. Cancer 2000

  • Clinicopathologic entity, rare condition

  • Review 240 cases, 1980-1999

  • Inclusion

    1. BMN diagnosed during life

    2. necrosis > 50% of the diameter of

    biopsy specimen


Underlying disease association
Underlying disease association ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Malignancy 91%

  • Hematologic malignancies 60%

  • Acute leukemia 41%, lymphoma 15%

  • ALL 18%, AML 13%

  • Children 18/25 cases were ALL

  • BMN developed before diagnosis: ALL 26/37 (AML 16/24)

  • After induction chemotherapy 1/37

  • At recurrence 10/37


Underlying disease association1
Underlying disease association ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Solid tumors 30%

  • Not found primary origin, even after extensive search 12/43

  • Non malignancy only 9%: sickle cell crisis, infection, drug, HUS, antiphospholipid syndrome, DIC, hyperparathyroidism, anorexia nervosa, idiopathic etc.

  • 16/22 documented infection: underlying malignancy were found later (early - 1 yr)


Infection ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • E.coli

  • Streptococcus

  • Staphylococcus

  • Citrobacter freundii

  • Salmonella

  • Mucormycosis

  • Q fever

  • TB

  • Parvovirus

  • HIV

Drug

  • Sulphasalazine

  • Sulphathiazol

  • Sulphanamide

  • Interferon-alpha

  • G-CSF

  • ATRA

  • Hydroxyurea

  • Fludarabine


Presentation
Presentation ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Bone pain 75%

  • Fever 68.5%

  • Anemia 91%

  • Thrombocytopenia 78%

  • Leukoerythroblastic picture 51%

  • Elevated LDH, alkaline phosphatase 50%


Leukoerythroblastic ( fever, ESR, CRP), + evidence Strep infection (ASO+) picture

primitive red cells (nucleated rbc or erythroblasts) primitive white blood cells (myelocytes)


Cytology ( fever, ESR, CRP), + evidence Strep infection (ASO+)

  • Bone marrow aspiration can be unsuccessful

  • Multiple aspirates from different sites

    (sternal, posterior iliac crest, etc.)

  • Serosanguineous, watery dark red or clear fluid

    Histology

  • Combination of gelatinous transformation and necrosis of the myeloid tissue

  • Background of gelatinous transformation surrounds cellular debris with indistinct cellular margins, pyknotic nuclei, and abnormal eosinophilic staining cytoplasm

  • The hallmark of bone necrosis: loss of osteocytes, osteoblasts, and osteoclasts


background of amorphous extracellular eosinophilic material that surrounds cells that disintegrate.


shows cells that lose their normal staining characteristics. irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis


Bone Marrow Scanning irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

  • Technetium 99m sulfur colloid and indium chloride localize selectively to the reticuloendothelial elements of the marrow

  • Dx BMN, residual BM activity, guided biopsy, recovery

    MRI: changes in bone marrow signal intensities


  • Life-threatening complication: irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

    embolization of fat and necrotic bone marrow

    to pulmonary arteries


Prognosis
Prognosis irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

  • Median survival of the hematologic malignancies :1 - 4 months, 69% died

  • Childhood ALL: 13/18 alive

    Survival: 4 months- 6 years

  • Solid tumor and BMN: widespread metastasis, short survival


Progression of the patient
Progression of the patient irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

  • High grade fever

  • Petechiae hemorrhage at face, periorbital

  • Increased hepatomegaly

  • Blood component therapy, antibiotic, pain management

  • Consult for liver biopsy

  • Repeat BMA and biopsy

  • Plan MRI liver and bone

  • Steroid


Hepatomegaly irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis


Developed massive GI bleeding irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

treatment: LPB, Platelet, FFP, antibiotic

Cardiac arrest: CPR x 30 min


Bone marrow aspiration at sternum irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis


BM biopsy irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysisfrom

1. right ASIS:

Abnormal blast cells infiltrate with focal necrosis and crush artifact

2. A small amount of marrow tissue from Lt ASIS: abnormal blast cells

3. Sternum: numerous blast cell infiltrate

: precursor T lymphoblastic leukemia/ lymphoblastic lymphoma

  • CD3+, CD20-, CD79a-, CD34-, MPO-, TdT+


Liver biopsy irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

: precursor T lymphoblastic leukemia/ lymphoblastic lymphoma

  • CD3+, CD20-, CD79a-, CD34-, MPO-, TdT+


Thank you
Thank You irregular or indistinct margins; the cytoplasm can shrink or vacuolate, and the nucleus shows pyknosis, karyorrhexis, and karyolysis

Extensive search for neoplastic disease

Multiple aspirates from different sites

: biopsy site in which viable hematopoietic bone marrow


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