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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
slide5

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
slide6

BMA: inadequate

  • 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
  • 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 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 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 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
  • 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
  • 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
  • Prolong fever
  • Polyarthritis
  • Anemia
  • Hepatomegaly
  • SEM gr II
  • S/P splenectomy
  • Homozygous HbE
slide14
DDx
  • Infection: TB, virus (EBV, CMV), Bacteria, fungus
  • Connective tissue disease: JRA, SLE
  • Malignancy: acute leukemia, lymphoma
  • IAHS
  • LCH
investigation1
Investigation
  • 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
  • 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
slide26

Ultrasound whole abdomen

:diffuse hepatomegaly

slide27

BMA: inadequate, rare nucleated cell, no blast

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

slide30

Splenichemagiomatosis

(1st admission)

slide31

Splenichemagiomatosis

(1st admission)

slide32

Bone marrow biopsy:

Bone marrow necrosis

No malignancy cell

slide33

Bone biopsy at Lt humerous:

Bone marrow necrosis

No malignancy cell

bone marrow necrosis bmn
Bone Marrow Necrosis (BMN)
  • 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
slide35

Hypoxemia after failure of microcirculation:

    • 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%

Extensive search for neoplastic disease is justified whenever BMN is diagnosed

slide37

1st 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
  • 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
  • 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)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
  • 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
  • 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)
slide43

Infection

  • 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
  • Bone pain 75%
  • Fever 68.5%
  • Anemia 91%
  • Thrombocytopenia 78%
  • Leukoerythroblastic picture 51%
  • Elevated LDH, alkaline phosphatase 50%
slide45

Leukoerythroblastic picture

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

slide46

Cytology

  • 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
slide47

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

slide48

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

slide49

Bone Marrow Scanning

  • 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

slide50

Life-threatening complication:

embolization of fat and necrotic bone marrow

to pulmonary arteries

prognosis
Prognosis
  • 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
  • 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
slide56

Developed massive GI bleeding

treatment: LPB, Platelet, FFP, antibiotic

Cardiac arrest: CPR x 30 min

slide59

BM biopsy from

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+
slide61

Liver biopsy

: precursor T lymphoblastic leukemia/ lymphoblastic lymphoma

  • CD3+, CD20-, CD79a-, CD34-, MPO-, TdT+
thank you
Thank You

Extensive search for neoplastic disease

Multiple aspirates from different sites

: biopsy site in which viable hematopoietic bone marrow

ad