Bone biopsy: left proximal tibia Primary non

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เด็กชายไทย อายุ 12 ปี 3 เดือน
 อาการสาคัญ
 ประวัตป
ิ ั จจุบัน
ปวดหลังมา 2 เดือน
2 เดือน ปวดหลังเป็ นๆหายๆ ไม่ สัมพันธ์ กับท่ าทาง ปวด
มากกลางคืน มีไข้ เป็ นๆหายๆ
1 เดือน มีไข้ สูงขึน้ ไปรพ.จังหวัด ได้ นอนโรงพยาบาล
วินิจฉัยว่ าเป็ นกรวยไตอักเสบ รักษาโดยการฉีดยา อาการไม่
ดีขนึ ้ ยังมีไข้ ปั สสาวะบ่ อย หิวนา้ บ่ อย อ่ อนเพลีย นา้ หนัก
ลดลง 5 กิโลกรัม
ประวัตปิ ั จจุบัน
 ระหว่ าง admit เริ่มอาการปวดบัน้ เอวมากขึน้ เดินได้ ปกติ ไม่ มี
อาการทางระบบประสาท ได้ film T-spine พบมี
collapse T -12 และตรวจเพิ่มเติม
ประวัตปิ ั จจุบัน
ตรวจเพิ่มเติมที่โรงพยาบาลจังหวัด
 Flim TL spine: compression fracture T12
 PPD skin test: negative
 CT chest: normal
 CT Whole abdomen: hepatosplenomegaly
ประวัตปิ ั จจุบัน
 MRI spine: multiple ring enhancement at
multiple vertebral lesion with pathological
fracture T12
 Bone scan: increased uptake lesions at upper
thoracic spine and all joint
 Bone marrow aspiration: no abnormal cell
 Bone marrow biopsy: reactive hyperplasia
ประวัตปิ ั จจุบัน
 MRI spine: multiple ring enhancement at
multiple vertebral lesion with pathological
fracture T12
 Bone scan: increased uptake lesions at upper
thoracic spine and all joint
 Bone marrow aspiration: no abnormal cell
 Bone marrow biopsy: reactive hyperplasia
History
ประวัติอดีต
ไม่มีโรคประจำตัว ออกกำลังกำยเป็ นประจำ
เรียน ม.1 กำรเรียนดี
ประวัติครอบครัว
ปฏิเสธโรควัณโรค มะเร็ง โรคเลือด ในครอบครัว
Physical Examination
A Thai boy, mild pallor, no jaundice
T 39oc PR112/min RR 20/min BP 120/70 mmHg
• Wt 45 kg Ht 150 cm
• Heart and lungs: normal
• Abdomen: liver 4 cm below RCM mild
tender, spleen 2 cm below LCM
Physical Examination
• Back: no scoliosis, tender along distal
thoracic to lumbar spine
• Extremities: no deformity, no limitation
of movement
• Neuro:



motor power upper extremities V
lower extremities IV
DTR 2+ all
Problem lists and Discussion
Initial investigations
CBC:
Hb 9.6 g%, Hct 30%, MCV 71.7 fl, MCH 23.1 pg,
MCHC 32.2 g/dL
WCB 10,400/mm3, N 73%, L 16%, M 11%
Plt 435,000/mm3
UA: normal
Anti HIV: negative
Initial investigations (2)
Electrolytes: Na 134, K 3.9, Cl 94, CO2 26.5
mmol/L
Ca 12.7, P 3.8, Mg 1.6 mg/dL
BUN 20, Cr 0.9 mg/dL
LDH: 670 U/L
CRP: 265 mg/L
Initial investigations (3)
Liver function test
AST 55, ALT 27 U/L,
TB 0.6, DB 0.3 mg/dL,
Total protein 78 g/L, albumin 31.5 g/L,
Alkaline phosphatase 212 U/L GGT 85 U/L
Investigation (4)
 Melioid titer: negative
 Hemo c/s: no growth
 PPD skin test: no induration
Investigations
 Left sided L1 transpedicular biopsy:
Necrotic material with some inflammation
cells, no acid fast bacilli, no fungus
Investigations
 BMA: normal cellularity, normal maturation
of erythroid, myeloid and megakarycyte, no
abnormal cell
 BM Biopsy:
 normal cellularity 80%, M:E ratio 3:1
 Active trilinear hematopoiesis
 Blast 5% of nucleated cells
 No fibrosis, no granuloma
MRI knee
 Extensive varying-size
Nodular appearing
marrow infiltrating
lesions
 Metastatics deposit
 Malignancy process
 Infectious process
Pathology
BCL-2
CD 20
CD3
CD10
Investigations
 Bone biopsy: left proximal tibia
 Primary non-Hodgkin lymphoma of bone, diffuse B
cell lymphoma
 Immunohistochemistry










CD20
CD79a
CD3
CD10
CD68
CD99
BCL-2
TdT
Desmin
Sarcomeric actin
positive in large lymphoid cells
positive in large lymphoid cells
negative in large lymphoid cells
negative in large lymphoid cells
positive in histiocytic cells
negative
negative
negative
negative
negative
Chest x-ray
Film spine
Destruction and collapse T12, L4-5 vertebrae
Bone survey
multiple osteolytic lesions
CT chest and abdomen
 mediastinum and intraabdominal
lympadenopathy
 mild hepatosplenomegaly
 Infiltrative involvement both
kidney, no hydronephrosis
 Multiple osteolytic lesions
including clavicles, scapulars,
head of humerus, whole spines,
pelvic bone and femur
Diagnosis
 Primary non-Hodgkin lymphoma of
bone, diffuse B cell lymphoma
Treatment
 Start chemotherapy Burkitt protocol
 Supportive care
After Treatment
Primary Bone Lymphoma
 Primary bone lymphomas (PBLs) are rare, less than
1% of all malignant lymphomas
 PBLs is defined as a lymphoma that is confined to
bone or BM without evidence of systemic
involvement
 2002 WHO classification of tumors of soft tissue
and bone, the criteria for a diagnosis of PBL
 a single skeletal tumor without regional LN
involvement
 multiple bone lesions without visceral or LN
involvement
Arch Pathol Lab Med Vol 133, November 2009
Primary Bone Lymphoma
 Most are diffuse large B-cell lymphomas
 The middle-aged to elderly population, median age
of 48 yrs
 Common presentation: bone pain and less-frequent
a palpable mass and bone fracture
 Very rarely, paraplegia from compression
 Rarely, hypercalcemia may be present
Arch Pathol Lab Med Vol 133, November 2009
Anatomic Location
 Beal et al reported a series of PBDLBCL that
included 82 patients
 Involvement was femur (27%), pelvis (15%),
tibia/fibula (13%), polyostotic (13%), humerus
(12%), spine (9%), other (5%), mandible (2%),
radius/ulna (1%), scapula (1%), and skull (1%)
 Rarely, small bones of the hands and feet are
involved in PBDLBCL
Arch Pathol Lab Med Vol 133, November 2009
Radiographic Findings
 The metaphysis is the most common site of
occurrence in long bones
 The lesion shows varying areas of sclerosis and
osteolysis, producing a ‘‘moth-eaten’’
appearance
Arch Pathol Lab Med Vol 133, November 2009
Case Reports
1. A 13-year-old girl presented with a 6
month history of pain in the lower
thoracic region


Examination and investigations: mass at
right thoracic and pelvic region, osteolytic
lesion
Biopsy: lymphoblastic lymphoma, CD20,
CD79 positive
2. A 6-year-old boy, progressive pain left
knee and rapidly, enlarging mass.


Exam and Ix: mass at left knee 5*5 cms,
osteolytic lesion at metaphyseal of distal
femur
Biopsy: burkitt lymphoma, CD19, CD20
positive
Eur J Pediatr 2001,160:239-242
Case Reports (con’t)
 Treatment with B-cell protocol NHL
consecutive blocks of polychemotherapy
 Vincristine, cytarabine, dexamethasone,
doxorubicine, etoposide, cyclophosphamide and
high dose methotrexate with leucovorin rescue
 Complete remission both cases
 Follow up of 24, 18 months respectively,
alive without disease
Eur J Pediatr 2001,160:239-242
Case Reports (con’t)
 PLB, defined localized disease and treat with
local radiation of primary site
 Treatment of adult PLB of RT had 50% overall
long term survival
 Lymphoma in children as systemic disease,
local RT not sufficient
 Pediatric oncology group treatment with multiagent chemotherapy without RT 95% of a 5 year
event-year free rate
Eur J Pediatr 2001,160:239-242
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