เด็�กชายไทย อาย� 12 ปี� 3 เด็�อน

อาการสำ�าคั�ญ ปีวด็หลั�งมา 2 เด็�อนปีระว�ติ�ปี�จจ�บั�น 2 เด็�อน ปีวด็หลั�งเปี"นๆหายๆ ไม$สำ�มพั�นธ์'ก�บั

ท$าทาง ปีวด็มากกลัางคั�น ม(ไข้*เปี"นๆหายๆ 1 เด็�อน ม(ไข้*สำ+งข้,-นไปีรพั.จ�งหว�ด็ ได็*นอนโรง

พัยาบัาลั ว�น�จฉั�ยว$าเปี"นกรวยไติอ�กเสำบั ร�กษาโด็ยการฉั(ด็ยา อาการไม$ด็(ข้,-น ย�งม(ไข้* ปี�สำสำาวะบั$อย ห�วน�-าบั$อย อ$อนเพัลั(ย น�-าหน�กลัด็ลัง 5 ก�โลักร�ม

ปีระว�ติ�ปี�จจ�บั�นระหว$าง admit เร�1มอาการปีวด็บั�-นเอวมากข้,-น

เด็�นได็*ปีกติ� ไม$ม(อาการทางระบับัปีระสำาท ได็* film T-spine พับัม( collapse T -12 แลัะติรวจเพั�1มเติ�ม

ปีระว�ติ�ปี�จจ�บั�นติรวจเพั�1มเติ�มท(1โรงพัยาบัาลัจ�งหว�ด็ Flim TL spine: compression fracture T12

PPD skin test: negative

CT chest: normal CT Whole abdomen: hepatosplenomegaly

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

ปีระว�ติ�ปี�จจ�บั�น

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

ปีระว�ติ�ปี�จจ�บั�น

Historyปีระว�ติ�อด็(ติ ไม$ม(โรคัปีระจ�าติ�ว ออกก�าลั�งกายเปี"นปีระจ�า

เร(ยน ม 1. การเร(ยนด็(ปีระว�ติ�คัรอบัคัร�ว ปีฏิ�เสำธ์โรคัว�ณโรคั มะเร�ง โรคัเลั�อด็ ในคัรอบัคัร�ว

Physical Examination A Thai boy, mild pallor, no jaundiceT 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 Vlower extremities IVDTR 2+ all

Problem lists and Discussion

Initial investigationsCBC:

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: negativeHemo c/s: no growthPPD skin test: no induration

Investigations

Left sided L1 transpedicular biopsy:

Necrotic material with some inflammation cells, no acid fast bacilli, no fungus

BMA: normal cellularity, normal maturation of erythroid, myeloid and megakarycyte, no abnormal cell

BM Biopsy: normal cellularity 80%, M:E ratio 3:1Active trilinear hematopoiesisBlast 5% of nucleated cellsNo fibrosis, no granuloma

Investigations

MRI kneeExtensive varying-size

Nodular appearing marrow infiltrating lesionsMetastatics depositMalignancy processInfectious process

Pathology

CD 20 CD3 CD10

BCL-2

Bone biopsy: left proximal tibiaPrimary non-Hodgkin lymphoma of bone, diffuse B

cell lymphomaImmunohistochemistry

CD20 positive in large lymphoid cells CD79a positive in large lymphoid cells CD3 negative in large lymphoid cells CD10 negative in large lymphoid cells CD68 positive in histiocytic cells CD99 negative BCL-2 negative TdT negative Desmin negative Sarcomeric actin negative

Investigations

Chest x-ray

Film spine

Destruction and collapse T12, L4-5 vertebrae

Bone surveymultiple osteolytic lesions

CT chest and abdomenmediastinum and intraabdominal

lympadenopathymild hepatosplenomegalyInfiltrative involvement both

kidney, no hydronephrosisMultiple 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 protocolSupportive care

After Treatment

Primary Bone LymphomaPrimary bone lymphomas (PBLs) are rare, less than

1% of all malignant lymphomasPBLs 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

involvementmultiple bone lesions without visceral or LN

involvement

Arch Pathol Lab Med Vol 133, November 2009

Most are diffuse large B-cell lymphomasThe middle-aged to elderly population, median age

of 48 yrsCommon presentation: bone pain and less-

frequent a palpable mass and bone fractureVery rarely, paraplegia from compressionRarely, hypercalcemia may be present

Primary Bone Lymphoma

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 FindingsThe metaphysis is the most common site of

occurrence in long bonesThe lesion shows varying areas of sclerosis and

osteolysis, producing a ‘‘moth-eaten’’ appearance

Arch Pathol Lab Med Vol 133, November 2009

Case Reports1. 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

Treatment with B-cell protocol NHL consecutive blocks of polychemotherapyVincristine, 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

Case Reports (con’t)

Eur J Pediatr 2001,160:239-242

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 multi-agent chemotherapy without RT 95% of a 5 year event-year free rate

Case Reports (con’t)

Eur J Pediatr 2001,160:239-242