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Extern ConferenceExtern Conference
SupervirsorDoctor Bunchoo Pongtanakul
Doctor Nithiwat Vatanavicharn
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An 11-year-old girlAn 11-year-old girl
Chief complaintNeck mass at Lt side 6 months PTA
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Present HistoryPresent History
6 mo PTAThe patient’s mother noticed that the patient’s left neck was enlarged. Later her mother decided to take her to the hospital. The clinician told that she had enlarged lymph node and prescribed her oral antibiotics for 2 weeks.
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Present HistoryPresent History
2 weeks laterHer clinical symptoms did not improve and the lymph node biopsy was done. But the pathological report suggested an inadequate tissue. So the clinician decided to continue oral antibiotics.
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Present HistoryPresent History
2 mo PTAHer lymph node was progressively enlarged, and her mother noticed that the right side was enlarged too. At the hospital, the physical examination was performed and reviewed that preauricular, submandibular and anterior cervical node enlargement both sides.
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Present HistoryPresent History
2 mo PTAThe lymph node biopsy was done again, and the pathological report suggested malignant lymphoma. The clinician referred the patient to Siriraj Hospital for further management.
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Present HistoryPresent History
1 mo PTAShe complained about bloating, loss of appetite and weight loss1 kg in 1 mo.
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Present HistoryPresent History
She had no history of fever, night sweating, bleeding, epistaxis, pale, dyspnea or chronic cough.No history of dysphagia, oral ulcer, oral thrush or hearing loss before.No palpable mass at the other sites.No history of contact TB.
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Past HistoryPast History
No significant medical history.No previous surgery.No history of head and neck trauma.
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Family HistoryFamily History
No history of malignancy in the family.
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Allergic HistoryAllergic History
No history of drugs, food or chemical allergy before.
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Physical ExaminationPhysical Examination
V/S : T 37.2 ºc, RR 14/min, PR 84/min, BP 104/65 mmHgGA : Thai 11-year-old girl, alert and active, sthenic built, not pale, no jaundice, no edema, no dyspnea, no tachypneaSkin : no rash, no petechiae, no ecchymosis
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Physical ExaminationPhysical Examination
HEENT : Head : normocephalic, atraumaticEye : WNLEar : WNLNose : normal mucosa, no visible massThroat : pharynx and tonsils not injected
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Physical ExaminationPhysical Examination
RS : normal breath sounds, no adventitious soundsCVS : normal S1 and S2, no murmurAbdomen : mild distend, soft, no tenderness, liver just palpable, liver span 7 cm, spleen 3 FB below LCM, active bowel sounds
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Physical ExaminationPhysical Examination
GU : WNLNS : E4V5M6, pupil 3 mm BRTL, full EOM, no visual field defect, no facial palsy, gag reflex +ve, Rinne’s BC>AC both, Weber’s no lateralization, motor power grade V all, sensory intact, stiff neck and Kernig’s sign -ve
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Physical ExaminationPhysical Examination
Lymph Node : Multiple cervical lymphadenopathy vary in size 0.5-2 cm in diameterLt epitrocheal node 1.5 cm in diameter Both inguinal node 0.5-1.5 cm in diameterNo tenderness, rubbery in consistency, smooth surface, movable, no signs of inflammation
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Initial InvestigationInitial Investigation
CBCPeripheral blood smearCXR
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CBC18/1/200
8
CBC18/1/200
8
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CBC30/1/200
8
CBC30/1/200
8
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Peripheral Blood Smear 30/1/08 Peripheral Blood Smear 30/1/08
Normochromic normocytic RBCPlatelet about 15-20 cell/OF. No platelet aggregation.WBC : L 60%, N 39%, M 1%. No blast.
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Electrolyte
18/1/2008
Electrolyte
18/1/2008
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CXR 18/1/08CXR 18/1/08
Intact bony structure Normal soft tissueMinimal widening of upper mediastinumNo pulmonary infiltration.Cardio-thoracic ratio 0.46
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Problem ListProblem List
Generalized lymphadenopathy at cervical, epitrocheal and inguinal region for 6 moBloating, loss of appetite and weight loss for 1 moSplenomegalyPancytopenia with lymphocytosis
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LymphadenopathyLymphadenopathy
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LymphadenopathyLymphadenopathy
The body has 600 lymph nodes Only in the submandibular, axillary or inguinal regions may normally be palpable Lymphadenopathy refers to nodes that are abnormal in either size, consistency or number
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LymphadenopathyLymphadenopathy
SizeLymphoid mass increases steadily after birth until age 8-12 yrs and undergoes progressive atrophy during puberty Newborns usually have small adenopathy (<0.5 cm)In young children :
• Anterior cervical nodes as large as 1.5 cm • Axillary nodes as large as 1 cm• Inguinal nodes as large as 1.5 cm
Should be considered abnormal if the epitrochlear or supraclavicular nodes larger than 0.5 cm.
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LocalizedIf only one area is involved
GeneralizedIf lymph nodes are enlarged in two or more noncontiguous areas
LymphadenopathyLymphadenopathy
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Axillary
Epitrochlear
Inguinal
Cervical
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Approach to Generalized LymphadenopathyApproach to Generalized Lymphadenopathy
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Generalized LymphadenopathyGeneralized Lymphadenopathy
239 children underwent peripheral lymph node biopsies for evaluation of lymphadenopathy. The etiology were noted
Reactive hyperplasia 52%Granulomatous disease 32%Neoplastic disease 13%Chronic dermatopathic or bacterial infection 3%
From Knight PJ ; Mulne AF ; Vassy LE : Pediatrics 1982 Apr ; 69(4) : 391-6
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Historical Clues Historical Clues
Age and durationThe vast majority of cases of lymphadenopathy in children is infectious or benign in etiology.Lymphadenopathy that lasts ≤ 2 weeks or ≥ 1 year with no progressively increasing in size has a very low likelihood of being neoplasm.
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Historical Clues Historical Clues
ExposureExposure to animalsTravel-related exposures and immunization statusEnvironmental exposures such as tobacco, alcoholUltraviolet radiationPatients with AIDS
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Historical Clues Historical Clues
ASSOCIATED SYMPTOMSConstitutional symptoms such as fatigue, malaise, and fever, significant fever, night sweats and unexplained weight lossSymptoms such as arthralgias, muscle weakness, or unusual rash may indicate the possibility of autoimmune diseases
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Generalized LymphadenopathyGeneralized Lymphadenopathy
InfectionInfection MalignancyMalignancy OtherOther
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Generalized Lymphadenopathy
Infection
• Infectious Mononucleosis • HIV• CMV • Varicella• Adenovirus • Roseola Infantum
• Salmonella typhi• Syphilis• Plague• Tuberculosis
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Generalized lymphadenopathy
Malignancy
• ALL• AML• Lymphoma• Langerhans cell histiocytosis • EBV associated lymphoproliferative disease
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Generalized lymphadenopathy
Other
• Drugs• Autoimmune disease eg. JRA , SLE
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Diffential DiagnosisDiffential Diagnosis
Hematologic malignancyLymphomaAcute leukemia
Chronic infectionTuberculosisHIV infection
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Hematologic MalignancyHematologic Malignancy
ProGeneralized lymphadenopathySplenomegalyNo response to ATBAbnormal CBC : pancytopenia with lymphocytosis
ConsNo sign of BM failure (Acute leukemia)
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Chronic InfectionChronic Infection
ProGeneralized lymphadenopathyNo response to ATB
ConsNo chronic coughNo Hx of contact TB
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InvestigationInvestigation
Lymph node biopsy
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Lymph Node BiopsyLymph Node Biopsy
Left cervical lymph node biopsy:Precursor T lymphoblastic lymphoma A complete hematologic work-up is highly recommended to exclude acute lymphoblastic leukemia of T-cell phenotype (T-ALL).
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BM AspirationBM Aspiration
BM AspirationDiluted BM, mild hypocellularity, normal megakaryocyte, decreased erythroid and myeloid series, increased lympoid series, lymphoblast 25-30%
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Final DiagnosisFinal Diagnosis
Acute Lymphoblastic Leukemia( T Cell )
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IntroductionIntroduction
Acute leukemia is the most common cancer in childrenALL > AML ~ 5Peak incidence 2-5 yrs
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Signs and SymptomsSigns and Symptoms
Musculoskeletal : bone painLymphadenopathy ~50%Headache ~5%Testicular enlargementMediastinal massPeripheral blood abnormalities
AnemiaThombocytopeniaLymphoblast on peripheral blood
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DiagnosisDiagnosis
The diagnosis and classification of leukemia are based upon specialized tests that are performed on cells derived from a bone marrow aspiration or tissue biopsy specimens ALL is the preferred term when the bone marrow contains > 25 % lymphoblasts, whereas lymphoma is the preferred term when the process is confined to a mass lesion with minimal or no blood and marrow involvement
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Risk assignment and suggested therapiesRisk assignment and suggested therapies
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Risk Group
Features % Recommended Therapy
Lesser Hyperdiploid or trisomies 4, 10, 17 20 Conventional anti-metabolite-based therapyt(12,21) 20
Standard WBC <50,000/microL 15 Intensified antimetabolite therapy
Age 1 to 9.9 years
High T-cell phenotype 15 Intensive multi-agent therapy
Age >10 years 15
WBC>50,000/microL, t(1;19) 6
Very high t(9;22) 3 Consider allogeneic hematopoietic cell transplantation in first remission
t(4;11); age <1 year 4
Induction failures and slow responders
2
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Adverse EffectsAdverse Effects
Tumor lysis syndromeThrombosisBleedingInfection
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Tumour Lysis SyndromeTumour Lysis Syndrome
The term applied a group of metabolic complication that usually occur after the treatment of neoplastic disorderEg. ALL, Burkitt’s lymphoma, T cell leukemia lymphoma
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FindingsFindings
HyperphosphatemiaHypocalcemiaHyperuricemiaHyperkalemia
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ManagementManagement
Prevention of tumour lysis syndromeAdequate hydration : at least 2 times of MT, adjust q 2-3 hr, keep urine sp.gr. < 1.010Urine alkalinization : add NaHCO3 30-100 mEq/L , keep urine pH 6.5-7.5Allopurinol 10 mg/kg/day q 8 hr
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Management (2)Management (2)
Treatment : correct metabolic disturbanceHyperkalemia : NaHCO3 , insulin, glucose, 10% calcium gluconate, KayexalateHyperphosphatemia : Ca X PO4 > 60 , Give Aluminium hydroxide 150 mg/kg/dayHypercalcemia : 10% calcium gluconate 0.5-1 ml/kg or calcium chloride 10 mg/kg, EKG monitoringHemodialysis