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SupervirsorDoctor Bunchoo Pongtanakul

Doctor Nithiwat Vatanavicharn

An 11-year-old girlAn 11-year-old girl

Chief complaintNeck mass at Lt side 6 months PTA

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.

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.

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.

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.

Present HistoryPresent History

1 mo PTAShe complained about bloating, loss of appetite and weight loss1 kg in 1 mo.

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.

Past HistoryPast History

No significant medical history.No previous surgery.No history of head and neck trauma.

Family HistoryFamily History

No history of malignancy in the family.

Allergic HistoryAllergic History

No history of drugs, food or chemical allergy before.

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

Physical ExaminationPhysical Examination

HEENT : Head : normocephalic, atraumaticEye : WNLEar : WNLNose : normal mucosa, no visible massThroat : pharynx and tonsils not injected

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

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

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

Initial InvestigationInitial Investigation

CBCPeripheral blood smearCXR

CBC18/1/200

8

CBC18/1/200

8

CBC30/1/200

8

CBC30/1/200

8

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.

Electrolyte

18/1/2008

Electrolyte

18/1/2008

CXR 18/1/08CXR 18/1/08

Intact bony structure Normal soft tissueMinimal widening of upper mediastinumNo pulmonary infiltration.Cardio-thoracic ratio 0.46

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

LymphadenopathyLymphadenopathy

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

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.

LocalizedIf only one area is involved

GeneralizedIf lymph nodes are enlarged in two or more noncontiguous areas

LymphadenopathyLymphadenopathy

Axillary

Epitrochlear

Inguinal

Cervical

Approach to Generalized LymphadenopathyApproach to Generalized Lymphadenopathy

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

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.

Historical Clues Historical Clues

ExposureExposure to animalsTravel-related exposures and immunization statusEnvironmental exposures such as tobacco, alcoholUltraviolet radiationPatients with AIDS

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

Generalized LymphadenopathyGeneralized Lymphadenopathy

InfectionInfection MalignancyMalignancy OtherOther

Generalized Lymphadenopathy

Infection

• Infectious Mononucleosis • HIV• CMV • Varicella• Adenovirus • Roseola Infantum

• Salmonella typhi• Syphilis• Plague• Tuberculosis

Generalized lymphadenopathy

Malignancy

• ALL• AML• Lymphoma• Langerhans cell histiocytosis • EBV associated lymphoproliferative disease

Generalized lymphadenopathy

Other

• Drugs• Autoimmune disease eg. JRA , SLE

Diffential DiagnosisDiffential Diagnosis

Hematologic malignancyLymphomaAcute leukemia

Chronic infectionTuberculosisHIV infection

Hematologic MalignancyHematologic Malignancy

ProGeneralized lymphadenopathySplenomegalyNo response to ATBAbnormal CBC : pancytopenia with lymphocytosis

ConsNo sign of BM failure (Acute leukemia)

Chronic InfectionChronic Infection

ProGeneralized lymphadenopathyNo response to ATB

ConsNo chronic coughNo Hx of contact TB

InvestigationInvestigation

Lymph node biopsy

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).

BM AspirationBM Aspiration

BM AspirationDiluted BM, mild hypocellularity, normal megakaryocyte, decreased erythroid and myeloid series, increased lympoid series, lymphoblast 25-30%

Final DiagnosisFinal Diagnosis

Acute Lymphoblastic Leukemia( T Cell )

IntroductionIntroduction

Acute leukemia is the most common cancer in childrenALL > AML ~ 5Peak incidence 2-5 yrs

Signs and SymptomsSigns and Symptoms

Musculoskeletal : bone painLymphadenopathy ~50%Headache ~5%Testicular enlargementMediastinal massPeripheral blood abnormalities

AnemiaThombocytopeniaLymphoblast on peripheral blood

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

Risk assignment and suggested therapiesRisk assignment and suggested therapies

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

Adverse EffectsAdverse Effects

Tumor lysis syndromeThrombosisBleedingInfection

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

FindingsFindings

HyperphosphatemiaHypocalcemiaHyperuricemiaHyperkalemia

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

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