Extern Conference

1. Extern Conference Supervirsor Doctor Bunchoo  Pongtanakul Doctor Nithiwat  Vatanavicharn

2. An 11-year-old girl • Chief complaint • Neck mass at Lt side 6 months PTA

3. Present History • 6 mo PTA • The 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.

4. Present History • 2 weeks later • Her 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.

5. Present History • 2 mo PTA • Her 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.

6. Present History • 2 mo PTA • The lymph node biopsy was done again, and the pathological report suggested malignant lymphoma. • The clinician referred the patient to Siriraj Hospital for further management.

7. Present History • 1 mo PTA • She complained about bloating, loss of appetite and weight loss1 kg in 1 mo.

8. Present 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.

9. Past History • No significant medical history. • No previous surgery. • No history of head and neck trauma.

10. Family History • No history of malignancy in the family.

11. Allergic History • No history of drugs, food or chemical allergy before.

12. Physical Examination • V/S : T 37.2 ºc, RR 14/min, PR 84/min, BP 104/65 mmHg • GA : Thai 11-year-old girl, alert and active, sthenic built, not pale, no jaundice, no edema, no dyspnea, no tachypnea • Skin : no rash, no petechiae, no ecchymosis

13. Physical Examination • HEENT : • Head : normocephalic, atraumatic • Eye : WNL • Ear : WNL • Nose : normal mucosa, no visible mass • Throat : pharynx and tonsils not injected

14. Physical Examination • RS : normal breath sounds, no adventitious sounds • CVS : normal S1 and S2, no murmur • Abdomen : mild distend, soft, no tenderness, liver just palpable, liver span 7 cm, spleen 3 FB below LCM, active bowel sounds

15. Physical Examination • GU : WNL • NS : 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

16. Physical Examination • Lymph Node : • Multiple cervical lymphadenopathy vary in size 0.5-2 cm in diameter • Lt epitrocheal node 1.5 cmin diameter • Both inguinal node 0.5-1.5 cm in diameter • No tenderness, rubbery in consistency, smooth surface, movable, no signs of inflammation

17. Initial Investigation • CBC • Peripheral blood smear • CXR

18. CBC18/1/2008

19. CBC30/1/2008

20. Peripheral Blood Smear 30/1/08 • Normochromic normocytic RBC • Platelet about15-20 cell/OF. No platelet aggregation. • WBC : L 60%, N 39%, M 1%. No blast.

21. Electrolyte18/1/2008

22. CXR 18/1/08 • Intact bony structure • Normal soft tissue • Minimal widening of upper mediastinum • No pulmonary infiltration. • Cardio-thoracic ratio 0.46

23. Problem List • Generalized lymphadenopathy at cervical, epitrocheal and inguinal region for 6 mo • Bloating, loss of appetite and weight loss for 1 mo • Splenomegaly • Pancytopenia with lymphocytosis

24. Lymphadenopathy

25. Lymphadenopathy • 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

26. Lymphadenopathy • Size • Lymphoid 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 abnormalif the epitrochlear or supraclavicular nodes larger than 0.5 cm.

27. Lymphadenopathy • Localized • If only one area is involved • Generalized • If lymph nodes are enlarged in two or more noncontiguous areas

29. Axillary Epitrochlear Cervical Inguinal

30. Approach to Generalized Lymphadenopathy

31. Generalized 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

32. Historical Clues • Age and duration • The 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.

33. Historical Clues • Exposure • Exposure to animals • Travel-related exposures and immunization status • Environmental exposures such as tobacco, alcohol • Ultraviolet radiation • Patients with AIDS

34. Historical Clues • ASSOCIATED SYMPTOMS • Constitutional symptoms such as fatigue, malaise, and fever, significant fever, night sweats and unexplained weight loss • Symptoms such as arthralgias, muscle weakness, or unusual rash may indicate the possibility of autoimmune diseases

35. Generalized Lymphadenopathy Infection Malignancy Other

36. Generalized Lymphadenopathy Infection • Infectious Mononucleosis • HIV • CMV • Varicella • Adenovirus • Roseola Infantum • Salmonella typhi • Syphilis • Plague • Tuberculosis

37. Generalized lymphadenopathy Malignancy • ALL • AML • Lymphoma • Langerhans cell histiocytosis • EBV associated lymphoproliferative disease

38. Generalized lymphadenopathy Other • Drugs • Autoimmune disease • eg. JRA , SLE

39. Diffential Diagnosis • Hematologic malignancy Lymphoma Acute leukemia • Chronic infection Tuberculosis HIV infection

40. Pro Generalized lymphadenopathy Splenomegaly No response to ATB Abnormal CBC : pancytopenia with lymphocytosis Cons No sign of BM failure (Acute leukemia) Hematologic Malignancy

41. Pro Generalized lymphadenopathy No response to ATB Cons No chronic cough No Hx of contact TB Chronic Infection

42. Investigation • Lymph node biopsy

43. Lymph 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).

44. BM Aspiration • BM Aspiration • Diluted BM, mild hypocellularity, normal megakaryocyte, decreased erythroid and myeloid series, increased lympoid series, lymphoblast 25-30%

45. Final Diagnosis Acute Lymphoblastic Leukemia ( T Cell )

46. Introduction • Acute leukemia is the most common cancer in children • ALL > AML ~ 5 • Peak incidence 2-5 yrs

47. Signs and Symptoms • Musculoskeletal : bone pain • Lymphadenopathy ~50% • Headache ~5% • Testicular enlargement • Mediastinal mass • Peripheral blood abnormalities • Anemia • Thombocytopenia • Lymphoblast on peripheral blood

48. Diagnosis • 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

49. Risk assignment and suggested therapies