Lymphoma Notes PDF

(L7) LYMPHOMA  LECTURE 07 LYMPHOMA DEFINITION AND TYPES  Malignant disease of lymphatic tissues.  Common malignant disease in childhood (3rd)  2 types: ① Hodgkin’s lymphoma. ② Non Hodgkin lymphoma (more Common in Pediatrics)...

(L7) LYMPHOMA  LECTURE 07 LYMPHOMA DEFINITION AND TYPES  Malignant disease of lymphatic tissues.  Common malignant disease in childhood (3rd)  2 types: ① Hodgkin’s lymphoma. ② Non Hodgkin lymphoma (more Common in Pediatrics) Hodgkin’s Disease Overview  One of malignant lymphomas.  Sex: males > females 3:1.  Age: 5-15Ys.  It is unicentric in origin. etiology  Unknown but there are theories.  e.g Related to Epstein-Barr Virus (EBV) infection which can cause infectious mononucleosis. Prevalence  Some countries have very high prevalence of EBV positivity in HD  e.g. Iran, Greece, Kenya 92-94% Dr.Noaman Page 1  Medicine - Delta Semester (7) Lymphoma Clinical picture ① Lymphadenopathy ⑥ Itching ② Splenomegaly ⑦ CNS manifestations ③ Hepatomegaly ⑧ Jaundice ④ Bone marrow involvement ⑨ Hematologic manifestations ⑤ B symptoms 1 Lymphadenopathy  Most common presentation is “peripheral L.N Enlargement” (90% of cases). ① Cervical 60-80% ② Axillary 20% ③ Inguinal 5%.  Characters of L.N.: ① Consistancy → Rubbery. ② Painless. ③ Not tender.  Mediastinal syndrome:  It can be presented by mediastinal L.N enlargement (60% of cases) Notes  Bulky: Nodal mass ≥ 6Cm  Bulky mediastinal mass: Mass/thoracic ratio ≥ 0.33%  Internal transverse diameter of a thorax between T5 and T6  Maximum inspiration  Upright position Dr.Noaman Page 2  Medicine - Delta Semester (7) Lymphoma 2 Splenomegaly  Stage of ds is at least stage III. 3 Hepatomegaly  Hepatomegaly & presence of Enlarged L.N at porta hepatis → Obstructive jaundice. 4 Bone marrow involvement ① Pallor → due to Anemia ② Bleeding → due to thrombocytopenia. 5 B symptoms  Systemic symptoms: 30% (B symptoms) ① Intermittent fever (pel-Epstien). ② Night sweats. ③ Loss of weight >10% during last 6 months. 6 Itching  15-25% of patients  Severe → advanced stage  B-symptoms  Return → Relapse 7 CNS manifestation ① Intra-cranial infiltration ④ Chemotherapy-related ② Metabolic: ↑ Ca, ↓ Na, ↓ glucose  Oncovin ③ Infection  Steroid  Herpes ⑤ Radiotherapy related.  Meningitis ⑥ Paraneoplasmic manifestations  Abscess Dr.Noaman Page 3  Medicine - Delta Semester (7) Lymphoma 8 Jaundice ① Hepatic involvement. ② Hemolytic anemia ③ Hepatitis (viral-Toxic-Toxoplasma) ④ Enlarged L.Ns in the porta hepatis ⑤ Blood transfusion 9 Hematologic ① Immune hemolytic anemia ④ Bone marrow suppression / infiltration ② Immune thrombocytopenia ⑤ Eosinophilia ③ Immune neutropenia  15% of patients DIAGNOSIS 1 Ultrasound Grey scale evaluation of LN  LN in lymphoma tend to be larger in size 1 Size  Progressive nodal enlargement on serial follow up is more important 2 Shape  Round in shape 3 Borders  Well-defined (except if infiltrating adjacent structure) 4 Echogenicity  Hypoechoic without echogenic hilus Suspicious LN Reactive LN Dr.Noaman Page 4  Medicine - Delta Semester (7) Lymphoma 2 L.N Biopsy  The most important diagnostic test.  Biopsy should be taken from the large L.N because it is the pathologic one.  Presence of Reed-sternberg giant cells surrounded by benign inflammatory cells is diagnostic for Hodgkin’s disease.  Accordingly the disease is histo-patholgically classified into 4 types (Rye classification): ① Lymphocyte predominance: Best prognosis. ② Lymphocyte depletion: Worst prognosis. ③ Mixed cellularity: Most common in children. ④ Nodular sclerosis: Most common in adults. 3 Radiologic studies ① Plain X-ray chest: to detect mediastinal L.N or lung disease. ② Abdominal ultrasound. ③ C.T scan: neck, chest, abdomen, pelvis. ④ MRI. ⑤ PET scan 4 Hematologic studies ① Complete blood picture:  Anaemia  Leucopenia ② Bone marrow exam:  For B.M infiltration. Dr.Noaman Page 5  Medicine - Delta Semester (7) Lymphoma 5 Biochemical studies These biochemical changes are indicative of active disease. ① ↑ ESR ② ↑ Serum Copper level. ③ ↑ Serum ferritin. ④ ↑ Fibrinogen level ⑤ ↑ Haptoglobin level. ⑥ ↑ Serum lactic dehydrogenase ⑦ ↑ Serum alkaline phosphatase Clinical staging of the disease Ann Arbor classification Stage Description  Single lymph node region I or  Of a single extra-lymphatic organ or site (IE).  Two or more lymph node regions on the same side of the diaphragm or II  localized involvement of an extra-lymphatic organ or site and of one more lymph node regions on the same side of the diaphragm (IIE).  Lymph node regions on both sides of the diaphragm III or  spleen, splenic hilar node, celiac node, porta hepatis node (IIIS).  Diffuse or disseminated involvement of one or more extra-lymphatic IV organs or tissues with or without associated lymph node enlargement. Each stage is further subdivided into A or B according to absence (A) or presence (B) of systemic symptoms. Dr.Noaman Page 6  Medicine - Delta Semester (7) Lymphoma Risk stratification Risk group Risk Criteria Low risk  Stage I or II without B symptoms or bulky disease (IA, IIA)  Stage IA, IIA with bulk disease Intermediate risk  IB, IIB, IIIA and IVA High risk  IIIB and IVB Prognosis  Good prognosis is suspected with: ① Young age. ⑤ Absence of systemic (B) symptoms ② Stages I or II. ⑥ Absence of bulk disease ③ Females. ⑦ Early good response to treatment ④ Lymphocytic predominance type TREATMENT  Combined chemotherapy &radiotherapy are the standard of careJ ① Radiotherapy:  For intermediate and high risk patients  2100cGy ② Chemotherapy: ABVD protocol in cycles (on D1 &D15) Low risk  4 cycles ± IFRT ① Adriamycin I.V. Intermediate risk  6 cycles + IFRT ② Bleomycin I.V. ③ Vinblastin I.V. High risk  8 cycles + IFRT ④ Decarbazine I.V  Autologus Bone marrow transplantation:  For recurrent and resistant disease. Dr.Noaman Page 7  Medicine - Delta Semester (7) Lymphoma NON Hodgkin’s Disease (NHL) OVERVIEW  Accounts for 60% of all childhood lymphomas.  Sex: males > females. 2.5:1.  Age: from 3 years old  Multicentric in origin. CLINICAL PICTURE ① Abdominal mass (Burkitt lymphoma):  It is the most common clinical presentation in our country. ② Peripheral L.N enlargement:  Cervical, axillary, inguinal…etc.  The glands are usually:  Firm (not rubbery)  Painless  Not tender ③ Mediastinal L.N enlargement → mediastinal syndrome. ④ Hepatosplenomegaly may occur. ⑤ Bone marrow infiltration may occur  Anemia  Leucopenia  Thrombocytopenia. DIAGNOSIS ① Lymph node biopsy. ② Bone marrow examination  To differentiate NHL from leukemia. ③ Peripheral blood exam. ④ Radiologic studies as for Hodgkin’s disease. Dr.Noaman Page 8  Medicine - Delta Semester (7) Lymphoma CLINICAL STAGING  As for Hodgkin’s disease but it is not sub classified to A or B.  Because the disease is more aggressive than Hodgkin’s disease. TREATMENT ① Surgery:  For emergency situation (intestinal obstruction). ② Chemotherapy:  The most important line of treatment (COPADM protocol)  Durgs used: 1. Cyclophosphamide. 2. Vincristine. 3. Prednisone. 4. Adriamycin 5. Methotrexate. ③ Radiotherapy:  For emergency situation e.g. SVC or Mediastinal syndrome ④ Bone marrow transplantation:  For recurrent and resistant diseases  Autologus or allogogeneic if the bone marrow is infiltrated or in relapsed cases post-transplant. Dr.Noaman Page 9