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Lymphomas Neoplasms of lymphoid tissues that typically causes lymphadenopathy. Classified pathologically into 2 types: Hodgkin lymphoma Non Hodgkin lymphoma.

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Presentation on theme: "Lymphomas Neoplasms of lymphoid tissues that typically causes lymphadenopathy. Classified pathologically into 2 types: Hodgkin lymphoma Non Hodgkin lymphoma."— Presentation transcript:

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2 Lymphomas Neoplasms of lymphoid tissues that typically causes lymphadenopathy. Classified pathologically into 2 types: Hodgkin lymphoma Non Hodgkin lymphoma

3 Epidemiology of lymphomas A common cancer; 5 th most frequently diagnosed cancer overall for both males and females males > females

4 Epidemiology of Hodgkin Lymphoma less frequent than non- Hodgkin lymphoma A bimodal peaks at the 3 rd and from the 6 th decades. 20s >50s a bimodal age-incidence curve

5 Age distribution of new NHL cases

6 Etiology of HL

7 Etiology of NHL Infection: Viral Infections:  EBV  Burkitt Chronic H.pylori infection  gastric lymphoma Immunodeficiency: AIDS Organ transplant Previous treatment for HL with chemo or radiotherapy

8 Modes of Spread of Lymphoma Hodgkin Lymphoma originate in a LN Almost always originate in a LN Contiguous spread Contiguous spread Extranodal disease to bone, brain or skin is rare.

9 Sites of involvment in HL Peripheral LN Peripheral LN Cervical and axillary LN (70%) Generalized lymphadenopathy is not typical in HL Thorax Thorax Anterior mediastinum in NS HL Lung Pleural effusion Pericardial effusion SVC obstruction Abdomen Abdomen Spleenomegaly Hepatomegaly Retroperitoneal LN

10 How a patient with lymphoma presents? Symptoms: Painless swelling Systemic symptoms; Weight loss Sweating Itching Fever Pain; Abdominal, bone, neurogenic or pain

11 Physical sings: Enlarged Lymph nodes

12 Physical signs: Splenomegaly Splenomegaly Hepatomegaly Hepatomegaly

13 Physical signs: Other physical findings Effusions Neurological deficits Compression syndromes SVC obstruction Intestinal obstruction Spinal cord compression Skin infiltration

14 Differential Diagnosis of Lymphadenopathy Infections Autoimmune disorders Malignant haematological diseases Leukemias Lymphoma Metastasis from other primary. AIDS Benign occiptial Posterior auricular Shotty inguinal nodes Cervical nodes

15 Differential diagnosis of Splenomegaly: Portal hypertension Haematological Malignancies. Leukemia Lymphoma Myeloma Myeloproliferative disorders Chronic Myeloid Leukemia. Myelofibrosis. Polycythemia Rubra Vera Essential thrombocytosis Autoimmune disorders Systemic Lupus Erythematosus Rheumatoid arthritis Infections Others Normal Splenic systs Thyrotoxicosis Sarcoidosis Amyloidosis

16 What is next?

17 Get a tissue diagnosis

18 A biopsy could be obtained from: Lymph node Lymph node Bone marrow biopsy Bone marrow biopsy Imaging guided biposy for retroperitoneal and mesentric masses Endocopic gastric biopsy

19 Send for pathological examination Routine microscopic examination Immunological examination Hodgkin vs Non Hodgkin CD20 positive or negative for some types of NHL Routine microscopic examination Immunological examination Hodgkin vs Non Hodgkin CD20 positive or negative for some types of NHL

20 Reed-Sternberg Cells Large malignant lympoid cells Bi-nucleated B-cell origin Present in small numbers Surrounded by reactive Tcells, plasma cells and eosinophils.

21 The pathology report Hodgkin Lymphoma Non-Hodgkin Lymphoma Reed Sternberg Cell

22 Which lymph node groups and organs are affected? THE EXTENT OF DISEASE

23 Staging investigations Evaluation of the chest 1-Chest X-Ray

24 Staging investigations Evaluation of the abdomen and retroperitoneum 2-Abdominal ultrasonography Lymphnodes Liver, spleen, kidneys

25 Staging investigations 3-CT scan of the chest, abdomen and pelvis

26 Staging investigations 4- Bone Marrow biopsy Indications of bone marrow biopsy: 1- ALL cases of Non Hodgkin Lymphoma. 2- Hodgkin Lymphoma when bone marrow involvement is suspected abnormal full blood count advanced stage of the disease.

27 Staging Investigations Hematological examination  Complete blood count  ESR  Liver function tests  Renal function tests  Serum LDH  Reflect level of tumour bulk and turnover  Particularly of relevance in aggressive NHL

28 Other staging investigations Endoscopy for GIT lymophomas Lumbar puncture In some cases of Non Hodgkin Lymphoma Burkitt lymphoma Lymphoblastic lymphoma Intermediate or high grade lymphoma involving; Testes, paranasal sinuses, extensive bone marrow involvment. MRI brain for AIDS related lymphoma PET scan: differentiate involved from non involved LN

29 Stage IStage IIStage IIIStage IV Staging of lymphoma Cotswolds Staging classification

30 Bulky Disease > 1. Mediastinal mass > ⅓ of the maximum transverse diameter of the chest > 2. Presence of nodal mass with a maximal dimension > 10cm

31 “B”symptoms Unexplained Fever > 38 o C Unexplained Weight loss > 10% body weight within the preceding 6 months. Drenching night sweets Stage A  No B symptoms Stage B  any one of the B symptoms LYMPHOMA STAGING

32 Lymphoma Stages I AI B II AII B III A IV AIV B

33 Hodgkin ’ s Lymphoma

34 Reed-Sternberg cell

35 Hodgkin lymphoma - Histological subtypes The WHO classification Nodular lymphocyte predominant HL (5%) Nodular lymphocyte predominant HL (5%) Slow growing localized Classical Hodgkin lymphoma (95%) Classical Hodgkin lymphoma (95%) nodular sclerosing mixed cellularity lymphocyte-rich lymphocyte depleted

36 INTENTION OF TREATMENT ISCURE With appropriate treatment: 90% of Stage IA are cured 70% of other stages are cured HODGKIN`S LYMPHOMA MANAGEMENT

37 Hodgkin lymphoma Treatment depends on: 1.Histological Subtype 2.Clinical Stage

38 Note: For Stage IA-IIA of Nodular Lyphocyte Predominant HL  Radiotherapy alone is enoughNote: Chemotherapy Chemotherapy (ABVD) 2-6 courses 1- Bulky disease 2- Residual disease 1- Bulky disease 2- Residual disease Chemotherapy Chemotherapy (ABVD) 8 courses

39 ADRIAMYCIN (DOXORUBICIN) BLEOMYCIN VINBLASTINE DACARBAZINE

40 Long term complications of treatment

41 Non-Hodgkin Lymphoma

42 NHL Low grade NHL Intermediate/High grade NHL Small cell size Round or cleaved nuclei Low mitotic rate Small cell size Round or cleaved nuclei Low mitotic rate Larger cell size Prominent nucleoli Higher mitotic rate Larger cell size Prominent nucleoli Higher mitotic rate Indolent/ non aggressive NHL Low proliferation rate Late symptoms Indolent course – uncurable with conventional therapy Indolent/ non aggressive NHL Low proliferation rate Late symptoms Indolent course – uncurable with conventional therapy Aggressive NHL High proliferation rate Rapidly produce symptoms Fatal if untreated Aggressive NHL High proliferation rate Rapidly produce symptoms Fatal if untreated

43 Non-Hodgkin lymphoma Incidence Diffuse large B-cell lymphoma (High Grade) Follicular Lymphoma (Low Grade) Other NHL 85% of NHL

44 Treatment of NHL 1- Grade of NHL ( Low or High) 2-STAGE OF NHL 1- Grade of NHL ( Low or High) 2-STAGE OF NHL

45 Indications for treatment Systemic Symptoms Rapid nodal growth Bone Marrow involvment. Compression Syndromes Indications for treatment Systemic Symptoms Rapid nodal growth Bone Marrow involvment. Compression Syndromes Radiotherapy Radiotherapy Chemotherapy (Chlarambucil, Fludarabine)Single agent (Chlarambucil, Fludarabine) (CVP)Or; Combination chemotherapy (CVP)Chemotherapy (Chlarambucil, Fludarabine)Single agent (Chlarambucil, Fludarabine) (CVP)Or; Combination chemotherapy (CVP) Observation and Follow up Palliative Radiotherapy for: SVC obstruction Spinal Cord Compression Pain Palliative Radiotherapy for: SVC obstruction Spinal Cord Compression Pain

46 Chemotherapy (CHOP) 3 cycles Chemotherapy (CHOP) 3 cycles Chemotherapy (CHOP) 6-8 cycles Chemotherapy (CHOP) + Rituximab For CD20 + Diffuse large B Cell lymphoma Chemotherapy (CHOP) + Rituximab For CD20 + Diffuse large B Cell lymphoma AND Radiotherapy AND Radiotherapy Radiotherapy to area of bulky disease Radiotherapy to area of bulky disease Autologus Stem Cell Transplantation

47 CYCLOPHOSPHAMIDEDOXORUBICIN VINCRISTINE (Oncovin) PREDNISOLONE

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49 Gastric MALToma Low grade histology Related to H.pylori infection Surgery is not routinely performed. Treatment:  Treat H.pylori infection  Chemotherapy if;  Large cell component  Deeply penetrating  Metastatic  Relapsing

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52 Classification of NHL The working formulation (1982) Clinical behaviour + histopathological features Not incorporated the origin of the cell ( B or T) Missing a large variaty of new clinicopathological entities. The WHO/REAL classification (1993) Incorporates immunophenotypes Differentiate between cells of T or B origin Recognizes seversal less common entities

53 The International Prognostic Index (IPI) for NHL Five independent prognostic factors 1- age older than 60 years 2- higher stage (III or IV) 3- More than one extranodal site involvement 4- lower performance status ( ECOG>1) 5- elevated serum LDH 0-1  5 yr survival is 73% 4-5  5 yr survival is 26%

54 HLNHLIncidence4: /yr12: /yr Reed-Sternberg cells PresentAbsent Cell Type B-cellB-cell(70%), T-cell(30%) SexMales>Females Medial Age 31 yrs65-70 yrs LN enlragement Usually supradiaphragmatic Any where Spread pattern ContiguousSkipped Extranodal involvement Less commonMore common Determinants of treatment Stage (I,II,III,IV) B symptoms Grade (Low/High) Stage(I,II,III,IV)

55 CategorySurvival of untreated patients CurabilityTo treat or not to treat Non- Hodgkin lymphoma Indolent Low Grade YearsGenerally not curable Generally defer Rx if asymptomatic Aggressive High Grade Weeks Months Curable in some Treat Hodgkin lymphoma All typesVariable – months to years Curable in most Treat


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