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Lymphoma Ismail M. Siala.

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Presentation on theme: "Lymphoma Ismail M. Siala."— Presentation transcript:

1 Lymphoma Ismail M. Siala

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; 5th 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 3rd and from the 6th decades. 20s >50s a bimodal age-incidence curve

5 Age distribution of new NHL cases

6 Etiology of HL

7 Etiology of NHL Infection: Immunodeficiency:
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 Almost always originate in a LN Contiguous spread Extranodal disease to bone, brain or skin is rare. Non Hodgkin Lymphoma Usually widespread at presentation Skippy spread Extranodal involvement is more common than in HL Bone marrow, GIT, Thyroid, Lung, Skin , testis, Brain and Bone.

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

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 The pathology report Hodgkin Lymphoma Non-Hodgkin Lymphoma

22 Which lymph node groups and organs are affected?
stage the disease 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 Staging of lymphoma Cotswolds Staging classification
Stage I Stage II Stage III Stage IV

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

31 LYMPHOMA STAGING “B” symptoms Stage A  No B symptoms
Unexplained Fever > 38oC 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 Stages I A I B II A II B III A IV A IV B

33 Hodgkin’s Lymphoma Thomas Hodgkin ( )

34 Reed-Sternberg cell

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

INTENTION OF TREATMENT IS CURE With appropriate treatment: 90% of Stage IA are cured 70% of other stages are cured

37 Hodgkin lymphoma Treatment depends on: Histological Subtype
Clinical Stage

38 Treatment of HL Stage I-II Stage III-IV
Note: For Stage IA-IIA of Nodular Lyphocyte Predominant HL  Radiotherapy alone is enough Chemotherapy (ABVD) 2-6 courses AND Radiotherapy Stage III-IV Chemotherapy (ABVD) 8 courses Radiotherapy ONLY FOR: 1- Bulky disease 2- Residual disease


40 Long term complications of treatment
Infertility sperm banking should be discussed premature menopause secondary malignancy skin, AML, lung, MDS, NHL, thyroid, breast... cardiac disease

41 Non-Hodgkin Lymphoma

42 NHL Low grade NHL Intermediate/High grade NHL Small cell size
Round or cleaved nuclei Low 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 Aggressive NHL High proliferation rate Rapidly produce symptoms Fatal if untreated

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

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

45 Treatment of Low Grade NHL
Stage I-II Radiotherapy Stage III-IV Observation and Follow up Indications for treatment Systemic Symptoms Rapid nodal growth Bone Marrow involvment. Compression Syndromes Chemotherapy Single agent (Chlarambucil, Fludarabine) Or; Combination chemotherapy (CVP) Palliative Radiotherapy for: SVC obstruction Spinal Cord Compression Pain

46 Treatment of high Grade NHL
Stage I-II – Non Bulky Chemotherapy (CHOP) 3 cycles AND Radiotherapy Stage I-II – Bulky Stage III-IV Chemotherapy (CHOP) 6-8 cycles Chemotherapy (CHOP) + Rituximab For CD20 + Diffuse large B Cell lymphoma Radiotherapy to area of bulky disease Relapsed Disease Autologus Stem Cell Transplantation

47 Repeat cycle every 3 weeks

48 Rituximab Monoclonal Antibody Against CD20 antigen.
Can be combined with other chemotherapy regimens for Diffuse Large B cell Lymphoma that is CD20 positive

49 Related to H.pylori infection Surgery is not routinely performed.
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

50 Thank you all for your attention


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 A practical way to think of lymphoma
HL NHL Incidence 4: /yr 12: /yr Reed-Sternberg cells Present Absent Cell Type B-cell B-cell(70%), T-cell(30%) Sex Males>Females Medial Age 31 yrs 65-70 yrs LN enlragement Usually supradiaphragmatic Any where Spread pattern Contiguous Skipped Extranodal involvement Less common More common Determinants of treatment Stage (I,II,III,IV) B symptoms Grade (Low/High) Stage(I,II,III,IV)

55 A practical way to think of lymphoma
Category Survival of untreated patients Curability To treat or not to treat Non-Hodgkin lymphoma Indolent Low Grade Years Generally not curable Generally defer Rx if asymptomatic Aggressive High Grade Weeks Months Curable in some Treat Hodgkin lymphoma All types Variable – months to years Curable in most

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