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2. Lymphoma
• Progressive neoplastic condition of lymphoreticular
system arising from stem cells
• 3rd most common mailgnancy among children comprising
15% of Paediatric cancers
3. Etiology
1. Genetic predispostion
2. Sjogren’s syndrome – 30
fold increase of NHL
3. HIV infection
4. Virus – Epstein-Barr virus
8. Celiac sprue – Intestinal
T cell lymphoma
9. H Pylori – associated
with MALT lymphoma
10.Occupation – Hair dye
workers
11.Ionising radiation
12.Smoking, Alcohol and
Tobacco usage
13.Common in Western
countries
4. Classification
• WHO modified REAL classification of Lymphoma (Revised
European American lymphoma)
1. B-cell neoplasm
• Precursor B cell neoplasm – ALL
• Peripheral B cell neoplasm – B cell related NHL
2. T-cell and putative NK cell neoplasms
• Precursor T cell neoplasm – ALL, LBL T cell related
• Peripheral T cell and NK cell neoplasm – T cell
related NHL
7. Hodgkin’s Lymphoma (HL)
• It is the most common type of lymphoma.
• Causes Epstein-barr virus, HIV infection and genetic
monoclonal B cell disorder (90%)
8. Hodgkin’s Lymphoma (HL)
• Slightly more in males (1.5:1)
• Bimodal (seen in young age of 20- 30 years as well as in
elderly greater than 50 years)
• Grossly lymph nodes: Fleshy, pinkish grey, and rubbery in
consistency.
• Microscopically: Cellular infiltration with lymphocytes,
reticulum cells, histocytes, fibrous tissue and Reed
Strenberg cells (giant cells with two large image nuclei)
10. Nodular sclerosis
• It is the most common subtype
• Incidence: Male = Female
• Microscopically Lacunar cells and occasional RS cells,
background T lymphocytes, eosinophils, macrophages
and plasma cells.
11. Lymphocyte depleted
• Uncommon
• More common in older males
• Associated with HIV- infected individuals
• Microscopically Frequent RS cells and variants, background Reactive
cells
• Worst prognosis
12. Clinical Features
• Painless rubbery lymphadenopathy, usually in the neck or
supraclavicular fossae; the lymph nodes may fluctuate in
size.
• Large mediastinal masses which are asymptomatic but may
cause dry cough and breathlessness.
• Isolated subdiaphragmatic nodes occur in less than 10% at
diagnosis.
13. Clinical Features
• Hepatosplenomegaly may be present but does not always
indicate disease.
• Spread is contiguous from one node to the next and
extranodal disease, such as bone, brain or skin involvement,
is rare.
• Systemic symptoms: Fever, night sweats, weight loss, Bone
pain
14. Investigations
• CBC: may be normal. If a normochromic, normocytic
anaemia or lymphopenia is present, this is a poor
prognostic factor.
• An eosinophilia or a neutrophilia may be present.
• ESR: may be raised.
• Renal function tests: are required to ensure function
is normal prior to treatment.
15. • Liver function test: may be abnormal in the absence
of disease or may reflect hepatic infiltration. An
obstructive pattern may be caused by nodes at the
porta hepatis.
• LDH measurements showing raised levels are an
adverse prognostic factor.
• Chest X-ray: may show a mediastinal mass.
• CT scan of chest, abdomen and pelvis permits staging.
Bulky disease (> 10 cm in a single node mass) is an
adverse prognostic feature.
• Lymph node biopsy
16. Stage I Stage II Stage III Stage IV
Ann-Arbor Staging of lymphoma
A: absence of (systemic) symptoms
B: fever, night sweats, weight loss
18. Management
Radiotherapy : Curative for stage I & IIA
Debulking surgery f/b chemo in stage I & II.
Chemotherapy: Stage III & IV
• MOPP regime : mustine, oncovin/vincristine,
procarbazine & prednisolone.
• ABVD regime: adriamycin, bleomycin, vinblastin,
dacarbazine.
19. •Non-Hodgkin’s lymphoma
• It represents monoclonal proliferation of lymphoid cells of B cell
(70%) and T cell (30%) origin
• Lymphoma is used for proliferations that arise as discrete tissue
masses
20.
21. Follicular lymphoma
• Most common type of “indolent” lymphoma.
• Affects older persons.
• Usually widespread at presentation
• Often asymptomatic.
• Not easily curable.
• Associated with bcl-2 gene rearrangement [t(14;18)]
• Cell of origin: germinal center b-cell
• Lymph node appears nodular with effacement of normal
architecture.
• Has to be differentiated from reactive lymphadenitis.
22. Diffuse large B-cell lymphoma
• Most common type of “aggressive” lymphoma.
• Usually symptomatic.
• Age group affected : wide variation.
• Extranodal involvement is common.
• Cell of origin: germinal center b-cell
• Curable in ~ 40%
• Aggressive neoplasm requiring intensive combined
chemotherapy.
23. Burkitt’s lymphoma
• B cell lymphoma with t(8;14)
• Aggressive lymphoma.
• Endemic in Africa & sporadic in other areas.
• Jaw(maxilla/mandible) involvement in endemic African forms.
• Abdominal viscera(bowel, retroperitoneum, ovaries) involved in
sporadic forms.
• Children and young adults are affected
24. Microscopy
• Tumor appears monotonous, with effaced
architecture.
• Medium sized tumor with high mitotic and
apoptotic rate.
• Presence of numerous benign tingible body
macrophages giving the appearance of “Starry sky
pattern”
.
25. Clinical features
• Lymphadenopathy
• Weight loss, Fever, Sweats, Itching
• Hepatosplenomegaly
• Extranodal involvement in bone marrow, gut, thyroid, lung, skin,
testis, brain, bone
• Compression syndromes like gut obstruction, ascites, superior vena
cava obstruction, spinal cord compression
26. Investigations
As HL but some addition as
oBone marrow aspiration
oImmunophenotyping of surface antigen to distinguish
T from B cell tumor
oCytogenetic analysis to detect chromosomal
translocation
oImmunoglobulin determination for treatment
response
oMeasurement of uric acid level to prevent renal
failure
oHIV testing
27. Management of NHL
Radiotherapy
Chemotherapy
oRituximab (R) + cyclophosphamide + vincristine +
prednisolone (RCVP)
oCHOP regimen (cyclophosphamide, doxorubicin,
vincristine and prednisolone)
oHumanised monoclonal antibodies to target
surface antigens on tumor cells leading to
apoptosis directly
28. Management of NHL…
Transplantation: High dose chemotherapy and Haematopoietic Stem
Cell Transplantation (HSCT) in patient with relapsed disease
29. Clinical difference between Hodgkin’s and
Non-Hodgkin’s lymphoma
Hodgkin Non-Hodgkin
More often localized to a single axial
group of nodes (cervical, mediastinal,
para-aortic).
More frequent involvement of multiple
peripheral nodes.
Orderly spread by contiguity. Noncontiguous spread.
Mesenteric nodes and Waldeyer ring
rarely involved.
Mesenteric nodes and Waldeyer ring
commonly involved.
Extranodal involvement uncommon. Extranodal involvement common.
30. 1. Fauci AS, Braunwald E, Kasper DL, Hauser SL,
Longo DL, Jameson JL, et al., editors. Harrison’s
principles of internal medicine. Hematology and
Oncology.19th ed. New York: McGraw Hill; 2014
2. H.G. Watson, J.I.O Craig, L.M. Manson, editors.
Davidson’s Principal & Practice of Medicine.
Blood Disorders. 22nd edition. Edinburgh: Elsevier
Limited. 2014
REFERENCES