LYMPHOMA
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Transcript of LYMPHOMA
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LYMPHOMA
Huang Jinwen
Hematology Dept. of SRRSH
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Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage of the disease.
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Lymphomas, multiple myeloma world map - Death - WHO2004
(per 100,000 inhabitants)
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100
150
200
250
300
350
1975 1976-1978
1981-1983
1986-1988
1991-1993
1996-1998
2001-2003
Period of diagnosis
% o
f 19
75 r
ate
under25 25-44 45-64 65-74 75+ All
Figure 1.4: Percentage change in age-specific incidence ofNHL from 1975, persons, Great Britain
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NHL CANCER INCIDENCE 2002
0 5 10 15 20
South Central Asia
Eastern Asia
Northern Africa
Middle Africa
Eastern Europe
Southern Africa
Central America
South-Eastern Asia
Western Africa
South America
Western Asia
Eastern Africa
Southern Europe
Northern Europe
Western Europe
Australia/New Zealand
Northern America
Rate per 100,000 population
Incidence
Mortality
Figure 1.5: Age-standardised incidence and mortality rates for NHLin males by region of the world, 2002 estimates
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WHO Classification of Lymphomanon Hodgkin lymphoma
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What should we do ?
when we meet a patient with suspected non-Hodgkin’s lymphoma
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PAST HISTORY • A personal or family history
• Relevant infectious illnesses
• Connective tissue diseases, immunodeficiency disorders etc.
• Agricultural to pesticides and Agent Orange
malignancy, radiation therapy, immunosuppressive agents, chemotherapy, organ transplantation , etc.
HIV-I,
HTLV-I,
Epstein-Barr virus (EBV), hepatitis C virus,
Pyothorax-associated lymphoma.
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CHIEF COMPLAINTS
Systemic complaints (B symptoms)
* Fever — temperature >38ºC
* Weight loss >10 percent over the past six months
* Sweats — the presence of drenching night sweats
Lymphadenopathy
* Rapid and progressive or Waxing and waning
* The duration, observed sites, and extent
* Peripheral lymphocytosis
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Symptoms in gastrointestinal lymphoma according to involved site
Symptom Stomach n = 277 Small bowel n = 32 Ileocecal n = 26 Multiple sites n = 24
Pain 78 75 77 58
Loss of appetite 47 41 23 58
Weight loss 24 34 15 25
Bleeding 19 6 12 8
Vomiting 18 31 8 21
Night sweats 11 12 19 46
Diarrhea 4 12 19 29
Constipation 3 25 23 12
Fever 2 6 8 4
Perforation 2 9 - -
Ileus - 38 19 4
No symptoms 4 - - -
This table shows the percent of patients with the listed symptom at each of the four major
sites of disease. Data from Koch, P, et al. J Clin Oncol 2001; 19:3861.
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ONCOLOGIC EMERGENCIES • Spinal cord compression• Pericardial tamponade• Hypercalcemia (adult T cell lymphoma)• Superior or inferior vena cava obstruction• Hyperleukocytosis (lymphoblastic lymphoma)• Acute airway obstruction (mediastinal lymphoma)• Lymphomatous meningitis and/or CNS mass• Hyperuricemia and tumor lysis syndrome• Hyperviscosity syndrome • Intestinal obstruction, intussusception• Ureteral obstruction, unilateral or bilateral• Severe hepatic dysfunction • Venous thromboembolic disease • Severe autoimmune hemolytic anemia and/or thrombocytopenia
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PHYSICAL EXAMINATION
• Waldeyer's ring • Standard lymph node sites • Liver and spleen• Abdominal nodal sites (mesenteric, retroperitonea
l)• Less commonly involved nodal sites (eg, occipital,
preauricular, epitrochlear, popliteal)
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PHYSICAL EXAMINATION : Head and Neck
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Waldeyer's ring
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Head and neck
• Waldeyer's ring is more frequently observed in patients with NHL than in HL.
• A useful clue to the presence of involvement of Waldeyer's ring is enlargement of preauricular nodes.
• Primary central nervous system lymphoma commonly involves the eye.
• Lymphoma involving the orbital structures is rare, but may be seen in marginal zone and mantle cell lymphoma.
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PHYSICAL EXAMINATION : Superior vena cava syndrome
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Chest and lungs
• ~ 20% of pts with NHL present with mediastinal adenopathy.
• A superior vena caval syndrome is part of the clinical presentation (3 to 8 %).
• Pleural disease is seen in about 10 percent of all patients with NHL at diagnosis.
• The differential diagnosis of mediastinal presentation includes infections,
sarcoidosis, Hodgkin's lymphoma, other neoplasms.
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Mediastinal lymphoma
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Abdomen and pelvis • Retroperitoneal, mesenteric, and pelvic involveme
nt is common in most histologic subtypes of NHL. • Diffuse hepatosplenomegaly is common in the ind
olent lymphomas, • Hepatic masses are more commonly seen in the ag
gressive or highly aggressive lymphomas.• Not all focal liver lesions in a patient with NHL ar
e due to lymphoma. • Ascites may be present
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PHYSICAL EXAMINATION : Abdomen and Pelvis
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Retroperitoneal lymphadenopathy
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Extranodal sites • Patients with NHL will have primary extrano
dal lymphoma at initial Diagnosis (10~35 %).
• The most common site of primary extranodal disease is the GI tract, followed by skin.
• Symptoms due to extralymphatic disease are usually associated with aggressive NHL.
• The skin should be carefully examined for lesions; all suspicious areas should be biopsied.
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PHYSICAL EXAMINATION : Extranodal sites
Colonic involvement with lymphoma Lymphoma affecting the kidney
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Lymphoma of bone Lymphoma of testis
PHYSICAL EXAMINATION : Extranodal sites
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INITIAL LABORATORY STUDIES • CBC with differential• Renal and hepatic function• Serum calcium, electrolytes, and uric acid• Serum protein electrophoresis• the tumor markers beta-2 microglobulin
lactate dehydrogenase
etc
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LYMPH NODE AND TISSUE BIOPSY
• Peripheral lymphonodes
• CT-guided core needle biopsies
• Bone marrow examination
• Laparoscopic multiple biopsies• Surgical operation
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Lymph node selection
• Size:
* Significant enlargement
* Persistence for more than four to six weeks
• Site: * Supraclavicular nodes — 75 to 90 percent
* Cervical and axillary nodes — 60 to 70 percent
* Inguinal nodes — 30 to 40 percent
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Studies on excised tissue
• An intact lymph node is critical for histologic, immunologic, molecular biologic assessment.
• The FNA findings of "lymphoma" requires to be confirmed.
• Immunologic, cytogenetic, and molecular studies are useful for making therapeutic decisions and assessing prognosis.
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Reactive lymph node versus follicular lymphoma, and versus diffuse lymphoma
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Bone marrow examination • BM involvement occurs commonly in the indolent
histologies. • BM aspirates / BM biopsy
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CLINICAL EVALUATION
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Ann Arbor staging classification for Hodgkin's and NHL
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"B" symptoms
• “B” symptoms are more common in aggressive/ highly aggressive histologies (47%) .
• < 25 % with indolent lymphomas have B symptoms.
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International Prognostic Index
• Age >60• Serum LDH• ECOG performance status 2 • Ann Arbor clinical stage III or IV • Number of involved extranodal disease sites >1
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ECOG Performance Status
Performance Status Definition
0 Fully active; no performance restrictions
1 Strenuos physical activity restricted; fully ambulatory and able to carry out light work
2 Capable of all selfcare but unable to carry out any work activities, Up and about 50 percent of waking hours
3 Capable of only limited selfcare; confined to bed or chair, less than50% of waking hours
4 Completely disabled; cannot carry out any selfcare; totally confined to bed or chair
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5-yr OS and CR rates according to IPI score
Score Risk group 5-yr OS CR rate
(%) (%)
0 to 1 Low risk 73 87
2 Low-intermediate risk 51 67
3 High-intermediate risk 43 55
4 to 5 High risk 26 44
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Treatment
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OS compared between conventional chemotherapies and R-CHOP
Patients over 60 (LNH98-5) 2002
100
80
60
40
20
0
0 5 10 15
CHOPMACOP-BProMACE-CytaBOMm-BACOD
Ove
rall
surv
ival
(%
)
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侵袭性局灶 NHL 的治疗 : 化疗或化疗 + 放疗
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LNH87-2 PROTOCOL
高危患者自体干细胞移植后生存优于化疗
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N Engl J Med, Vol. 346, No. 25.June 20, 2002
OS According to Gene-Expression Profiles in DLBCL
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Genetic, molecular and characteristics of the DLBCL subgroups and PMBL recognized by expression profiling
WHO classification of tumours and haematopoietic and lymphoid tissues,2008
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Blood, 11 June 2009, Vol. 113, No. 24, pp. 6069-6076
Differential efficacy of bortezomib plus chemo within subtypes of DLBCL
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THANKS