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Overview of Lymphomas

Overview of Lymphomas. Jessica Hals, DO June 16 th 2005. Definition. Lymphomas are malignant transformations of normal lymphoid cells which reside predominantly in lymphoid tissues They are divided into two major types: Non-Hodgkin’s lymphoma (NHL) Hodgkin’s Lymphoma. Some Stats 1.

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Overview of Lymphomas

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  1. Overview of Lymphomas Jessica Hals, DO June 16th 2005

  2. Definition • Lymphomas are malignant transformations of normal lymphoid cells which reside predominantly in lymphoid tissues • They are divided into two major types: • Non-Hodgkin’s lymphoma (NHL) • Hodgkin’s Lymphoma

  3. Some Stats1 • It is estimated that there will be 63,740 new cases of lymphoma diagnosed in 2005. • 56,390 are expected to be NHL • 19,200 of these pts are expected to die from NHL • 7,350 are expected to be Hodgkin’s Lymphoma • 1,410 of these pts are expected to die

  4. How to Diagnosis NHL • The initial evaluation must establish: • The precise histologic type of NHL • The extent and sites of disease • The performance status of the patient • All of this is important to establish prognosis and treatment

  5. Where to start • As always with the H&P: • Key points to obtain in your “history”: • Lymphadenopathy: more than 2/3 of pt will present with peripheral adenopathy • Ask about waxing and waning of lymph nodes • As about the duration of lymphadenopathy

  6. The History Cont’d • B Symptoms: • Fever defined as T>38ºC • Weight loss defined by unexplained loss of >10% of body wt over 6 mos • Night sweats defined by drenching night sweats

  7. The Physical Exam • Exam all sites of potential involvement including: • Waldeyer’s ring (tonsils, base of tongue, nasopharynx) • Std L.N. sites (cervical, inguinal, etc) • Liver and spleen • Abdominal L.N. (mesenteric, retroperitoneal) • Others: occipital, preauricular, epitrochlear, etc.

  8. Unusual Sites/Presentations • 10-35% will have primary extranodal NHL and about 50% will have extranodal disease during their illness • Most common site of extranodal disease is the GI tract followed by the skin • Symptoms from extranodal disease usually assoc with aggressive NHL

  9. Extranodal Sites • Testicular NHL accounts for ~1% of NHL and 2% of extranodal NHL. It is the most common malign. involving the testis in men over 60 y.o • NHL can present as solitary lesion of bone • Renal involvement occurs in 2-14% of pts • Rarer sites include: prostate, bladder, ovary, orbit, heart, breast, salivary gland, thyroid and adrenal gland • Examine skin carefully and bx any suspicious lesions • NHL can account for poorly differentiated carcinoma of unknown primary

  10. Diagnostic tests • Lymph node biopsy • Preferably to have an entire intact lymph node over FNA or core bx • This allows the pathologist to accurately determine the pattern of involvement and allows for enough tissue for immunologic and molecular testing

  11. Tests Cont’d • Bone marrow bx • This is to determine stage • Controversial whether bilateral or unilateral bx’s are required. • Most oncologists advocate bilateral biopsy

  12. Lab tests • CBC • Serum chemistries • LDH • Uric acid

  13. Imaging tests • CT chest/abd/pelvis • PET scan

  14. Classification3

  15. Staging3

  16. Prognostic Tools3

  17. The FLIPI Score • The IPI was designed for aggressive lymphomas. Few Follicular lymphomas fell into the high risk group based upon the IPI and therefore it’s application to FL was being questioned • Therefore the FLIPI has been proposed as a prognostic score for follicular lymphomas

  18. FLIPI • Five factors: • Age >60 • Ann Arbor stage III or IV • Hb <12g/dL • Number of nodal areas >4 • LDH >ULN

  19. FLIPI Risk Groups • Low risk: 0-1 adverse factor (5 &10yr OS=91% & 71% respectively) • Intermediate Risk: 2 adverse factors (5&10yr OS=78% & 51% respectively) • High risk: 3 or more (5&10 yr OS=52% &36% respectively)

  20. Aggressive NHL3

  21. Tx Aggressive NHL • Are highly curable lymphomas • If early disease present (localized, non-bulky stage I or II) may use XRT only for cure • However, most advocate combined therapy for early stage disease

  22. Historical tx of aggressive NHL • In 1972 Levitt, et al reported curability of large cell NHL with combination chemo2 • In 1975 DeVita et al described curing pts using COPP (Cytoxan, adriamycin, vincristine, procarbazine and prednisone)2 • During the 70’s this regimen was simplified to the classic CHOP regimen we use today (Cytoxan, adriamycin, vincristine and prednisone)

  23. TX of Early Stage • A SWOG protocol randomized pts to either 3 cycles of CHOP followed by involved field XRT vs. 8 cycles of CHOP alone3 • This showed that pts had a better 5 yr. PFS and OS with combined therapy • 76% vs. 67%, PFS respectively • 82% vs. 74%, OS respectively

  24. Early Stage Cont’d • The GELA trial2: • A French group has investigated a more intensive chemo regimen. They randomized pts to either 3 cycles of CHOP with XRT vs. ACVBP (adriamycin, Cytoxan, vindesine, bleomycin, prednisone followed with consolidation with ifosfamide, VP-16 and AraC) • This new regimen did improve EFS and OS, but at significant toxicity

  25. Early Tx Summary • For most patients with early, non-bulky (<10cm) stage I or II, 3 cycles of CHOP followed by involved field XRT is std • The role of using rituximab in early stage is gaining evidence: • Early studies suggest a benefit to adding rituximab to chemotherapy

  26. Advanced Stage Tx • CHOP is still the most commonly used regimen, and now with the addition of rituximab • Several groups are investigating more aggressive chemo regimens • Here are a few:

  27. The German group2 • They divided pts into three groups: young good px, young poor px, and elderly • They then randomized pts to one of four arms: • Arm 1: CHOP 21 (traditional 21 day cycle) • Arm 2: CHOP 14 (14 day cycle of CHOP) • Arm 3: CHOEP 21 (CHOP+VP-16 q 21 days) • Arm 4: CHOEP 14 (above q 14 days)

  28. The German Results • CHOEP 21 improved EFS, but CHOP 14 and CHOEP 14 improved EFS, CR and OS over std CHOP 21 • The Germans now consider CHOEP 14 preferred chemo for young good px pt • Based on the results of the MInT tx in young good px pts (CHOP-like chemo w/ Rituxan), they also will add Rituxan to their chemo • They are also using this same regimen for young poor px pts

  29. The French Approach3 • Study randomized pts to 3 cycles CHOP +XRT vs. 3 cycles ACVBP followed by consolidation. • EFS (82% vs. 74%), OS (90% vs. 81%) were in favor of the chemo only arm • Ongoing study using above +Rituxan

  30. The North Americans • CHOP+Rituxan considered std of care • Trials are ongoing to improve outcomes

  31. Indolent NHL • Follicular lymphoma is most common type of indolent NHL • Majority of pts present w/ stage III/IV disease with multiple enlarged LN that have been present for a long time • Generally not considered curable

  32. Natural Hx of Indolent NHL • Can have long symptom free intervals • Several studies show no OS advantage to early treatment vs. waiting until progression or symptoms develop. • Can go for years w/o needing tx. and obs alone is a feasible approach. Median survival for stage III/IV is 7-10 yrs

  33. Tx Early Stage I/II indolent NHL • For stage I/II XRT may be reasonable sole tx

  34. Tx for advanced disease • Chemotherapy remains the mainstay of treatment. • Various regimens exist • CVP, Fludarabine, FC, FCR, CVP-R • All appear to have same RR • Rituximab can be used alone or in combo w/ other regimens • Radio-labeled monoclonal antibodies are also available for refractory/relapsed disease (Bexxar and Zevalan) • Transplantation has been investigated for relapsed disease

  35. Summary of Tx Indolent NHL

  36. Tx summary Cont’d

  37. Marginal Zone Lymphomas (MZL) • 3 main types: • Splenic • MALT lymphoma • Nodal • Can occur in GI tract, salivary glands, thyroid, orbit, conjunctiva, breast and lung • Surgery or XRT usually sufficient to treat

  38. Splenic lymphoma • <5% NHL • Median age 65 • Present w/ splenomegaly, lymphocytosis • Course is indolent. Survival 70% @10yr • Tx of choice is splenectomy

  39. MALT lymphomas • Extranodal lymphoma associated w/ mucosal tissue • ~5% of NHL, 50% of these are gastric • Most are stage I/II at presentation • Gastric MALTomas assoc w/ H.pylori infection. Tx w/ Abx causes regression of lymphoma in majority of cases • XRT or resection can be used for other sites of MALToma

  40. Extra-nodal MZL • Are extremely rare • Usually indolent • Surgery can be used w/ or w/o XRT

  41. Mantle Cell Lymphoma • Considered intermediate aggressive. Median survival is 3-4 yrs. • Median age of 63 w/ male predominance. • Usually stage IV at dx • Distinctive features include: Cyclin D1+ and t(11:14)

  42. Tx Mantle Cell Lymphoma • CVP (Cytoxan, vincristine, prednisone) • Hyper-CVAD (mtx, adriamycin, Cytoxan, vincristine, dexamethasone, AraC-C) w/ and w/o rituximab has also been used. • Relapses are common even after BMT

  43. AID-related lymphomas • AIDS defining malignancies: • Kaposi’s sarcoma, NHL, primary CNS lymphoma, invasive cervical carcinoma • AIDS related NHL: • Primary CNS lymphoma (PCNSL) • Systemic NHL • 1º effusion NHL

  44. HIV related NHL • Usually in pts w/ CD4 count <100cell/µL • High grade NHL, (diffuse large B cell immunoblastic variant or Burkitt’s lymphoma are most common) • Indolent NHL are much less common • Most present w/o adenopathy and w/ stage IV dz.

  45. TX systemic AIDS related NHL • “std” chemo considered CHOP, although there is controversy. • Rituximab is investigational but early studies suggest synergism • HAART therapy should be continued or initiated • These pts do worse than in HIV(-) pts

  46. PCNSL in HIV • Usually w/ CD4 counts <50cell/µL • Present w/ focal or non-focal neurological symptoms: • confusion, lethargy, memory loss, hemiparesis, aphasia, and/or seizures that have usually been present for less than three months • DX w/ MRI/LP/EBV DNA in CSF/brain bx/rule out toxoplasmosis

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