medicine.Lymphomas.(dr.sabir)
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medicine.Lymphomas.(dr.sabir) Presentation Transcript

  • 1. Lymphomas Dr. Sabir M. Amin
  • 2. Definition
    • collection of lymphoid malignancies in which malignant lymphocytes accumulate in lymph nodes and
    • lymphoid tissues, leading to lymphadenopathy, extranodal disease, and constitutional symptoms
  • 3. Classification
    • Stage I
    • .. involvement of a single lymph node region or extra lymphatic organ or site
    • • Stage II
    • .. involvement of two or more lymph node regions or an extra lymphatic site and one or more lymph node regions on same side of diaphragm
    • • Stage III
    • .. involvement of lymph node regions on both sides of the diaphragm; may or may not be accompanied by single extra lymphatic site or splenic involvement
  • 4. Classification…cont
    • Stage IV
    • • diffuse involvement of one or more cxtralymphatic organs including BM
    • • subtypes:
    • A = absence of B symptoms
    • B = presence of B symptoms
    • • unexplained fever >38°C
    • • unexplained weight loss (>10% of body weight in 6 months)
    • • night sweats
  • 5. Hodgkin’s disease
    • malignant proliferation of lymphoid cells with Reed-Sternberg cells (thought to arise from germinal center B-cells)
    • Bimodal distribution with peaks at the age of 20 years and >50 years
    • .. association with Epstein-Barr virus in up to 50% of cases
  • 6. Clinical features
    • asymptomatic lymphadenopathy (70%)
    • • non-tender, rubbery consistency
    • • cervical/supraclavicular (60-80%), axillary (10-20%), inguinal (6-12%) • splenomegaly (50%) ± hepatomegaly
    • • mediastinal mass found on routine CXR, may be symptomatic (cough) rarely may present with SVC syndrome, pleural effusion
  • 7. Cl. Features…cont
    • B symptoms (especially in widespread disease; fever in 30%), pruritus
    • • alcohol-induced pain in nodes. nephrotic syndrome
    • .. starts at a single site in lymphatic system (node), spreads first to adjacent nodes disease progresses in contiguity with lymphatic system
  • 8. Investigation
    • CBC
    • .. anemia (chronic disease, rarely hemolytic), eosinophilia, leukocytosis, platelets normal or increased early, decreased in advanced disease
    • Biochemistry
    • LFTs (liver involvement)
    • RFTs (prior to initiating chemotherapy)
    • .. ALP, Ca (bone involvement)
    • • ESR, LDH (monitor disease progression)
  • 9. Investigation
    • i maging
    • .. CXR, CT chest (lymph nodes, mediastinal mass), CT abdomen/pelvis (liver or spleen involvement), gallium scan (assess treatment response)
    • cardiac function assessment - ( Echo for EF) for pts at high risk of pre-treatment cardiac disease (age :>60, history of HT, CHF, CAD, MI, CVA)
    • .. PFTs - if history of lung disease (COPD. smoking. previous radiation to lung)
    • .. excisional lymph node biopsy confirms diagnosis
    • .. bone marrow biopsy to assess marrow infiltration (only necessary if B symptoms, stage III or IV, bulky disease or cytopenia)
  • 10. Diagnosis
    • Diagnosis can be made with lymph node biopsy. There are four main histologic subtypes based on the No. of lymphocytes, Reed - Sternberg cells, and the presence of fibrous tissue:
    •     1.Lymphocyte predominance       ( after age 50 ) 2.Mixed cellularity     most common, young)   ) 3.Nodular sclerosis       4.Lymphocyte depletion
  • 11. Treatment
    • 1) stage I-II : chemotherapy (ABVD) followed by involved field XRT ( Adriamycin, Bleomycin, Vinblastin, Dacarbazine)
    • 2) stage III-IV : chemotherapy (ABVD, BEACOPP), with XRT for bulky disease
    • 3) Relapse, resistant to therapy: high dose chemotherapy, bone marrow transplant
    • BEACOPP( bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, prednisolone)
  • 12. Complications of treatment
    • cardiac disease - 2° to XRT, adriamycin is cardiotoxic
    • pulmonary disease - secondary to bleomycin( interstitial pneumonitis)
    • infertility - recommend sperm banking
    • secondary malignancy in irradiated field
    • .. <2% risk of MDS, AML (2° to treatment, usually within 8 years)
    • • solid tumours of lung, breast, >10 years after treatment
    • • non-Hodgkin's lymphoma
    • hypothyroidism - post XRT
    • infection - post splenectomy (give Pneumovax, HiB, and pneumococcal conjugate vaccines), during treatment
  • 13. Adverse prognostic factors
    • 1. serum albumin <4 gm/dL
    • 2. hemoglobin <10.5 gm/dL
    • 3. male
    • 4. stage IV disease
    • 5. age:<45 years
    • 6. leukocytosis (WBC >15000)
    • 7. lymphocytopenia (lymphocytes <8% of WBC )
  • 14. Non-Hodgkin’s lymphoma
    • malignant proliferation of lymphoid cells without Reed-Sternberg cells.
    • Originates from both B- (85%) and T- or NK- (15%) cells
    • • B cell NHL - e.g. Burkitt&quot;s lymphoma, mantle cell lymphoma. follicular lymphoma
    • • T cell NHL - e.g. mycosis fungoides. anaplastic large cell lymphoma
  • 15. Grade
    • WHO/REAL classification system - 3 categories of NHLs based on natural history:
    • 1) Low-grade ( indolent ) 35-40% - e.g. follicular lymphoma, small lymphocytic Iymphoma/CLL, mantle cell lymphoma
    • 2) Intermediate-grade ( aggressive ) 50% - e.g. diffuse large B-cell lymphoma
    • 3) High-grade ( highly aggressive ) 5% - e.g. Burkitt's lymphoma
  • 16. Clinical features
    • painless superficial lymphadenopathy, usually >1 lymph region
    • • usually presents as widespread disease
    • • B symptoms (fever, weight loss, night sweats) not as common as in Hodgkin's disease
    • • cytopenia: anemia ± neutropenia ± thrombocytopenia if BM fails
  • 17. Clinical features
    • • hepatosplenomegaly
    • • retroperitoneal and mesenteric involvement (2nd most common site of involvement)
    • • oropharyngeal involvement in 5-10% with sore throat and obstructive apnea
    • • extranodal involvement - most commonly Gl tract, also testes. bone. kidney
    • • CNS involvement in 1% (often with HIV)
  • 18. Investigation
    • CBC
    • normocytic normochromic anemia
    • • autoimmune hemolytic anemia
    • • advanced disease: thrombocytopenia, neutropenia, and leukoerythroblastic anemia
    • peripheral blood film sometimes shows lymphoma cells
    • Biochemistry
    • increase in uric acid
    • • abnormal LFTs in liver metastases
    • • increased LDH (rapidly progressing disease. poor prognostic factor)
  • 19. Imaging
    • CXR and CT for thoracic involvement
    • • CT for abdominal and pelvic involvement
    • • gallium scan is useful for monitoring response to treatment and evaluation of residual tumour following therapy
    • Diagnosed by lymph node biopsy
    • • fine needle aspiration occasionally sufficient. excisional biopsy preferred
    • bone marrow biopsy
  • 20. Treatment
    • Localized disease (e.g. Gl. brain, bone, head and neck)
    • surgery (if applicable)
    • • radiotherapy to primary site and adjacent nodal areas
    • • adjuvant chemotherapy
  • 21. Treatment..con
    • indolent lymphoma - goal of treatment is symptom management
    • watchful waiting
    • radiation therapy for localized disease
    • chemotherapy (single agent, combination or rituximab/ Rituxan, an anti-CD20 Ab)
  • 22. Treatment..con
    • Aggressive lymphoma - goal of treatment is curative
    • -combination chemotherapy: CHOP is mainstay. plus rituximab if B-cell lymphoma
    • -radiation for localized /bulky disease
    • -CNS prophylaxis with high-dose methotrexate (intrathecal or systemic) if certain sites involved
    • -relapse, resistant to therapy: high dose chemotherapy, BMT
  • 23. Treatment…con
    • Highly aggressive lymphoma
    • -Burkitt&quot;s Lymphoma - short bursts of intensive chemotherapy
    • -'CODOX-M&quot;' chemotherapy regimen also often used ± IVAC
    • -CNS prophylaxis and tumour lysis syndrome prophylaxis
  • 24. Complication
    • 1)Hypersplenism
    • 2) infection
    • 3) autoimmune hemolytic anemia and thrombocytopenia
    • 4)vascular obstruction (from enlarged nodes)
    • 5) tumour lysis syndrome (particularly in very aggressive lymphoma)
  • 25. Prognosis
    • poor prognostic factors
    • • >60 years old
    • • poor response to therapy
    • • multiple nodal regions
    • • elevated LDH
    • nodes >5 cm
    • • previous history of low-grade disease or AIDS
  • 26. Chronic lymphocytic leukemia (CLL)
    • indolent disease characterized by clonal malignancy of mature B-cells
    • most common leukemia in Western world
    • • mainly older patient; median age 65 years
    • • M>F
  • 27. Pathophysiology
    • accumulation of neoplastic lymphocytes in blood, bone marrow, lymph nodes and spleen
  • 28. Clinical features
    • 25% asymptomatic (incidental finding)
    • • 5-10% present with B symptoms (≥1 of: unintentional weight loss ≥10% of body weight within previous 6 months, fevers >38°C or night sweats for ≥2 weeks without evidence of infection, extreme fatigue)
    • • lymphadenopathy (75%), splenomegaly (35%), hepatomegaly (20%)
    • • immune dysregulation - autoimmune hemolytic anemia (Coombs +), immune thrombocytopenia purpura (ITP), hypogammaglobulinemia ± neutropenia
    • • BM failure - late, secondary to marrow involvement by CLL cells
  • 29. Investigation
    • CBC : absolute lymphocytosis >5000
    • • peripheral blood film
    • • lymphocytes are small and mature
    • • smudge cells
    • flow cytometry
    • cytogenetics - FISH
    • bone marrow aspirate
    • lymphocytes >30% of all nucleated cells
  • 30. Natural history and treatment
    • natural history - indolent but incurable, with slow progression; thus select gentlest treatment that will control symptoms
    • observation if early, stable, asymptomatic
    • intermittent chlorambucil or fludarabine
    • corticosteroids, IVIG - especially for autoimmune phenomena
    • • radiotherapy
    • • chemotherapy - including Rituximab (anti-CD20 mAb)
    • • small minority present with aggressive disease usually associated with chromosomal abnormalities (e.g. p53 deletion)
    • • 9 year median survival, but varies greatly
  • 31. Complications
    • 1) 5% undergo Richter's Transformation - aggressive transformation to Diffuse Large B-Cell Lymphoma
    • 2) hyperuricemia with treatment