Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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ECOG performance status Grade ECOG 0 Fully active, able to carry on all pre-disease performance without restriction 1 Restricted in physically strenuous activity but ambulatory and able to carry out work of a light or sedentary nature, e.g., light house work, office work 2 Ambulatory and capable of all self care but unable to carry out any work activities. Up and about more than 50% of waking hours 3 Capable of only limited self care, confined to bed or chair more than 50% of waking hours 4 Completely disabled. Cannot carry out any self care. Totally confined to bed or chair 5 Dead Staging procedures-all patients Clinical ■ Clinical history with reference to B symptoms and family history ■ Physical examination with particular attention to node-bearing areas, Waldeyer’s ring, and size of liver and spleen ■ Performance status (ECOG) including co-morbidity Radiology ■ CXR ■ Chest and abdominopelvic computed tomography (CT) Haematology ■ FBC, differential and film ■ Bone marrow aspirate and trephine ■ Immunophenotyping of marrow +/- blood in low grade lymphomas and any other lymphomas with morphological evidence of marrow/blood involvement Biochemistry ■ LDH, urea and electrolyte, creatinine, albumin, aspartate transaminase (AST), bilirubin, alkaline phosphatase, serum calcium, uric acid ■ Pregnancy test in females of child-bearing age 5 Serology ■ ■ Hepatitis B and C HIV status Staging procedures (sometimes indicated) Radiology ■ ■ ■ Plain bone X-ray and bone scintigraphy Neck CT Head CT or magnetic resonance imaging (MRI) ■ PET scan Haematology ■ Coagulation screen ■ ESR ■ DCT Biochemistry ■ Serum immunoglobulins/electrophoresis ■ Beta 2 microglobulin ■ CRP ■ Tissue transglutaminase test (tTG) to exclude coeliac disease Serology ■ EBV, HTLV serology Molecular genetics ■ FISH or PCR on involved marrow/blood for specific lymphoma-associated translocations ■ IgH and TCR rearrangements on marrow/blood if molecular staging clinically indicated Others ■ ECHO and PFTs ■ Lumbar puncture if lymphomatous meningitis is suspected or if indications for prophylactic treatment are present. CNS prophylaxis is currently used in patients with Burkitt Lymphoma, lymphoblastic lymphoma, HIV-related lymphoma, HTLV-1 related lymphoma and post-transplant lymphoproliferative disease. About 5% of patients with DLBCL develop CNS disease but there is no consensus about which patients need to have a diagnostic lp.
Pathologic Diagnosis of Lymphoma General comments 1. Primary diagnosis a. Complete lymph node excision in the presence of nodal disease is the optimum diagnostic material and should be utilised wherever possible. * b. The complete lymph node excision should be transported immediately in a fresh state to the laboratory. c. The fresh lymph node should be handled as per algorithm on page 15. d. *Where full lymph node excision is not possible e.g. inaccessible mass or extranodal disease, core biopsy is the next best option for accurate primary diagnosis unless a resection has been or will be undertaken. Multiple large cores should ideally be submitted fresh to the laboratory on saline soaked gauze otherwise they should be submitted in 10% buffered formalin. e. All cases must be received with relevant clinical information. 2. The role of Flow Cytometry (FCM) a. FCM is useful for accurate diagnosis in all small cell / follicular pattern lesions. b. FCM on aspirated material may provide a reasonable alternative to biopsy in recurrent disease where tissue is not easily obtainable e.g. elderly/frail patient, inaccessible site. Such FNA samples require co-ordination with the laboratory as special fixation (RPMI) and immediate processing is required 3. Optimum handling of fresh lymph node excision a. Ensure immediate transfer from the theatre to laboratory. b. Bisect node and perform touch preparations. i. Air dried x 2 – Giemsa stain ii. Air dried X6-8 FISH studies if required iii. Fixed x 2 – H & E stain c. Sample portion of tissue for: i. Freezing – 1 portion in “RNA later”, 1 portion fresh frozen (this will preserve material for molecular study should this be required). ii. RPMI for FCM (this will preserve the cells and allow transport to nearest centre offering FCM and/or Cytogenetics service). iii. Formalin for routine fixation and processing. 4. Classification and grading a. Use WHO classification 4th edition 2008 b. Where applicable use WHO grading e.g. Follicular Lymphoma (see page 29) 5. The role of FCM/Cytogenetics/ Gene expression profiling Material should be preserved in RPMI and frozen for availability for cytogenetic analysis and other relevant evaluation 6
Page 1 and 2: Guidelines on Diag
Page 3 and 4: Guidelines on Diag
Page 5 and 6: Standards In Diagnosis of Lymphoma
Page 7: Standards in Staging of Lymphoma An
Page 11 and 12: Hodgkin lymphoma and its differenti
Page 13 and 14: Potential diagnostic pitfalls for l
Page 15 and 16: Summary of the usual Immunostaining
Page 17 and 18: Mature B-Cell Neoplasms Mature B-Ce
Page 19 and 20: Immunophenotype The tumour cells ex
Page 21 and 22: Lymphoplasmacytic Lymphoma Definiti
Page 23 and 24: Splenic Marginal Zone Lymphoma Defi
Page 25 and 26: Extra-nodal Marginal Zone B-Cell Ly
Page 27 and 28: Persistent / progressive disease or
Page 29 and 30: Follicular Lymphoma Definition and
Page 31 and 32: Potential Pitfalls a. Failure to di
Page 33 and 34: Mantle Cell Lymphoma Definition and
Page 35 and 36: Diffuse Large B-Cell Lymphoma Defin
Page 37 and 38: Stage 1 (bulky) and stages II - IV
Page 39 and 40: Treatment Standard chemotherapy for
Page 41 and 42: Burkitt Lymphoma/Leukaemia Definiti
Page 43 and 44: CODOX-M, (3 cycles) for low risk di
Page 45 and 46: Potential pitfalls Failure to diffe
Page 47 and 48: Precursor T Cell Neoplasms PRECURSO
Page 49 and 50: Mature T-Cell and NK-Cell Neoplasms
Page 51 and 52: Potential Pitfalls Treatment planni
Page 53 and 54: Recommended Investigations Evaluati
Page 55 and 56: Mycosis Fungoides and Sézary Syndr
Page 57 and 58: ■ ■ Lymph node biopsy if there
Page 59 and 60:
Primary Cutaneous CD30-Positive T-C
Page 61 and 62:
Angioimmunoblastic T-Cell Lymphoma
Page 63 and 64:
Prognostic factors The IPI is of li
Page 65 and 66:
Hodgkin Lymphoma (HL) Definition an
Page 67 and 68:
Prognostic Factors / Index: i. Earl
Page 69 and 70:
Immunodeficiency Associated Lymphop
Page 71 and 72:
of cases and EBV in about 50%, but
Page 73 and 74:
Common Issues in Lymphoma Managemen
Page 75 and 76:
Tumour Lysis Syndrome Tumour Lysis
Page 77 and 78:
Haematopoietic Recombinant Growth F
Page 79 and 80:
Follow-Up Follow-up for patients wi
Page 81 and 82:
Follow-up for patients with concern
Page 83 and 84:
77 Appendix
Page 85:
Lower border: The lower of (i) 5 cm
Page 88 and 89:
Glossary of Treatment Regimens Chlo
Page 90:
2nd Edition. May 2010. Supported by
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Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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