Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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Mature B-Cell Neoplasms Chronic Lymphocytic Leukaemia/ Small Lymphocytic Lymphoma Definition and Incidence: Chronic lymphocytic leukaemia / small lymphocytic lymphoma (CLL/SLL) is a neoplasm of monomorphic small, round B- lymphocytes in the peripheral blood, bone marrow and lymph nodes admixed with prolymphocytes and para-immunoblasts expressing CD5 and CD23. The term SLL is restricted to cases with the tissue morphology and immunophenotype of CLL but without a leukaemic component. CLL comprises 90% of chronic leukaemias in the USA and Europe and 7% of NHLs present as CLL/SLL. The majority of patients are >50 years old (median age 65) and the M: F ratio is 2:1. The incidence is 0.72cases /100,000 per year. ICD – O Codes: CLL 9823/3 B-SLL 9670/3 Clinical presentation Most patients with CLL are asymptomatic and the disease is diagnosed incidentally on routine full blood count. Presenting features can include lymphadenopathy, fatigue, auto-immune haemolytic anaemia, infection, or evidence of bone marrow failure. Pathology The lymphoid infiltrate effaces normal lymphoid architecture with a pseudo-follicular pattern of regularly distributed pale areas containing larger cells in a dark background of small cells The cells are slightly larger than normal lymphocytes, have a high nuclear-cytoplasmic ratio, round nucleus and occasional small nucleolus. The pseudofollicles contain a continuum of small, medium and large cells i.e. lymphocytes, prolymphocytes and para-immunoblasts. The size of the pseudofollicles and the number of para-immunoblasts vary but there is no well-documented correlation between histological findings and clinical outcome. Cell morphology can vary and may be confused with mantle cell lymphoma (MCL). Plamacytoid differentiation may also be present. In the blood and bone marrow similar small lymphocytes are found and smudge, smear or basket cells are typically seen on blood films. Prolymphocytes, which are larger cells with a prominent nucleolus, usually account for
Immunophenotype The tumour cells express pan B markers CD19 and 20 (weak), surface IgM and IgD (weak) and CD5 and CD23. The lymphocytes do not express CD10, cyclin D1, FMC7 and CD79b. Staging There are two clinical staging systems for CLL, the Rai (0-IV) and Binet (A-C) classifications. Recommended investigations Clinical: History and examination with reference to any family history Diagnostic Imaging: CXR, CT thorax abdomen and pelvis. Abdominal Ultrasound is acceptable for patients being treated with low intensity treatment or on a watch and wait policy Prognostic factors and Genetics Conventional bad prognostic indicators are; male gender, lymphocyte doubling time of
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 and 8: Standards in Staging of Lymphoma An
Page 9 and 10: Pathologic Diagnosis of Lymphoma Ge
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: Mature B-Cell Neoplasms Mature B-Ce
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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