Lymphoid Malignancy Flashcards
Lymphoma
A cancer of lymphoid origin. This can present with enlarged lymph nodes, extranodal involvement or bone marrow involvement.
Systemic B Symptoms
Weight loss (> 10% in 6 months), fever, night sweats, pruritus, fatigue
How is the classification of lymphoma decided
Biopsy of site of tumour
Clinical examination and imaging (CT) tell us where it is.
Science of lymphoma
Malignancy occurs in the lymphoid region (germinal centre). To the lymph glands and the secondary lymphoid tissue.
Lymphoproliferative Disorders
Acute lymphoblastic leukaemia (ALL)
Chronic lymphocytic leukaemia (CLL)
Hodgkin lymphoma
Non-Hodgkin lymphoma (NHL)
Most common lymphoproliferative disorder
Non-hodgkin Lymphoma (High grade diffuse large B-cell lymphoma)
Acute Lymphoblastic Leukaemia
Neoplastic disorder of lymphoblasts diagnosed by >20% lymphoblasts present in the bone marrow.
Epidemiology of ALL
Incidence 1-2/100,000 population/year
75% cases occur in children < 6 years
75-90% cases are of B-cell lineage
Presentation of ALL
2-3 week history of bone marrow failure (with possible raised white cell count) or bone and joint pain, infection, night sweats
Emergency treatment in every lymphoma
Steroids
Standard treatment of ALL
Induction chemotherapy to obtain remission
Consolidation therapy
CNS directed treatment
Maintenance treatment for 18 months
Stem cell transplantation if they are high risk (20-30% mortality)
Indicators of poor prognosis in ALL
Increasing age
Increased white cell count
Immunophenotype (more primitive forms)
Cytogenetics/molecular genetics t(9,22), t(3;11)
Adult outcome ALL
complete remission rate 78–91%
leukaemia-free survival at 5y 30–35%
Child outcome ALL
5y overall survival ~90%
Chronic Lymphocytic Leukaemia
Diagnosed by blood >5x10^9/L lymphocytes.
Bone marrow >30% lymphocytes
Characteristic Immunophenotyping (CD 19, 20, 23) & CD5 positive (not supposed to be there)
Epidemiology of CLL
> 1700 new cases CLL per year in the UK
Commonest leukaemia worldwide
2 males: 1 female
Presentation of CLL
Often assymptomatic at presentation
Frequent findings: Bone marrow failure (anaemia, thrombocytopenia) Lymphadenopathy Splenomegaly (30%) Fever and sweats (B-symptoms) (< 25%)
Less common findings:
Hepatomegaly
Infections
Weight loss
CLL associated findings
Immune paresis (loss of normal immunoglobulin production)
Haemolytic Anaemia
CLL stage A
<3 lymph node areas
median survival is same as age matched control
CLL stage B
3 or more lymph node areas
Median survival is around 8 years
CLL stage C
Stage B + anaemia or thrombocytopenia
Median survival is around 6 years
Indications for treatment of CLL
Progressive bone marrow failure Massive lymphadenopathy Progressive splenomegaly Lymphocyte doubling time <6 months or >50% increase over 2 months Systemic symptoms Autoimmune cytopenias
Treatment of CLL
Often nothing – “watch and wait”
Cytotoxic chemotherapy e.g. fludarabine, bendamustine
Monoclonal antibodies e.g. Rituximab, obinutuzamab
Poor Prognostic Markers of CLL q
Advanced disease (Binet stage B or C) Atypical lymphocyte morphology Rapid lymphocyte doubling time (<12 mth) CD 38+ (plasma cell marker) expression Loss/mutation p53; del 11q23 (ATM gene) Unmutated IgVH gene status
Presentation of lymphoma
lymphadenopathy/hepatosplenomegaly
Extranodal disease
“B symptoms”
bone marrow involvement
Assessment (staging)
Lymph node biopsy
CT scan
Bone marrow aspirate
Trephine
Stage 1 lymphoma
One area of lymph node disease (usually the neck)
Stage 2 lymphoma
2 areas of lymph node disease both above the abdomen
Stage 3 Lymphoma
Above and below the abdomen
Stage 4 lymphoma
Extranodal disease
How is Non-Hodgkin Lymphoma classified
lineage (B-cell or T-cell)
-Majority are B-cell in origin
grade of disease (high grade or low grade)
histological features of disease
Low grade lymphoma
Indolent, often asymptomatic
responds to chemotherapy but incurable
median survival varies by subtype
High grade lymphoma
Aggressive, fast-growing
Require combination chemotherapy, steroids, antibodies (anti-CD20 monoclonal antibody + chemo)
Can be cured, but again varies widely
Diffuse large-B cell lymphoma
Sheet of lymphoma
Commonest subtype of lymphoma (of any kind)
High-grade lymphoma
Follicular Lymphoma
2nd commonest type of lymphoma (low grade lymphoma)
Looks like balls under the microscope
Epidemiology of Hodgkin Lymphoma
30% of all lymphomas bimodal age curve: -1st peak at 15-35y -2nd peak later in life 1.9 males: 1 female
Treatment of Hodgkin Lymphoma
Combination chemotherapy (ABVD)
+/- radiotherapy
Use of PET scan to assess response to treatment and to limit use of radiotherapy
