Chronic lymphocytic leukaemia Flashcards
What is CLL
Type of blood cancer occurs due to monoclonal proliferation of B lymphocytes
Who gets CLL
Older adults
Exceedingly rare in children
More common in men
Causative factors
Cause unknown but more likely if:
Deletion in chromosome 13 can be 11 or 17 too
FH - 6-9x risk if first degree family had
Immunity - HIV/AIDS 3x likely
Pathophysiology of CLL
CD5+, CD23+ clonally expand, low surface IgGs
-> accumulation lymphocytes bone marrow -> lymph nodes and other lymphoid tissues
Main mechanisms of CLL
Deletion of chromosome
Hypogammaglobinaemia and haematopoieses
Transformation to aggressive forms
Genetic changes in CLL
13q chromosome deletion 50^
deletions at 11q, 17p (p53 tumour supressor) chromosomes
What is 17q deletion CLL ass with
p53 tumour supressor gene inactivated
Rapid progression, short remission, decreased overall survival
What haem disorders are people with CLL more likely to get
AI haemolytic anaemias
AI thrombocytopenia
What can CLL transfomr to
B cell pro lymphocytic leukaemia -> non hodgkin lymphoma
What is richters transformation
Richters transformetion = CLL -> diffuse large B cell lymphoma
Clinical features CLL
Often asymptomatic esp in early stages
Symmetrical enlarged lymph nodes
hepatomegaly/splenomegaly
Anaemia - tired
Recurrent infections as low Igs
Bleed/brusie - TO
Signs that CLL is active
B cell symptoms
Frequent sev night sweats
Unezplained weight loss >10% BW in last 6 months
High fever absence infection >38
Investigating CLL what do
FBC
Reticulocyte count
Direct antiglobulin test - DAT
immunophenotype
Biochem and serum IgGs
Peripheral blood smear
What do lymphocytes do in CLL
> 5000 B lymphcytes/uL - lymphocytosis
What test confirms CLL
Immunophenotyping of lymphocytes
How confrim lymphocytosis
Peipheral blood smear
Small lymphcytes - scant cytoplasm and nuclei w clumped chromatic
Smudge cells
What are smudge cells
Artefacts from damaged lymphocytes in slide prep as so fragile
What cells are suggestive of CLL on smear
Smudge cells
What genetic test should be performed on all patients before treatment with CLL
Detect TP53 gene
Adverse prognosis if deleted
Determines treatment
Additional investigaitons for CLL
Bone marrow aspiration and biopsy - before therapy as baseline
DAT/coombs test - in all anaemic patients and before commencing therapy
Imaging - CT scan chest abdo pelvis - uropathy or AW obstruction from lymph node compression on organ or structures