Haematology 4 Flashcards
Malignant lymphomas
Diseases of tissue based and nodal lymphocytes
Classification of haematological malignancy
Myeloid-> including myeloproliferative disorders, myelodysplasia
-> myeloid stem cells produce all but lymphocytes
Lymphoid-> including lymphomas, immunosecretory disorders
-> lymphoid stem cells-> lymphocytes
Leukaemias
And myeloproliferative disorders
Maligant neoplasms derived from cells of the bone marrow
Acute-> proliferation of immature cells (blast cells)
Chronic-> proliferation of mature cells
Myeloid disorders
Acute myeloid leukaemia-> adults Chronic myeloid leukaemia Myeloproliferative disorders:-> abnormal bone marrow stem cell proliferation Essential thrombocytopenia Polycythaemia rubra Vera Myelofibrosis Idiopathic hyper eosinophilic syndrome Systemic mastocytosis
Myelodysplastic syndromes
Lymphoid disorders
Acute lymphoblastic leukaemia Chronic lymphocytic leukaemia Plasma cell disorders Non Hodgkin's lymphomas T cell lymphomas
Myeloproliferative disorders
Abnormal bone marrow stem cell proliferation
Excess of mature cells in blood
Polycythaemia rubra Vera-> excess red cells
Essential thrombocythaemia-> excess platlets
-> both may lead to myelofibrosis or acute leukaemia
->both have Janus kinase gene JAK2 problems
-> abnormal clotting
Myelofibrosis-> proliferation of fibroblasts in bone marrow-> scarring-> bone marrow failure
Chronic granulocytic leukaemia
Acute Leukaemias
Malignancy of bone marrow
Very rapid cell growth
May fill marrow before spilling out in to blood
High WBC not always present
Present with bone marrow failure-> anaemia, thrombocytopenia, neutropenia
Either lymphoblastic or non lymphoblastic
Treating acute leukaemia
Delay makes infective complications worse
Commence chemotherapy immediately
AML(acute myeloid leukaemia)-> strong IV chem in short sharp bursts
Young patients have better prognosis as can tolerate chemo better
ALL-> mix of strong chemo and persisting milder tablets to prevent relapse
Acute lymphoblastic leukaemia
Commonest malignancy in children -> peaks at 4-5 year
May present with cytopenias or chest masses
90% can be brought into remission with induction chemo
85% cured
Higher relapse rate in older children
ALL
Chronic leukaemia
Adults over 40 mainly
Present with high white cells but not usually bone marrow failure
Chronic myeloid leukaemia
Presents with-> high WBC, Splenomegally, priapism
Adults between 30-40
Due to t(9:22)-> Philadelphia chromosome
75% transform in to AML rest to ALL
Treatment involves specific inhibition of this tumour cell specific enzyme by IMATINIB
Chronic lymphocytic leukaemia
30% of all leukaemia
Mainly over 50’s
Proliferation of small mature lymphocytes, majority B cell
Majority of patients die of unrelated causes
Treatment only required for symptoms
Reproductive rate is less than normal blood cells, but defective apoptosis means they don’t die normally
Presents on routine FBC or lymphadenopathy
Diagnosing CLL
High lymphocyte count
Smudge cells on blood film
Appropriate cell surface markers on lymphocytes
Hypogammaglobulinsemia-> low anti body levels common association but not diagnostic
May have lymphadenopathy, Splenomegally
May have immune disturbance
May have marrow suppression
CLL prognosis
Binet staging
Stage A-> lymphocytosis only-> 10 years
Stage B-> lymphocytosis and 3 areas lymphadenopathy-> 5 years
Stage C-> lymphocytosis, LN, hb 2 years
Non Hodgkin’s lymphoma
Neoplastic proliferation of B, T lymphocytes or histiocytic cells
Starts in lymph nodes, 30% occur outside
Spreads to spleen, liver and bone marrow
Nodal-> originate in lymph nodes
Extra nodal-> originate in specialised groups of lymphoid cells
More common in elderly
Viruses, chemical exposure, sunlight exposure?
Subtypes of lymphoma
1) slow growing indolent B cell lymphoma 50% of cases
2) rapid growing, aggressive B cell lymphomas 30%
3) slow growing T cell lymphomas 10%
4) rapid growing T cell 10%
B lymphoma symptoms
Usual clue
Weight loss > 10% in 6 months
Drenching night sweats
Fevers >38
Hodgkin’s lymphoma
Technically a subtype of non Hodgkin’s
Related to EBV infection
Neoplastic proliferation of an atypical from of lymphoid cell
Two age peaks-> teens and elderly
Presents with B symptoms and contagious nodal spread
Bulk of the tumour is normal white cells
Diagnosis
Almost impossible to say mystery illness is not lymphoma
History of fever, weight loss, night sweats
Lymphadenopathy on examination/scan
Biopsy is vital
CT scan used to stage tumour extent and response to therapy
Immunosecretory disorders
Myeloma Asymptomatic myeloma Monoclonal gammopathy of uncertain significance Localised plasmactyoma Walden storms macroglbulinaemia Cold haem agglutinin disease Amyloidosis
Normal antibodies
IgD-> expressed by newly mature B lymphocytes
IgM-> clears pathogens from circulation
IgG-> clears pathogens from tissue fluid
IgA-> prevents pathogens invading mucosal surfaces
IgE-> protects against
Para proteins
Normal plasma proteins can be analysed by electrophoresis
Normal antibodies occur on a polyclonal smear
Para protein-> monoclonal antibody, causing a spike in the normal globulin pattern
Urine para protein-> isolated light chains-> bence jones protien
Myeloma
Tumour of plasma cells
1) plasma cells in marrow
2) detectable paraprotien in blood or urine
3) lytic lesions on skeletal survey
Bone pain
Abnormal plasma cells retrain the capacity to produce antibodies
Abnormal amounts of antibody normally IgG-> linked in to chains-> electrophoresis
-> increased blood viscosity
-> free light chains deposit in tissues
Diagnosing myeloma
Disease of the elderly Anaemia High ESR Back pain Unexplained renal failure Unusual fractures Poor prognosis
Waldenstorms macroglbulinaemia
Disease of the elderly
IgM paraprotien
Marrow shows sparse infiltrate of lymphoplasmacytoid lymphocytes
Health risk is hyper viscosity of blood
If IgM has haemolytic activity-> cold haem agglutinin disease-> haemolytic anaemia
Hyper viscosity syndrome
Due to excess plasma protiens or extreme Polycythaemia
Blood is sticker-> doesn’t flow so easily
-> fatigue, head aches, confusion
May progress to stroke, heart attack
Anaemia lowers viscosity so correcting with transfusion is dangerous
Plasmapheresis to remove protein
Leukostasis has similar presentation but due to high WBC
Myelodysplasia
Disordered maturation of blood cells in bone marrow
Any or all cell lines can be effected
Easy to diagnose if chromosome abnormality or severe
Hard to distinguish from reactive marrow changes
Can progress to acute leukaemia
