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?