Haem 2 Flashcards
What are leukaemia’s?
What does it result in?
Malignant neoplasms of haemopoietic stem cells which result in diffuse replacement of bone marrow and normal blood precursor cells by neoplastic cells.
Bone marrow failure leads to anaemia, neutropenia and thrombocytopenia
Outline the classification of Leukaemia’s
Classification based on the Cell Line and the Cell Maturity of affected cells.
Cell line will either be Myeloid or Lymphoid.
L’ is Acute (immature) if more than 50% cells present on clinical presentation are (myeloma/lympho) blasts.
Chronic (mature) if less than 50pc
What two investigations are carried out to diagnose leukaemia?
Blood film and Bone marrow check.
From blood film we are interested in WCC and percentage of blasts present.
From marrow check we are interested in hypercellularity and number if blast cells.
What are the 3 main prognostic indicators from investigations into Leukaemia’s?
Type of Leukaemia
Cell phenotype
Presence of chromosomal abnormalities
Generally, the more normal the picture looks the better.
Describe the potential aetiological factors for leukaemia
Viruses (such as HTLV) are the most likely for most pt.
Other factors:
- Ionising radiation
- Industrial chemicals (benzene, alkylating agents)
- Genetic Factors
- Acquired Haematological disorders such as aplastic anaemia
What are the consequential s/s for leukaemia due to
- Marrow Infiltration
- Tissue infiltration
Marrow infiltration causes lack of prod of normal cells –> Thrombocytopoenia, Neutropenia, Anaemia
TCPoenia –> bleeding and bruising/petechiae (capillaries break down and bleed into skin)
Neutropoenia –> fever and increased infection risk inc abnormal response to infection
Anaemia–> pallor/malaise
If tissue infiltration occurs, cells commonly accumulate in areas causing
- lymphadenopathy
- hepatosphlenomegaly
- CNS
Compare Acute v Chronic Leukaemia
Acute affects younger patients. In acute, immature blasts cells make up >50pc because cells lose ability to differentiate but retain ability to replicate (monoclonal disorder). In chronic, error kicks in later in maturation process so affects differentiated cells. Blast pop <50% pop.
Compare ALL and AML.
What are the symptoms unique to each one?
Give Management of both.
ALL has a younger peak incidence (4/5 years) whereas AML affects older patients
ALL–> bone+joint pain, testicular swelling
AML–> leukostasis, priapism, gingival hypertrophy
ALL management:
In children remission induced using non-myelosuppressive chemotherapy (60%< cure rate)
In Adults combination chemo used. 20% cure rate. Prophylactic CNS tx. 2 years maintenance therapy increases survival
AML management
4-5 intensive chemo courses (each 5-10 days) delivers 80% cure rate. but, 15% cases resistant to chemo’. No maintenance therapy required.
Compare the main clinical features of CLL to CML
CLL- recurrent infections, lymphadenopathy, splenomegaly, pancytopoenia
CML- early stage asymptomatic, later stages –> hypermetabolism, leukostasis, hyperuricaemia
Give details about CLL.
Target market and Tx.
Affects elderly, 2:1 m:f ratio.
Tx
Asymptomatic pt do not require tx. and due to target market, 30% of early stage pt die of other shit anyway cos old.
If they progress to later stage, give chemo which is normally quite effective. If they are young and have poor prognosis, consider bone marrow transplantation.
Death from CLL normally due to infection or bm failure.
Give details about CML
onset around 40-50 yrs. Slight male predominance. Relatively benign.
90% of CML cells have philadelphia chromosome. This is a balanced transolocation between 9 and 22 resulting in an oncogene with tyrosine kinase activity.
Lead to first targeted therapy- Imatinib (a tyrosine kinase inhibitor)
What is lymphoma?
What are the two groups of lymphoma?
On what basis are they differentiated?
Lymphoma is solid tumours found in lymph nodes, MALT, spleen and bone marrow.
Lymphoma can either be Hodgkin’s where cancer cells are nodal and continguous, is not associated with immunodeficiency and has good outcome. Or can be Non-hodgkin’s where cells are extranodal and non-contiguous, disease has a more variable outcome and is associated with immunodeficiency.
Lymphoma is Hodgkin’s if the Reed-Sternberg B Cell is present.
Describe Hodgkin’s Lymphoma Aetiology, Clinical features
What is meant by ‘B Symptoms’ and what are they?
Aetiology associated with Epstein-Barr Virus.
Painless rubbery lymphadenopathy (cervical and supra-clavicular)
Anorexia/fatigue
Mediastinal Involvement
Itchy red rash
Constitutional B Symptoms worsen prognosis
- fever, night sweats, weight loss
Describe the Ann-Arbor staging system for Hodgkin’s Lymphoma
I- single LN region
II- two LN regions
III- LN regions either side of diaphragm, may include spleen (IIIs)
IV- widespread disease outside of lymphatic tissues
Describe investigations and tx for Hodgkin’s Lymphoma
Investigations FBC- normochromic normocytic anaemia Elevated ESR Chest X Ray LN Biopsy- Reed sternberg cells
Tx
Early stage –> Radiotherapy
As advances –> combo chemo
Prognosis depends on Ann-arbor staging. B symptoms worsen it greatly
