Haematological Malignancies 2 Flashcards
What is Hodgkin lymphoma
(5)
Clonal B-cell malignant that develops within the lymphatic system
The malignant Reed-Sternberg Cell typically has a bilobed nucleus that gives an “owls eyes” appearance
Diagnosis, excisional lymph node biopsy
Spreads in an orderly fashion to adjacent nodes
Painless lymphadenopathy, constitutional “B” symptoms (fever, night sweat, weight loss), pruritus, hepatosplenomegaly
Write about Reed-Sternberg
Cell may contain more than one nucleus
Presence in peripheral blood indicate advanced stage of disease
What are the three main types of lymphoma
NK cell
B cell
T cell
What cells are involved in leukaemia
Neutrophil
Monocyte
RBC
Megakaryocyte
Write about acute lymphoblastic leukaemia
Usually occurs before 14 years of age
Peak incidence between 2 and 9 years
Less common in adults -> peak at about 50
Low RCC, Hb, Hct, platelet count, low normal or high WBC count
Accumulation of malignant, poorly differentiated lymphoid cells within the Bone marrow, peripheral blood and 20% of the time at extramedullary sites
Chromosomal aberrations are the hallmark of ALL, but are not sufficient to generate leukaemia.
Characteristic translocations
More recently, a variant with a similar gene expression profile to Ph positive ALL but without the BCR-ABL1 rearrangement has been identified (poor prognosis)
Comment on the prevalence of ALL
Usually occurs before 14 years of age
Peak incidence between 2 and 9 years
Less common in adults -> peak at about 50
What are the clinical findings of ALL?
(5)
Low RCC
low Hb
low Hct
low platelet count
low normal or high WBC count
Increased lymphoblast
What exactly happens in ALL
Accumulation of malignant, poorly differentiated lymphoid cells within the Bone marrow, peripheral blood and 20% of the time at extramedullary sites
What causes ALL
Chromosomal aberrations are the hallmark of ALL, but are not sufficient to generate leukaemia.
Characteristic translocations
More recently, a variant with a similar gene expression profile to Ph positive ALL but without the BCR-ABL1 rearrangement has been identified (poor prognosis)
Write about the clinical findings of bone marrow aspirate in ALL
Hypercellularity
High lymphoblasts greater than 20%
Write about acute myeloid leukaemia
Most common type of acute leukaemia in adults
Accounts for 30% of all leukaemia
300-400 cases of AML in Ireland/year
Outcome in patients with AML ranges from death within a few days of beginning treatment to likely cure
The major reason patients are not cured is resistance to treatment, often manifested as relapse from remission, rather than, even in older patients, treatment related mortality, whose incidence is decreasing
Knowledge of the pre-treatment mutation statis of various genes has improved our ability to assign initial treatment and, of particular importance, knowledge of whether patients apparently in remission have measurable residual disease should influence subsequent management
What is the most common type of leukaemia in adults
Acute myeloid leukaemia in adults
Accounts for 30% of all leukaemia in adults
300-400 cases per year
Comment on the severity of AML
One of the harder leukaemias to beat
Outcome in patients with AML ranges from death within a few days of beginning treatment to likely cure
Why is AML so hard to cure
The major reason patients are not cured is resistance to treatment, often manifested as relapse from remission, rather than, even in older patients, treatment related mortality, whose incidence is decreasing
Knowledge of the pre-treatment mutation statis of various genes has improved our ability to assign initial treatment and, of particular importance, knowledge of whether patients apparently in remission have measurable residual disease should influence subsequent management
How is AML classified
M0 -> M7
What is M0 AML
Undifferentiated acute myeloblastic leukaemia
5%
What is M1 AML
Greater number of myeloblasts with less than 10% granulocytic differentiation
What is M2 AML
Myeloblasts in great number with granulocytic differentiation >10%
NSE < 20%
What is M3 AML
Promyelocytes that are hyper granular with many Auer rods on CAE or Wright-stain and variant form cells with reniform nuclei, multilobed or bibbed, primeval cells with multiple Auer rods or relative scarcity of Hypergranular promyelocytes
What is M4 AML
> 20% but <80% NSE-butyrate positivity in Monocytic cells
What is M5 AML
Monocytic cells with >80% NSE positivity
a. Monocytic differentiated
b. Monocytic, differentiated
What is M6 AML
> 30% myeloblasts with more than 50% erythroblasts eliminating the erythrois cells