Haem Cancers and MPD's Flashcards
Define leukaemia
A group of haemtological malignancies in which there is malignant monoclonal expansion of a single cell line
Name a malignant proliferation of either B or T cells that results in arrested maturation and uncontrolled proliferation of immature precursor cells called blasts within the bone marrow
Acute Lymphoblastic Leukaemia
Which leukaemia is known to infiltrate tissues outside the bone marrow and name some sites
ALL
CNS, Lymph, testes
ALL is the most common cancer of which age group
Childhood
Which three blood abnormalities do leukaemia’s present with
Anaemia
Thrombocytopenia
Neutropenia
If leukaemia’s can present with leukocytosis, why are the patients still prone to infection
Although WCC count is high, the cells are non functioning, with this along with low neutrophils creating susceptibility to infection
What is the difference in chemotherapy for ALL vs AML
ALL –> long duration of chemotherapy with several agents
AML –> intensive chemotherapy regime
Describe the pathophysiology behind Acute Myeloid Leukaemia
monoclonal uncontrolled proliferation of immature blast cells of the myeloid lineage
Which leukaemia can characteristically infiltrate the gums
Acute promyelocytic leukemia
What is the most common acute leukaemia in adults
AML
What specific blood film sign is diagnostic of AML
Auer rods
Which cancer can be treated using all trans retinoic acid
Treatment of Acute Promyelocytic Leukemia (APL)
Which blood cancer is associated with Autoimmunie Haemolytic Anaemia and Idiopathic thrombocytopenic purpura
Chronic Lymphocytic Leukaemia
Which cells can be seen in Chronic Lymphocytic Leukaemia
Smear/Smudge cells
What is Richter’s Transformation
B cell CLL changes to Diffuse B cell Lymphoma
What kind of Haemolytic Anaemia is CLL associated with
Warm (IgG)
What disease is Cold haemolytic anaemia (IgM) associated with
Lymphoma
Electrophoresis of Chronic Lymphocytic Leukemia will show what
Hypogammaglobulinemia
Which malignancy is associated with a 9,22 translocation and what is this called
Chronic Myeloid Leukemia
Philadelphia chromosome
Is CML is BCR-ABL 1 positive or negative
Positive
What are the three stages of CML
Chronic
Accelerated
Blast crisis
What is the result of the BCR-ABL 1 gene in CML
Tyrosine kinase causes abnormal stem cell proliferation
Why might someone with CML get gout
High cell turnover
How will bone marrow look on biopsy in CML
Hypercellular
What drug can be used in the management of CML
Tyrosine Kinase Inhibitor
Imatanib
What are the 3 MPD’s which are BCR-ABL 1 negative
Polycythaemia Rubra Vera
Essential Thrombocythaemia
Myelofibrosis
What is a buzzword appearance on the blood film of someone with Myelofibrosis
Leucoerythroblastic film with teardrop poikilocytes
When should you consider an MPD at play
High Granulocyte count
High Red cell count / haemoglobin
High Platelet count
Eosinophilia/basophilia
Splenomegaly
Thrombosis in an unusual place
Which symptom is fairly specific to Polycythaemia Rubra Vera
Aquagenic Itch
Which mutation should you look for in someone presenting with hyperviscosity symptoms and an itch after a bath
JAK2 mutation status
looking for PRV
What are some causes of secondary polycythaemia
COPD, heavy smoking), increased EPO production (renal and hepatic carcinoma).
What does the JAK2 mutation cause in Polycythaemia Rubra Vera
Results in loss of autoinhibition of erythropoiesis
How is Polycythaemia Rubra Vera treated
o Low risk: venesection + aspirin.
o High risk: Hydroxycarbamide + aspirin.
High risk is if > 60 or previous thrombosis
What is Essential Thrombocythaemia and what causes it
Clonal proliferation of megakaryocytes, which results in excessively high levels of abnormal functioning platelets
If the JAK 2 mutation is not seen in Essential Thrombocythaemia, which mutation may be present
CALR –> seen in those who are not JAK2 +.
What does the increased amount of abnormal platelets result in, with regards to Essential Thrombocythaemia
o Thrombosis.
o Bleeding –> due to utilisation of all VWF
Erythromelalgia + aquagenic itch = what diagnosis
Polycythaemia Rubra Vera
How is Essential Thrombocythaemia treated
Aspirin + Cytoreductive therapy to control proliferation
hydroxycarbamide
What is Idiopathic Myelofibrosis
Myelofibrosis occurs as a consequence of megakaryocyte proliferation, which can be idiopathic or secondary to PRV or essential thrombocytosis.
The increase in megakaryocytes is associated with an increased release of platelet derived growth factor, which causes stimulation of fibroblasts and bone marrow fibrosis.
Fibrosis of the marrow causes marrow failure, which is compensated for by extra medullary haematopoiesis in the liver and spleen
What does leukoerythroblastosis mean and which disease is it seen in
Both myeloid and erythroid precursors seen in the blood
Seen in myelofibrosis
Which diseases can Myelofibrosis occur secondary to
Polycythaemia Rubra Vera or Essential Thrombocytosis
What are the treatment options for Myelofibrosis
Supportive care, Allogeneic stem cell transplantation Splenectomy(CONTROVERSIAL)
JAK2 inhibitors
Name a JAK2 inhibitor which could be used for myelofibrosis treatment
Ruxolitinib
What are lymphomas
A group of malignant proliferations of lymphocytes that accumulate in lymph nodes causing lymphadenopathy and can infiltrate the blood and other tissue –> spleen
What is the general presentation of a lymphoma
Lethargy. Fever. Night sweats. Itch – without rash Lymphadenopathy
What is the diagnostic investigation for Lymphoma
Excisional lymph node biopsy
Which cells are pathognomic of Hodgkin’s lymphoma
Reed-Sternberg cells CD30+
What is the most common subtype of Hodgkin’s lymphoma
Nodular sclerosing
Which type of lymphoma has a starry sky appearance on blood film and which gene mutation is it associated with
Burkitt’s Lymphoma
C-MYC
What is the most aggressive subtype of Hodgkin’s lymphoma
Lymphocyte depleted
What is a specific symptom of Hodgkin’s Lymphoma
Drinking alcohol causes pain in chest
Which lymphoma is associated with EBV infection
Hodgkin’s
Non-Hodgkin’s lymphoma has which CD marker on it’s cells
CD 20
How is Hodgkin’s lymphoma treated
Chemotherapy (Adriamycin, Bleomycin, Vincristine, Dacarbazine)
+ anti CD30 therapy (Brentuximab)
What is the most common Non-hodgkin’s lymphoma
Diffuse large B cell lymphoma
Extra-nodal disease is more common in which type of lymphoma
Non-Hodgkin’s
What is the standard treatment of Non-Hodgkin’s lymphoma
R Rituximab -> anti CD 20 monoclonal antibody. \+ C Cyclophosphamide. H Hydroxyduanrubicin. O Vincristine. P Prednisolone.
What is the most common type of myeloma
IgG
What is monoclonally produced in myelomas
Malignant plasma cells
What is a paraprotein
Monoclonal immunoglobulin
Purpura, Haemorrhages, Neuopathies, heart failure, stroke
What feature of haem malignancies could cause all these symptoms
Hyper-viscosity syndrome
What is the 1st line investigation for suspected myeloma
Immunoglobulin electrophoresis
What are the 1st and 2nd line investigations for suspected lymphoma
USS + node biopsy
Immunohistochemistry to classify subtype
What are the CD markers for B cells and T cells
B cells –> CD20
T cells –> CD30
Which subtype of Hodgkin’s lymphoma has the best prognosis
Lymphocyte rich
Which cell type is more commonly involved in Lymphomas
B cells > T cells
Which lymphoma classically affects the jaw
Burkitt’s Lymphoma
