Week 4 Flashcards
T or F
Chronic Myeloid Leukaemias originate from a single abnormal haemopoetic stem cell.
True
Chronic Myeloid leukaemia is from what type of lineage?
Granulocyte
What is increased in the presence of a Chronic Myeloid Leukaemia?
Increase myeloid cells, Erythrocytes and platelets
Marked myeloid hyperplasia in bone marrow
According to WHO Classification what are the 4 predominant Myeloproliferative neoplasm disorders?
- Chronic myeloid leukaemia
- Polycythemia Vera
- Primary myelofibrosis
- Essential thrombocythaemia
Chronic myeloid leukemia has a preponderance of immature cells of which granulocyte lineage?
neutrophil
List some clinical findings of Chronic Myeloid Leukemia.
- Frequent infections
- Anaemia
- Bleeding
- Splenomegaly
- Fatigue
- Abdominal discomfort
- Weight loss
T-F : Philadelphia chromosome is found on myeloid, monocytic, erythroid, megakaryocytic, B-cells and sometimes T-cell
T
This is a description of the peripheral blood of what type of leukemia? • High WCC that can exceed 300 x 109/L • Dramatic left shift • Promyelocytes, myelocytes, bands • Occasional blast • Thrombocytosis is common • NRBC rare
Chronic Myeloid Leukemia
What other abnormal findings are likely with CML?
- High uric acid
- High cell turnover, breakdown of nucleic acids
- Secondary gout, uric acid stones in kidneys
- High lactate dehydrogenase (LDH)
- Intracellular enzyme
- Reflects high rate of cell turnover
- Vascular stasis due to high WCC
List some therapies for CML
- Bone marrow and stem cell transplantation
* Synthetic proteins that bind the abnormal BRC/ABL protein
ABL = Chromosome \_\_\_ BCR = Chromosome \_\_\_\_
9, 22
Polycythemia vera has Increases in all components of _____ origin in peripheral blood: (3)
myeloid
• Erythrocytes
• Granulocytes
• Platelets
Summarise Polycythemia Vera peripheral blood films
Peripheral blood • Elevated • Hb, RCC, Hct • Can be elevated • WCC, Platelet count, Blood film • Normochromic normocytic • Normal maturity of leucocytes and platelets
What are the 2 phases of PV?
stable phase and spent phase
What are the features of the spent phase of PV?
- Splenomegaly, pancytopenia
- May develop marrow fibrosis
- Tear drop poikilocytes
- Ineffective haematopoiesis
What can be used as a supportive treatment for PV?
Phlebotomy
What are some examples of inappropriate erythropoietin production?
- Renal disease e.g. carcinoma
* Tumors e.g. fibromyoma and liver carcinoma
What are some complications associated with PV?
15% of patients progress to acute leukaemia.
Some chemotherapy’s can increase risk
phlebotomy may increase risk of thrombosis and bleeding
What are some causes of apparent polycythaemia or pseudopolycythaemia due to plasma
volume contraction
• No change in total RBC mass
- Stress
- Cigarette smoker or alcohol intake
- Dehydration
- Plasma loss
- E.g. burn injury
WHO classification requires 4 major criteria for essential thrombocytosis:
- PCT > 450 x 109/l
- Bone marrow
• Significant increase in megakaryopoiesis
• In absence of increase in erythropoiesis, granulopoiesis
• No left shift in granulocytes
• Large mature-looking megakaryocytes - Cannot meet the criteria of other neoplasms
- Abnormal genetic markers or absence of reactive thrombocytosis
Summarise the clinical presentation of essential thrombocythaemia
50-60yr olds
Sometimes due to vascular occlusion or bleeding in GI and respiratory tract
50% splenomegaly
Increased collagen production in bone marrow with Increase in circulating haemopoietic cells
is
Myelofibrosis
Usual age of patients diagnosed with Myelofibrosis.
• Usually over 60 years
What are some possible general presentations of Myelofirbrosis
- Fatigue
- Weakness
- Shortness of breath
- Palpitations
- Weight loss
- Discomfort associated with splenomegaly
What are some bone marrow features of myelofibrosis?
- Fibrosis
- Hypercellularity
- Increased megakaryocytes
- Enlarged, pleomorphic nuclei
What are some peripheral blood features of myelofibrosis?
- Immature granulocytes
- Normoblasts
- Teardrop red cells
- Other bizarre red cell shapes
What disease is this describing?
• Heterogeneous collection of haemopoietic stem cell disorders affecting older adults
• Underlying ineffectiveness of haemopoiesis
• Results in dysplasia of bone marrow precursors
• Peripheral cytopaenias
• Acquired clonal disorders with defects in maturation:
• Erythroid
• Myeloid and/or
• Megakaryocyte
Myelodysplastic syndrome
Myelodysplastic syndrome is haemopoietic stem cell mutation. What are some possible causes of this mutation?
- Radiation
- Chemical
- Viral
- Smoking?
List so typical peripheral blood findings in Myelodysplastic syndrome
- Oval macrocytes
- Hypochromic microcytes
- Dimorphic blood picture
- Basophilic stippling
- RNA
- Howell-Jolly bodies
- Abnormal granulation of neutrophils
- Hypersegmentation Hyposegmentation
- Nuclear rings
List some bone marrow findings in myelodysplastic syndrome
• RBC precursors • more than 1 nucleus • Abnormal nuclear shapes • Ringed sideroblasts • Accumulation of iron in peri-nuclear mitochondria • Iron stain
What morphology would be expected of platelets in myelodysplastic syndrome?
- Giant platelets
- Abnormal granulation
- Circulating megakaryocytes
Acute leukaemias have at least _____ % blasts in the BONE MARROW
20%
List some lab/clinical findings in Acute Lymphoblastic Leukaemias
- Only 50% of patients have leucocytosis
- Many do not have circulating lymphoblasts
- Common findings:
- Anaemia
- Neutropaenia
- Thrombocytopaenia
- What symptoms might you expect?
- Lymphadenopathy common
- Splenomegaly, hepatomegaly may be seen
- Bone pain from invasion of periosteum
- Malignant cells in meninges, testes, ovaries
What are the two major groups of Acute lymphoblastic leukaemia?
B lymphoblastic leukaemia
T lymphoblastic leukaemia
What are the two morphologic types of Acute lymphoblastic leukaemia?
small lymphoblast
large lymphoblast
What is the most common leukaemia in children under 1yr old?
Acute myeloid leukaemia
What is the most common leukaemia in adults?
Acute myeloid leukaemia according to slides but the internet says Chronic Lymphocytic Leukaemia is??
90% of Acute myeloid leukaemia patients have _______ in their peripheral blood
myeloblasts
Which leukaemia has auer rods as a characteristic?
Acute myeloid leukaemia - • Needle shaped clumps of primary granules
Leukaemia Acute lymphoblastic Blast size Cytoplasm Chromatin Nucleoli Auer rods
Small Scant Dense Indistinct No
In Acute myeloid leukaemia describe the below: Blast size Cytoplasm Chromatin Nucleoli Auer rods
Large Moderate Lacy Prominent 50% of patients
In acute myeloid leukaemia symptoms reflect decreased production of normal marrow elements - list some
- Anaemia
- Neutropenia
- Thrombocytopenia
- DIC
- Infiltration of malignant cells into gums, mucosa, skin
A metabolic complication of malignancy that invovled the breakdown products of dying tumour cells is called
Tumour lysis syndrome
What is the difference between secondary polycythaemia and relative polycythaemia?
Relative polycythaemia - caused by smoking, stress,dehydration, plasma loss
Secondary polycythaemia - is polycythaemia secondary to:
• Renal disease e.g. carcinoma
• Tumors e.g. fibromyoma and liver carcinoma
What could cause a ‘normal’ compensatory increase in erythropoietin
- High altitude
- Pulmonary diseases
- Heart diseases eg- cyanotic heart disease
- Abnormal hemoglobin- High affinity Hb
- Heavy cigarette smoker
Outline the cause of and lab findings in tumour lysis syndrome
Increase in Uric acid Potassium Phosphorous Decrease in Calcium
