Chronic Myeloproliferative Disorders and Chronic Myeloid Leukaemia Flashcards Preview

Clinical Pathology > Chronic Myeloproliferative Disorders and Chronic Myeloid Leukaemia > Flashcards

Flashcards in Chronic Myeloproliferative Disorders and Chronic Myeloid Leukaemia Deck (29):
1

Clonal proliferation of bone marrow stem cells usually leading to excess production of one or more haemopoietic lineage

Chronic myeloproliferative disorders

2

3 types of chronic myeloproliferative disorders

Polycythaemic vera- RBCs
Esstential thrombocytosis- platelets
Idiopathic myelofibrosis-reactive fibrosis of bone marrow and extramedullary haemopoeisis in the liver and spleen

3

What percentage of chronic myeloproliferative disorders transform to acute leukaemia

10%

4

Immature RBCs released into the blood following a bleed

Reticulocytes

5

Symptoms of polycythaemia vera

Stroke, MI, splenomegaly, gangrene, gout (everytime you breakdown cells, uric acid released into the blood), plethoric face

6

Lab findings of polycythaemic vera

increased haematocrit

7

Haematocrit

RBCs relative to plasma

8

3 types of polycythaemia vera

Primary e.g. PV
Relative e.g. not enough plasma- alcoholics, D&V
Secondary e.g. COPD, altitude, EPO production tumours, renal disease

9

What is the distinguishing features between primary and secondary polycythaemia

If EPO is high, likely to be secondary
JAK2 mutation, likely to be primary

10

What is the result of the JAK2 mutation

Erythrocyte replication cycle constantly on

11

Persistent elevation of peripheral blood platelet count as a result of increased marrow platelet production in the absense of a systemic cause

Thrombocytosis

12

Clinical features of thrombocytosis

Thrombosis (gangrene, cerebral, coronary arteries)
DVT
Superficial thrombophlebitis
Headaches, visual disturbance
Splenomegaly (50%)

13

Lab findings for essential thrombocytosis

Platelet count >405x109/L
JAK2 mutation in 50%
Hypercellular bone marrow and increased numbers of megakaryocytes
CALR mutation in 45%

14

What is the effect of the CALR mutation

Cell signalling protein found in myeloid progenitors- may activate cell signal pathways

15

Treatment for thrombocytosis

Antiplatelet treatment
Interferon- inhibitor of megakaryocytes
Hydroxycarbaminde- decreased DNA synthesis

16

Prognosis for idiopathic myelofibrosis

5 years- much worse than the others

17

What are the 3 main characteristics of idiopathic myelofibrosis

Splenomegaly
Extramedullary haematopoiesis
Replacement of bone marrow with fibrosis

18

Clinical features of idiopathic myelofibrosis

Massive splenomegaly- left hypochondrial pain
Fever, weight loss, pruritis, hepatomegaly and night sweats
Abdominal swelling caused by portal hypertension

19

CML is characterised by excess production of what cells?

Myeloid cells- neutrophils and granulocytes

20

Describe the genetic mutation in CML

Leukaemic cells have t(9;22) translocation known as the philadelphia chromosome (BCR-ABL gene fusion forms TK gene that tells cells to proliferate)

21

Clinical features of CML

Occurs at all ages
Weight loss, night sweats, itching, left hypochondrial pain, gout, splenomegaly

22

Lab features of CML

Leucocytosis

23

Treatment for CML

Imatinib (Gleevec) inhibits TK encoded by BCR-ABL gene

24

B cell clonal lymphoproliferative disease- lymphocytes accumulated in the blood, bone marrow, lymph nodes and spleen

Chronic lymphocytic leukaemia

25

What age does CLL typically present?

Over 72 yrs

26

Clinical features of CLL

Lymphadenopathy, night sweats, weight loss due to bone marrow failure, splenomegaly
Hypogammaglobulinaemia-predisposed to infection
Autoimmune haemolytic anaemia

27

Lab findings of CLL

Increased peripheral blood lymphocytes. Weak expression of IgM

28

Peripheral blood cytopenias, mostly frequently affecting more than 1 lineage, usually in association with a hypercellular marrow indicating ineffective haemopoiesis

Myelodysplasia

29

Lab findings for myelodysplasia

Macrocytic anaemia
Neutropenia
Hypercellular bone marrow