Hdp 3 Session 2 Haem And Lymph Do

Front 1

What are myeloproliferative disorders?

Back 1

A group of disorders caused by uncontrolled clonal proliferation of one or more of the stem cells lines in the bone marrow. Include PV, essential thrombocythaemia, myelofibrosis and Chr Myeloid Leukemia

Front 2

Define Polycythaemia

Back 2

An increase in haemoglobin, PCV and RBCs. May be relative (low Plasma volume, normal RBC mass) or absolute (Increase RBC mass).
Relative may be acute and due to dehydration. Absolute is distinguished by Red Cell Mass estimation and is caused by Primary (PCV) or Secondary (due to hypoxia or inappropriate increase in EPO secretion).

Front 3

What is Polycythaemia Vera

Back 3

A malignant proliferation of erythrocytes and other cells in the bone marrow. The erythroid progenitor offspring avoid apoptosis. There is an xs proliferation lead to hyperviscosity and thrombotic complications. Affects pts >60yrs.

Front 4

What are the signs of PCV

Back 4

insidious onset, >60yrs, tiredness, depression, vertigo, tinnitus, visual disturbances, hypertension, angina, intermittent claudication, gout, thrombosis, splenomegaly, hepatomegaly.

Front 5

What investigations do you do for polycythemia vera (primary)

Back 5

FBC: increase RCC, Hb, PCV, hemocrit, B12 levels
Erythroid hyperplasia in Bone Marrow

Front 6

What is treatment for polycythemia vera

Back 6

Aim to keep hemocrit levels <0.45 to decrease risk of thrombosis (eg: venesection, hydroxyurea, , allopurinol to block uric acid production.

Front 7

What is secondary polycythaemia?

Back 7

Not a myeloproliferative disorder.
The abnormal increase in PCV occurs secondary to another disease or condition. Either appropriate (response to anoxia) or inappropriate (response to tumours).

Front 8

What is prognosis of Polycythemia

Back 8

Primary develop mylofibrosis in 30% cases and AML in 5%, also uncontrolled at high risk of haemorrhage or thrombosis following surgery.

Front 9

Define haemostasis

Back 9

Complex process depending on interactions between vessel wall, platelets, and coagulation and fibrinolytic mechanisms to stop blood flow through damaged vessels.

Front 10

What steps make up normal haemostasis

Back 10

1. Vessel Wall integrity
2. Platelets
3. Coagulation pathways
4. Fibrinolysis and factors limiting coagulation.

Front 11

What is the role of platelets and the clotting factor cascades and normal anticlot mechanisms in the body

Back 11

See page 2.4 workbook

Front 12

Outline DVT
patho, clinical features, invest, treatment,

Back 12

Risk factors, age, pregnancy, OCP, Surgery, past DVT
Patho: Thrombus forms in a deep vein and any inflamm of vein wall is secondary; most often in deep vein of calf
CF: calf warmth/tenderness/swelling/redness. Mild fever, pitting oedema, + Homan sign.
Invest: D-dimer blood test, venography,
Tx: Anticoag therapy, bed rest, pressure stockings, warfarin for 3 months

Front 13

Outline thrombophilia
Patho, CF, Invest, Tx, Prognosis

Back 13

Def: inherited or acquired defects of haemostasis leading to predisposition to venous or arterial thrombosis.
Causes/associations - recurrent VT, unusual venouse thrombosis, recurrent miscarriages, arterial thrombosis without arterial disease.
Tx: heparin, warfarin

Front 14

Outline varicose veins

Back 14

Def: a dilated tortous vein
Aet: incompetence of perforator veins allows for reflux of blood from deep system to superficial vein, thin walled tributary veins become elongated and engorged, values are imcompetent and distension spreads.
Aggravating factors: obesity, pregnancy, constipation, standing.
cf: unsightly varices, discomfort, ache, tiredness and swelling of limb.

Front 15

How are haemostatic systems investigated

Back 15

Is bleeding suggestive of Vascular or platelet defects - easy bruising, bleeding into skin; Coagulation defect - haemarthrosis, muscle haemotoma; Continued bleeding after injury or surgery?

Front 16

What are the causes and clinical presentations of vascular disorders (eg henoch-schonlein purpura)

Back 16

Def: bleeding d.o characterised by easy bruising and bleeding into skin or mucous membranes. Causes: vascular wall defect, congenital, acquired, allergic, drugs. HSP is a small vessel vasculitis, which presents with purple nodules which done disappear on pressure (intradermal bleeding).

Front 17

What are the causes and clinical presentations of platelet disorders (eg thrombocytopenia)

Back 17

A platelet count below normal reference range for the age and gender of the pt. Aet: reduced platelet prod or excess peripheral destruction by spleen, autoimmune.
CF: easy bruising, purpura, regular epitaxis, menorrhagia
Invest: FBC: low platelet count, platelet autoantibodies
Tx: Acute - give platelet transfusion,, steroid therapy to reduce inflammatory response. Splenectomy cures 80%.

Front 18

Define haemophilia (see pg 2.8 of workbook)

Back 18

Def: a congenital coagulation disorder usually due to a xu of one coag factor. Haem A: Factor VIII, X-linked recessive. Haem B: Factor IX, X-linked recessive. Von Willebrand's Disease (vWF); Xu of vWF and carrier of clotting factor VIII. There are 3 types.

Front 19

Outline causes and CF of acquired disorders, eg disseminated intravascular coagulation

Back 19

Acq Bleeding Disorders - Vit K xu, Liver Disease, DIC
Disseminated Intravascular Coagulation = life threatening condition involving xs blood clotting and xs bleeding at same time throughout whole body. So many clotting factors are removed by widespread clotting that too few remain to permit clotting. Clots cause ischaemia and necrosis = multisystem organ failure.
Patho: a) release of tissue factor into circulation triggers extrinsic coag pathway b) widespread injury to endothelial cells, which promotes platelet aggregation and activated both ext and intrinsic pathways c) activation of leukocytes to release cytokines.
Cause: infection, hypoxia, low blood flow, trauma, hypotensions, hemolysis.
CF: no bleeding to widespread haemorrhage.
Invest: High FDPs including D-Dimer.
Tx: treat cause, transfusion of RBC, platelets and plasma

Front 20

What is ET

Back 20

Essential thrombocythaemia. Microvascular thrombosis occurs (sim to PCV) but only platelets.
Sx: bruising, bleeding, systemic sx

Front 21

What are s/s of haemophilia

Back 21

Prolonged or severe hemorrhage following minor tissue trauma. Hematoma, hemarthrosis. Haematuria may occur.