Blood and lymphatic disordersdisorders Flashcards

Question

What are clotting disorders?

Answer 1

Problems in the body's ability to control how the blood clots i.e. too little clotting (bleeding disorders), or too much clotting (thrombophilia)

Answer 2

An umbrella term for deficiences in clotting factors, affecting the blood's ability to clot

Answer 3

It is usually inherited but can be due to spontaneous gene mutations

Answer 4

Haemophilia A - deficiency of clotting factor VIII Haemophilia B (Christmas disease) - deficiency of clotting factor IX Haemophilia C (Rosenthal’s) - deficiency of clotting factor XI

Answer 5

A common hereditary disease where there is a deficiency of Von Willebrand factor which causes slower clotting

Answer 6

Persistent bleeding from gums, repeated nosebleeds Petechiae – pinpoint rash from bleeding capillaries Bruises Haemoptysis Haematemesis Blood in faeces

Answer 7

Signs of PE and DVT or MI and strokes

Answer 8

Disseminated intravascular coagulation (DIC) is a rare but serious condition that causes abnormal blood clotting throughout the body's blood vessels. Often life-threatening condition involving both excessive bleeding and excessive clotting. It occurs as a complication of various primary problems which activate coagulation in the microvasculature throughout the body. (May be the result of excessive tissue injury or the release of tissue thromboplastin) Multiple thrombosis and infarctions occur which consume clotting factors and platelets and stimulate fibrinolysis. This leads to haemorrhage and hypovolaemic shock.

Answer 9

Sepsis Cancer Major trauma Serious obstetric complications (e.g. placental abruption)

Answer 10

Low plasma fibrinogen Thrombocytopenia Prolonged bleeding time Haemorrhage Petechiae Mucosal bleeding Vascular occlusions Respiratory impairment Neurological effects – seizures, reduced consciousness Acute renal failure.

Answer 11

An abnormally high number of red blood cells in the blood (increase in erythrocytes, granulocytes and thrombocytes), as a primary disease or secondary condition (usually associated with lung or heart disease or living at high altitude). Increased blood viscosity Risk of thrombosis Increased blood pressure Cardiac hypertrophy Spleen and liver enlargement Fatigue or weakness Headaches Dizziness Shortness of breath Visual disturbances Nose bleeds Bleeding gums Heavy menstrual periods Bruising

Answer 12

Over proliferation of certain blood cells e.g. polycythaemia and leukaemia

Answer 13

A group of neoplastic disorders involving the white blood cells - Hyper production of immature non-functioning leukocytes within the bone marrow, large quantities released into the circulation and they then infiltrate the lymph nodes, spleen, liver, brain and other organs as numbers increase.

Answer 14

Chronic leukaemia’s are more common in older people Acute leukaemia’s primarily occur in children and young adults

Answer 15

Acute leukaemia is characterised by a high proportion of immature cells Chronic leukaemia has a higher concentration of more mature cells, although their function is typically impaired.

Answer 16

Proliferation of white blood cells is so high that other cells are unable to be produced causing a reduction in RBCs and platelets - leading to signs of anaemia and increased/spontaneous bleeding. The increased pressure within the bone itself causes severe pain

Answer 17

Recurrent infections – unresponsive to treatment Severe haemorrhage Signs of anaemia Bone pain Weight loss Fever Spleen, liver, lymph node pain

Answer 18

Anaemia is a term for a number of disorders/diseases characterised by decreased haemoglobin content i.e. a reduction in production or an increase in destruction

Answer 19

Iron deficient anaemia

Answer 20

The body contains 4 grams of iron (normally) and 75% of this is in haemoglobin (normally), the rest is stored in reserve in the liver, bone marrow & spleen, plus a little circulating as it’s on it’s journey to the erythroblast. In women 1ml of blood contains 0.5 milligrams of iron, slightly less in men.

Answer 21

Decreased haemoglobin content due to low iron levels

Answer 22

Complete Blood Count - to look at haemoglobin and haematocrit levels. Iron level test Blood smear test - will look at the shape and age of the red cells. If they are small this is termed ‘microcytic’ and pale is termed ‘hypochromic’.

Answer 23

Microcytic

Answer 24

Hypochromic

Answer 25

Blood loss (usually chronic) – GI bleeds, menstruation++, peptic ulcers, haemorrhoids etc. Poor intake - pregnancy (in later stages of pregnancy the iron demand of the foetus is high), low iron diet Absorption problem - coeliac, colitis, bowel surgery etc.

Answer 26

Pallor – look conjunctiva, palms, buccal mucosa Glossitis Lethargy, fatigue Irritability – hypoxia SOB on exertion Koilonychia Degenerative changes to hair Tachycardia, palpitations, syncope Delayed healing Menstrual irregularities

Answer 27

A smooth tongue Iron is needed for new epithelial tissue maturing, iron deficient anaemia means a lack of availab;e iron to do this properly

Answer 28

Spoon shaped nails, can be a symptom of iron deficient anaemia - the pathophysiology around this has not been proven, but it is assumed to be low levels of O2 in the nail matrix or possibly to do with epithelial tissue maturation in the nail bed

Answer 29

Iron deficient anaemia can cause irregular periods. The mechanism is not fully understood but it may be that periods become irregular as the body does not want to let go of any red cells

Answer 30

Vitamin B12 (folate) deficient anaemia - a type of anaemia where the body is not producing enough healthy red cells

Answer 31

An autoimmune process where the immune system attacks the parietal cells of the GI mucosa causing a decrease in intrinsic factor production and thereby **redued B12 absorption** – without vitamin B12 the body cannot make healthy red blood cells. The exact cause like many automimmune diseases is unknown however: -There is a possible genetic link. -It is more common in older people and the parietal cells become less efficient and produce less intrinsic factor. -It is linked to other autoimmune disorders such as type 1 diabetes, primary Addison's disease etc. -People who have had stomach surgery where their parietal cells have been damaged or had a portion removed may also suffer with this

Answer 32

Pernicious anaemia is categorised by the presence of many large, immature, nucleated red cells circulating in the blood. (usually only new red cells have a nucleus and as they mature the nucleus disappears)

Answer 33

Similar to that of iron deficient anaemia (pallor, breathlessness, glossitis, fatigue, tachycardia etc.) plus: Nerve damage (paraesthesia in the extremities, clumsiness) Stomach problems as the stomach lining will become thinner

Answer 34

IM viatmain B12 injections for life Blood transfusion idf serious enough Long term monitoring

Answer 35

Aplastic anaemia is a serious condition affecting the blood, where the bone marrow and stem cells do not produce enough blood cells. It is also called bone marrow failure and can happen suddenly (acute) or develop over a period of time (chronic). AKA "Bone Marrow Failure" (Anaemia + Thrombocytopenia + Leukocytopenia)

Answer 36

In the red bone marrow which is in flat bones such as sternum, ribs & pelvis.

Answer 37

Causes are not well understood. -It is an autoimmune disorder where the haematopoietic stem cells become targeted for destruction -There are some genetic links -A person can get acquired aplastic anaemia due to exposure to toxins such as radiation, some pesticides or medications (e.g. Chloramphenicol) Currently there is no way to prevent it.

Answer 38

It is most commonly an early childhood disease with another peak in the 20-25 age range

Answer 39

Most will have same as iron deficient anaemia (pallor, breathlessness, glossitis, fatigue, tachycardia etc.) plus: Most common sign is unexplained & frequent bruising Bleeding with extended time for clot formation Repeated infections

Answer 40

Bone marrow transplant & blood transfusions (often effective) Immunosuppressive therapy (usually first ine treatment) Androgens such as danazol or oxymetholone

Answer 41

To try to slow the immune system destruction of the haematopoietic stem cells. Might also be anti-rejection medications that are given to transplant patients.

Answer 42

To stimulate erythropoietin production

Answer 43

A group of inherited health conditions that affect the red blood cells. With SCD, the red blood cells have a tendency to go out of shape and become sickle-shaped (like a crescent moon) - instead of their normal disc shape.

Answer 44

To get SCD, you need to have two altered haemoglobin genes, one from each parent. If you only have one of these genes, you will have sickle cell trait, which is very much milder and does not normally cause illness. If you have the trait, the majority of red cells in the blood are normal round shaped cells. Some sickle shaped cells may be present under certain conditions.

Answer 45

It is abnormal haemoglobin production caused by an inherited variant of haemoglobin - haemoglobin-S (the normal variant is haemoglobin-A), resulting in a substitution of valine in place of what should be glutamic acid. This causes the HbS to become rigid under low O2 conditions. When deoxygenated the haemoglobin crystalises and alters the shape of the erythrocyte – from a disc to a sickle. HbS is really sensitive to low oxygen conditions.

Answer 46

Low O2 conditions (including ones caused internally by vasoconstriction) such as: High altitude Infection Blood loss Dehydration (think weather changes) Temperature (weather hot/cold) Exercise Stress Swimming in cold water

Answer 47

Sickle cell trait is found in 1 in 4 West Africans and 1 in 10 Afro-Caribbeans and is also found in people who originate from the Mediterranean, Asia and the Middle East. It is less common in white Europeans

Answer 48

Because in utero the baby relies on foetal haemoglobin, this continues through birth and until the baby is about 6 months old when it begins to rely on its own haemoglobin.

Answer 49

Sickle cell disease, an inherited condition

Answer 50

20 days, normal is 120

Answer 51

One of the functions of the spleen is to filter out damaged and dead RBCs. Sickle celled RBCs die quicker and are recogised as damaged even before they die. The spleen can deal witha small number of these circulating cells but these abnormal red cells can become stuck in the spleen, if this is in a few areas then the spleen will continue to work but become enlarged as it’s trying to cope with the back up of red cells waiting to get through – this is **splenomegaly** Sometimes these abnormally shaped, crystalised cells become trapped in the spleen and block bigger areas of the filtering mechanism. The spleen gets much larger very quickly and many RBCs are held there, not enough for the rest of the body - this is called **splenic sequestration** and it is a medical emergency.

Answer 52

Usually an acute presentation over 1-2 days in a child with sickle cell disease: Pallor Fatigue SoB Pain in L side abdomen radiating to the R

Answer 53

There is no way to prevent it Treatment would be blood transfusion and possible splenectomy

Answer 54

Obstruction in small blood vessels – thrombus formation, infarction, necrosis Acute chest syndrome Dactylitis “Hand & Foot Syndrome” Sickle cell nephropathy Sickle cell maculopathy (eyes) Cerebral ischemia LV dilation Pulmonary hypertension Leg ulcers Gallstones (Excess bilirubin from excess haemolysis)

Answer 55

Acute chest syndrome is a severe pneumonia-like lung-related complication of sickle cell disease. It occurs due to vaso-occlusion within the pulmonary vasculature of patients with sickle cell disease. This results in deoxygenation of hemoglobin and sickling of erythrocytes, which can cause further vaso-occlusion, ischemia, and endothelial injury. Acute chest syndrome can progress quickly and is the most common cause of death in patients with sickle cell disease.

Answer 56

Pyrexia SoB Low SpO2 Cough Chest pain Possible wheezing/pneumonia

Answer 57

Anaemia signs – pallor, fatigue, tachycardia etc. Hyperbilirubinemia (from excess breakdown of haeme)– jaundice, gallstones Splenomegaly Vascular occlusion (crisis) – pain, tissue damage Occlusion in the lungs – acute chest syndrome (pain, fever) Heart failure Frequent infections – reduced healing Signs do not normally present until after 12 months

Answer 58

Analgesia around the clock (usually morphine) IV fluids Bed rest Warm compresses (esp. on extremities) Remove restrictions (watches/jewellery/tight clothing/BP) Blood transfusion (if haemolytic crisis i.e. they don’t have enough circulating red cells) Folic acid supplement to help with RBC production (not iron)

Answer 59

The amount of iron in a sickle patient is not usually an issue, if iron was given it could cause iron toxicity where iron collects in the organs and the level becomes toxic.

Answer 60

A new medication for sickle cell disease - it discourages sickled cells from adhering to the endothelial vessel surface, only some patiets are eligible.