Haem Flashcards

Question

What is the pathophysiology of pernicious anaemia?

Answer 1

The parietal cells of the stomach produce a protein called intrinsic factor, intrinsic factor is eseential for the absorption of vit B12 in the ileum Pernicious anaemia is an autoimmune condition where antibodies form against the parietal cells or intrinsic fsctor A lack of intrinsic factor prevents the absorption of vit B12 and the patient becomes vitamin B12 deficient

Answer 2

Peripheral neuropathy with numbness or paraesthesia (pins and needles) Loss of vibratuon sense or proprioception Visual changes Mood or cognitive changes

Answer 3

Intrinsic factor antibody= first line investigation | Gastric parietal cell antibody can also be tested but is less helpful

Answer 4

Dietary deficiency can be treated with oral replacement- cyanocobalamin unless the deficiency is severe

Answer 5

In pernicious anaemia oral replacement is inadequate because the problem is with absorption rather than intake In pernicius anaemia they can be treated with 1mg of IM hydroxycobalamin 3 times weekly for 2 weeks, then every 3 months

Answer 6

It is important to treat the B12 deficiency first before correcting the folate deficiency. Treating patients with folic acid when they have B12 deficiency can lead to subacute combined degeneration of the cord

Answer 7

Inherited Haemolytic Anaemias ``` Hereditary Spherocytosis Hereditary Elliptocytosis Thalassaemia Sickle Cell Anaemia G6PD Deficiency ``` Acquired Haemolytic Anaemias ``` Autoimmune haemolytic anaemia Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn) Paroxysmal nocturnal haemoglobinuria Microangiopathic haemolytic anaemia Prosthetic valve related haemolysis ```

Answer 8

Anaemia due to the reduction in circulating RBC Splenomegaly as the spleen becomes filled with destroyed red blood cells Jaundice as bilirubin is released during the destruction of red blood cells

Answer 9

FBC- shows a normocytic anaemia Blood film- shows schistocytes (fragments of red blood cells) Directs coombs test (positive in autoimmune haemolytic anaemia)

Answer 10

Hereditary soherocytosis It is an autosomal dominant condition Hereditary soherocytosis causes sphere shaped red blood cells which are fragile and easily break down when passing through the spleen.

Answer 11

Jaundice, gallstones, splenomegaly and notably aplastic crisis in the presence of the parvovirus It is diagnosed by family history and clinical features with spherocytosis on the blood film

Answer 12

Folate supplementation and splenectomy

Answer 13

Heinz bodies

Answer 14

Autoimmune haemolytic anaemia occurs when antibodies are created against the patient’s red blood cells. These antibodies lead to destruction of the red blood cells. There are two types based on the temperature at which the auto-antibodies function to cause the destruction of red blood cells. Warm type and cold type autoimmune haemolytic anaemia

Answer 15

Blood transfusions Prednisolone Rituximab ( a monoclonal antibody against B cells) Splenectomy

Answer 16

Haemolytic anaemia is a key complication of prosthetic heart valves. It occurs in both bioprosthetic and metallic valve replacement. It is caused by turbulence around the valve and collision of red blood cells with the implanted valve. Basically the valve churns up the cells and they break down. Management involves: Monitoring Oral iron Blood transfusion if severe Revision surgery may be required in severe cases

Answer 17

A genetic condition which causes sickle (crescent) shaped red blood cells It makes red blood cells fragile and more easily destroyed leading to an haemolytic anaemia Patients with sickle cell anaemia are prone to various types of sickle cell crises

Answer 18

Autosomal recessive condition where there is an abnormal gene for beta globin on chromosome 11 One copy of the gene results in sickle cell trait, two abnormal copies = sickle cell disease

Answer 19

``` Anaemia Stroke Increased risk of infection Avascular necrosis in large joints ie: the hip Pulomonary hypertension CKD Sickle cell crises Acute chest syndrome ```

Answer 20

Avoid dehydration and other triggers of crises Ensure vaccines are up to dare Abx prophylaxis with Pen V Hydroxycarbamide can be used to stimulate production of fetal haemoglobin Blood transfusion for severe anaemia Bone marrow transplant can be curative

Answer 21

This is an umbrella term for a spectrum of acute crises related to sickle cell They range from mild to life threatening Have a low threshold for admission to hospital Treat any infection Keep warm Keep well hydrated (IV fluids may be required) Simple analgesia such as paracetamol and ibuprofen Penile aspiration in priapism

Answer 22

It is caused by the sickle shapdd blood cells clogging the capillaries leading to distal ischaemia It is associated with dehydration and raised haematocrit (the ratio of the volume of red blood cells to the total volume of blood) Symptoms= pain, fever, priapism

Answer 23

This is caused by red blood cells blocking blood flow within the spleen, this causes an acutely enlarged and painful spleen The pooling of the blood in the spleen can lead to a severe anaemia and circulatory collapse (hypovolaemic shock) Splenic sequestration crisis is an emergency and management is supportive with blood transfusions and fluid resucitaton to treat anaemia and shock. Splenectomy prevents sequestration crisis and is often used in cases of recurrent crises. Recurrent crises can lead to splenic infarction and therefore susceptibility to infections.

Answer 24

Aplastic crisis describes a situation where there is a temporary loss of the creation of new blood cells. This is most commonly triggered by infection with parvovirus B19. It leads to significant anaemia. Management is supportive with blood transfusions if necessary. It usually resolves spontaneously within a week.

Answer 25

A diagnosis of acute chest syndrome requires: Fever or respiratory symptoms with New infiltrates seen on a chest xray This can be due to infection (e.g. pneumonia or bronchiolitis) or non-infective causes (e.g. pulmonary vaso-occlusion or fat emboli). Is a medical emergency with a high mortality and requires prompt supportive management and treatment of the underlying cause: Antibiotics or antivirals for infections Blood transfusions for anaemia Incentive spirometry using a machine that encourages effective and deep breathing Artificial ventilation with NIV or intubation may be required

Answer 26

The name for cancer of a particular line of stem cells in the bone marrow This causes unregulated prodyction of certain types of blood cells They can be classified depending on how rapidly they progress (slow= chronic, fast= acute) and the cell line that is affected (myeloid or lymphoid… Acute myeloid leukaemia Acute lymphoblastic leukaemia Chronic myeloid leukaemia Chronic lymphocytic leukaemia

Answer 27

``` Fatigue Fever Failure to thrive (kods) Pallor due to anaemia Petechiae and abnormal bruising (thrombocytopenia) Abnormal bleeding Lymphadenpathy Hepatosplenomegaly ```

Answer 28

``` Leukaemia Meningococcal septicaemia Vasculitis Henoch schonlein purpura Idiopathic thrombocytopenic purpura (ITP) Non accidental injury ```

Answer 29

Full blood count NICE recommend a full blood count within 48 hours for patients with suspected leukaemia Children or young adults with ptechiae or hepatosplenomegaly should be referred immediately to the hospital

Answer 30

Blood film- abnormal cells and inclusions Lactate dehydrogenase- a blood test that is often raised in leukaemia but is not specific to leukaemia, it can be raised in other cancers and many non cancerous diseases Bone marrow biopsy is the main investigation for establishing a definitive diagnosis of leukadmi CXR may show infection or mediastinal lymphadenopathy Lymph node biopsy Lumbar puncture may be used if there is central nervous system involvement CT/MRI/ PET

Answer 31

This is where there is malignant change in one of the lymphocyte precursor cells It causes an acute proliferation of a single type of lymphocyte, usually B lymphocytes Excessive proliferation of these cells causes them to replace the other cell types being created in the bone marrow, leading to pancytopenia This is the most common cancer in children and peaks around 2-4 years!

Answer 32

Blast cells

Answer 33

Philadelphia chromosome

Answer 34

This is where there is a chronic proliferation of a single type of well differentiated lymphocyte, usually B lymphocytes This usually affects adults over 55 years of age

Answer 35

This is where CLL transforms into a high grade lymphoma

Answer 36

Smear or smudge cells

Answer 37

Chronic phase Accelerated phase Blast phase The chronic phase can last around 5 years, is often asymptomatic and patients are diagnosed incidentally with a raised WCC Accelerated phase occurs where the abnormal blast cells take up a high proportion of the cells in the bone marrow and blood, in the accelerated phase the patients become more symptomatic The blast phase follows the accelerated phase and involves an even higher proportion of blast cells and blood (>30%), this phase has severe symptoms and pancytopenia, it is often fatal The characteristic change that is characteristic of CML is the philadelphia chromosome

Answer 38

Radiotherapy Bone marrow transplant Surgery

Answer 39

``` Failure Stunted growth and development in children Infections due to immunodeficiency Neurotoxicity Infertility Secondary malignancy Cardiotoxicity Tumour lysis syndrome ```

Answer 40

This is caused by the release of uric acid from cellsthat are being destroyed by chemo The uric acid can form crystals in the interdtitial tissue and tubules of the kidneys and causes acute kidney injury Allopurinol and rasburicase are used to reduce the high uric acid levels Other chemicals released; - potassium - phosphate - calcium will be low as a result of the phosphate so ghis should also be monitored and treated appropriately

Answer 41

Hodgkins and non hodgkins

Answer 42

HIV EBC Autoimmune conditions- rheumatoid arthritis and sarcidosis Family history

Answer 43

Lymphadenopathy = the key presenting symptom Lymphadenopathy may become painful when drinking alchohol Pts may get B symptoms- fever, weight loss, night sweats Other symptoms; fatigue, itching, cough, SOB, abdominal pain, recurrent infections

Answer 44

Lymph node bipsy is the key diagnostic test = the reed sternberg is the key finding from lymphnofe biopsy in patients with hodgkins lymphoma LDH is a blood test which is often raised in hodgkins lymphoma, it is not specific and can be rasised in other cancers and many non cancerous diseases CT, MRI, PET scans can be used for diagnosing and staging lymphoma and other tumours Ann arbor staging system is used for both hodgkins and non hodgkins lymphoma

Answer 45

Chemotherapy (ceates a risk of leukaemia and infertility) | Radiotherapy creates a risk of cancer, damage to tissues and hypothyroidism

Answer 46

Burkitt lymphoma MALT lymphoma Diffuse large B cell lymphoma

Answer 47

``` HIV EBV H pylori (MALT lymphoma) Hep B or Hep C Exposure to pesticides Family history ```

Answer 48

A cancer of plasma cells (type of B lymohocyte that produce antibodies) because it is a cancer of plasma cells it results in a large quantity of a single type of antibody being produced

Answer 49

This is where the myeloma (cancer causing lots of monoclonal antibodies) affects multiple areas of the body

Answer 50

This is where there is an excess of a single type of antibody or antibody components without other features ofmyeloma or cancer It may progress to myeloma and patients are often followed up routinely to monitor for progression

Answer 51

Progression of MGUS with higher levels of antibodies/ antibody components, it is premalignant and is more likely to progress to myeloma than MGUS

Answer 52

Bence jones protein

Answer 53

This is a result of increased osteoclast activity and suppressed osteoblast activity Osteoclasts absorb bone and osteoblasts deposit bone This results in the metabolism of bone becoming imbalanced as more bone is being reabsorbed than constructed

Answer 54

Osteolytic lesions

Answer 55

High levels of immunoglobulins (antibodies) can block the flow through the tubules Hypercalcaemia Dehydration Bisphosphonates

Answer 56

Easy bruising Easy bleeding Reduced or loss of sight due to vascular disease in the eye Purple discolouration to the extremities (purplish palmar erythema) Heart failure

Answer 57

``` CRAB Calcium (elevated) Renal failure Anaemia (normocytic normochromic) Bone lesions/ pain ```

Answer 58

``` Older age Male Black African ethnicity Family history Obesity ```

Answer 59

``` FBC (low white blood cell count in myeloma) Calcium (raised in myeloma) ESR (raised in myeloma Plasma viscosity (raised in myeloma) ``` If any of these are positive or myeloma is still suspected then do an urgen serum protein electrophoresis and a urine bence jones protein test BLIP Bence jones protein Serum free light chain assay Serum immunoglobulins Serum protein electrophoresis Bone marrow biopsy is necessary to confirm the diagnosis of myeloma and get more information on the disease Imaging is required to assess for bone lesions, the order of preference to establish this is… Whole body MRI Whole body CT Skeletal survery (xray images of the fhll skeleton)

Answer 60

Punched out lesions Lytic lesions “Raindrop skull” caused by many punched out (lytic) lesions throughout the skull that give the appearance of raindrops splashing on a surface

Answer 61

Myeloma bone disease can be improved using bisphosphonates. These suppress osteoclast activity. Radiotherapy to bone lesions can improve bone pain. Orthopaedic surgery can stabilise bones (e.g. by inserting a prophylactic intramedullary rod) or treat fractures. Cement augmentation involves injecting cement into vertebral fractures or lesions and can improve spine stability and pain

Answer 62

Thrombocytopenia describes a low platelet count Low platelet count can be split into problems with production or destruction ``` Problems with production- Sepsis B12/folate deficiency Liver failure causing thrombopoietin production in the liver to be reduced Leukaemia Myelodysplastic syndrome ``` ``` Problems with destruction Meds- sodium valproate, methotrexate, isoretinoin, antihistamines, PPI Alcohol ITP TTP Heparin induced thrombocytopenia Haemolytic uraemic syndrome ```