Henoch-Schönlein purpura is a common vasculitis that affects children and young adults, typically between the ages of 4 and 15. The condition is characterized by palpable purpura on dependent areas of the body, such as the lower limbs, and areas exposed to skin pressure. Other symptoms may include subcutaneous edema, joint pain, and gastrointestinal issues. Skin biopsy can reveal a leukocytoclastic vasculitis, and elevated levels of immunoglobulin A (IgA) are present in about half of patients. In some cases, Henoch-Schönlein purpura may follow a respiratory tract infection. Glomerulonephritis may also be present, which can be identified by microscopic hematuria, proteinuria, and red-cell casts. While renal involvement occurs in up to 40% of older children, it is serious in only about 10% of patients. Treatment may involve prednisolone for severe cases, with the addition of azathioprine if glomerulonephritis is present and associated with deteriorating renal function.

It is important to note that sickle cell patients require the pneumococcal polysaccharide vaccine every 5 years, as per current NICE CKS guidance. Therefore, advising them that they do not need this vaccination would be incorrect. This is because sickle cell patients, along with those with asplenia, splenic dysfunction, and chronic renal disease, are likely to experience a rapid decline in antibody concentration. In contrast, patients with conditions such as chronic respiratory disease or diabetes mellitus may only require vaccination once in their lifetime.

Managing Sickle-Cell Anaemia

Sickle-cell anaemia is a genetic blood disorder that causes red blood cells to become misshapen and break down, leading to a range of complications. When a crisis occurs, management involves providing analgesia, rehydration, oxygen, and potentially antibiotics if there is evidence of infection. Blood transfusions may also be necessary, and in some cases, an exchange transfusion may be required if there are neurological complications.

In the longer term, prophylactic management of sickle-cell anaemia involves the use of hydroxyurea, which increases the levels of HbF to prevent painful episodes. Additionally, it is recommended that sickle-cell patients receive the pneumococcal polysaccharide vaccine every five years to reduce the risk of infection. By implementing these management strategies, individuals with sickle-cell anaemia can better manage their condition and improve their quality of life.

The probability of an underlying hereditary thrombophilia is high in the 54-year-old woman who has an unprovoked deep vein thrombosis and a first-degree relative with the same condition.

Deep vein thrombosis (DVT) is a serious condition that requires prompt diagnosis and management. The National Institute for Health and Care Excellence (NICE) updated their guidelines in 2020, recommending the use of direct oral anticoagulants (DOACs) as first-line treatment for most people with VTE, including as interim anticoagulants before a definite diagnosis is made. They also recommend the use of DOACs in patients with active cancer, as opposed to low-molecular weight heparin as was previously recommended. Routine cancer screening is no longer recommended following a VTE diagnosis.

If a patient is suspected of having a DVT, a two-level DVT Wells score should be performed to assess the likelihood of the condition. If a DVT is ‘likely’ (2 points or more), a proximal leg vein ultrasound scan should be carried out within 4 hours. If the result is positive, then a diagnosis of DVT is made and anticoagulant treatment should start. If the result is negative, a D-dimer test should be arranged. If a proximal leg vein ultrasound scan cannot be carried out within 4 hours, a D-dimer test should be performed and interim therapeutic anticoagulation administered whilst waiting for the proximal leg vein ultrasound scan (which should be performed within 24 hours).

The cornerstone of VTE management is anticoagulant therapy. The big change in the 2020 guidelines was the increased use of DOACs. Apixaban or rivaroxaban (both DOACs) should be offered first-line following the diagnosis of a DVT. Instead of using low-molecular weight heparin (LMWH) until the diagnosis is confirmed, NICE now advocate using a DOAC once a diagnosis is suspected, with this continued if the diagnosis is confirmed. If neither apixaban or rivaroxaban are suitable, then either LMWH followed by dabigatran or edoxaban OR LMWH followed by a vitamin K antagonist (VKA, i.e. warfarin) can be used.

All patients should have anticoagulation for at least 3 months. Continuing anticoagulation after this period is partly determined by whether the VTE was provoked or unprovoked. If the VTE was provoked, the treatment is typically stopped after the initial 3 months (3 to 6 months for people with active cancer). If the VTE was

Lower gastrointestinal tract investigation should be conducted on any patient in this age group who has an unexplained microcytic anaemia to rule out the possibility of colorectal cancer.

Colorectal cancer referral guidelines were updated by NICE in 2015. Patients who are 40 years or older with unexplained weight loss and abdominal pain, those who are 50 years or older with unexplained rectal bleeding, and those who are 60 years or older with iron deficiency anaemia or a change in bowel habit should be referred urgently to colorectal services for investigation. Additionally, patients with positive results for occult blood in their faeces should also be referred urgently.

An urgent referral should be considered if there is a rectal or abdominal mass, an unexplained anal mass or anal ulceration, or if patients under 50 years old have rectal bleeding and any of the following unexplained symptoms or findings: abdominal pain, change in bowel habit, weight loss, or iron deficiency anaemia.

The NHS offers a national screening programme for colorectal cancer every two years to all men and women aged 60 to 74 years in England and 50 to 74 years in Scotland. Patients aged over 74 years may request screening. Eligible patients are sent Faecal Immunochemical Test (FIT) tests through the post. FIT is a type of faecal occult blood test that uses antibodies to detect and quantify the amount of human blood in a single stool sample. Patients with abnormal results are offered a colonoscopy.

The FIT test is also recommended for patients with new symptoms who do not meet the 2-week criteria listed above. For example, patients who are 50 years or older with unexplained abdominal pain or weight loss, those under 60 years old with changes in their bowel habit or iron deficiency anaemia, and those who are 60 years or older who have anaemia even in the absence of iron deficiency.

Management of Iron Deficiency Anaemia

Iron deficiency anaemia is a common condition that can present with symptoms such as fatigue, weakness, and shortness of breath. In a 28-year-old woman with normal menses and no signs of gastrointestinal bleeding, a trial of iron supplementation for three months is appropriate to establish whether ferritin levels increase and haemoglobin normalises. Although a negative anti-TTG test is possible in patients with selective IgA deficiency, the absence of bowel symptoms makes underlying coeliac disease unlikely.

If there are no other symptoms and signs, urgent referral to colorectal under the two-week wait is necessary for unexplained iron deficiency anaemia in a male with a Hb of <120 g/L or a non-menstruating female with a Hb of <100 g/L. Upper and lower GI endoscopy would only be considered if there is a failure of ferritin level and anaemia to respond to iron supplementation. Proper management of iron deficiency anaemia is crucial to prevent complications and improve quality of life.

Individuals with G6PD deficiency may experience haemolytic anaemia as a result of taking malaria prophylaxis, such as primaquine.

Understanding G6PD Deficiency

G6PD deficiency is a common red blood cell enzyme defect that is inherited in an X-linked recessive fashion and is more prevalent in people from the Mediterranean and Africa. The deficiency can be triggered by many drugs, infections, and broad (fava) beans, leading to a crisis. G6PD is the first step in the pentose phosphate pathway, which converts glucose-6-phosphate to 6-phosphogluconolactone and results in the production of nicotinamide adenine dinucleotide phosphate (NADPH). NADPH is essential for converting oxidized glutathione back to its reduced form, which protects red blood cells from oxidative damage by oxidants such as superoxide anion (O2-) and hydrogen peroxide. Reduced G6PD activity leads to decreased reduced glutathione and increased red cell susceptibility to oxidative stress, resulting in neonatal jaundice, intravascular hemolysis, gallstones, splenomegaly, and the presence of Heinz bodies on blood films. Diagnosis is made by using a G6PD enzyme assay, and some drugs are known to cause hemolysis, while others are considered safe.

Compared to hereditary spherocytosis, G6PD deficiency is more common in males of African and Mediterranean descent and is characterized by neonatal jaundice, infection/drug-induced hemolysis, and gallstones. On the other hand, hereditary spherocytosis affects both males and females of Northern European descent and is associated with chronic symptoms, spherocytes on blood films, and the presence of erythrocyte membrane protein band 4.2 (EMA) binding.

Managing High INR Levels in Patients on Warfarin: Choosing the Right Course of Action

When a patient on warfarin presents with a high international normalised ratio (INR), prompt management is crucial to prevent haemorrhage. The appropriate course of action depends on the severity of the INR elevation and whether the patient is bleeding.

If the patient has no active bleeding and their INR is between 5-8, warfarin should be withheld for 24-48 hours and restarted at a reduced dose. The INR should be rechecked 2-3 days later, and the cause investigated.

If the patient is bleeding, warfarin should be stopped and intravenous vitamin K administered. It can be restarted once the INR is below 5.

For an INR greater than 8 in a patient with no bleeding, the correct management is to stop warfarin, give vitamin K orally, and repeat the INR in 24 hours.

Dose reduction alone is not sufficient for an INR greater than 5, and warfarin should also be withheld for 1-2 days, with rechecking sooner (3-4 days).

While a confirmatory laboratory sample may be reasonable, it should not delay action being taken. Point-of-care testing is comparable in accuracy to laboratory samples, and management should reflect this.

In summary, managing high INR levels in patients on warfarin requires careful consideration of the severity of the INR elevation and whether the patient is bleeding. Prompt action and appropriate monitoring can prevent haemorrhage and ensure optimal patient outcomes.

Von Willebrand’s disease is a commonly inherited clotting disorder that is often characterized by prolonged bleeding after minor injuries, particularly mucosal membrane injuries. This autosomal dominant condition is caused by a reduction or structural abnormality of von Willebrand’s factor, which plays a crucial role in promoting normal platelet function and stabilizing coagulation factor VIII. Although screening tests may yield normal results, a specialist investigation and assay of von Willebrand Factor may be necessary for diagnosis. While most patients with mild disease respond well to desmopressin (DDAVP), clotting factor concentrates may be required for a minority. It is important to note that prolonged bleeding following dental extraction may be a sign of von Willebrand’s disease.

Understanding Von Willebrand’s Disease

Von Willebrand’s disease is a genetic bleeding disorder that is inherited in an autosomal dominant or recessive manner. It is the most common inherited bleeding disorder, and it behaves like a platelet disorder. Patients with this condition often experience epistaxis and menorrhagia, while haemoarthroses and muscle haematomas are rare.

The disease is caused by a deficiency or abnormality in von Willebrand factor, a large glycoprotein that promotes platelet adhesion to damaged endothelium and serves as a carrier molecule for factor VIII. There are three types of von Willebrand’s disease: type 1, which involves a partial reduction in vWF and accounts for 80% of cases; type 2, which is characterized by an abnormal form of vWF; and type 3, which involves a total lack of vWF and is inherited in an autosomal recessive manner.

To diagnose von Willebrand’s disease, doctors may perform a bleeding time test, measure APTT, and check factor VIII levels. Defective platelet aggregation with ristocetin is also a common finding. Treatment options include tranexamic acid for mild bleeding, desmopressin to raise levels of vWF, and factor VIII concentrate. The type of von Willebrand’s disease a patient has doesn’t necessarily correlate with their symptoms, but common themes include excessive mucocutaneous bleeding, bruising without trauma, and menorrhagia in females.

Understanding the CHA2DS2-VASc Score for Stroke Risk Assessment

The CHA2DS2-VASc score is a tool used to assess the risk of stroke and guide the use of thromboprophylaxis. It takes into account various risk factors such as congestive heart failure, hypertension, age, diabetes, prior stroke or thromboembolism, vascular disease, and sex. Each factor is assigned a certain number of points, and the total score is used to determine whether oral anticoagulation is necessary.

If the score is 1 or more, oral anticoagulation is recommended, while a score of 0 indicates that no anticoagulation is needed. However, if the score is 1 but the only point scored is for female gender, then it is treated as a score of 0. It is important to note that aspirin, clopidogrel, and dipyridamole are not recommended alone or in combination.

In summary, the CHA2DS2-VASc score is a useful tool for assessing stroke risk and guiding thromboprophylaxis use. By taking into account various risk factors, healthcare professionals can make informed decisions about the appropriate treatment for their patients.

Blood Donation Eligibility Criteria

To ensure the safety of blood transfusions, there are certain eligibility criteria that potential donors must meet. Here are some corrections to common misconceptions:

Travel to an endemic malaria area: Donors must wait six months after traveling to an endemic malaria area before donating blood. If they fell ill abroad or were resident for more than six months in Sub-Saharan Africa, they must wait even longer.

Age: Donors must be between 17 and 66 years old (up to 70 if they have given blood before). If they are over 70 years old, they need to have given blood in the last two years to continue donating.

Body piercing and tattoo: Donors are deferred if they have had body piercing or a tattoo in the previous four months.

Underweight: Donors should weigh at least 50 kg. For other contraindications, please refer to the provided link.