Temporal arteritis is a well-known historical condition. Immediate treatment with high doses of steroids, such as prednisolone at 1 mg/kg/day, is crucial to minimize the risk of vision loss.

Temporal arteritis is a type of large vessel vasculitis that often occurs in patients over the age of 60 and is commonly associated with polymyalgia rheumatica. This condition is characterized by changes in the affected artery that skip certain sections while damaging others. Symptoms of temporal arteritis include headache, jaw claudication, and visual disturbances, with anterior ischemic optic neuropathy being the most common ocular complication. A tender, palpable temporal artery is also often present, and around 50% of patients may experience symptoms of PMR, such as muscle aches and morning stiffness.

To diagnose temporal arteritis, doctors will typically look for elevated inflammatory markers, such as an ESR greater than 50 mm/hr or elevated CRP levels. A temporal artery biopsy may also be performed to confirm the diagnosis, with skip lesions often being present. Treatment for temporal arteritis involves urgent high-dose glucocorticoids, which should be given as soon as the diagnosis is suspected and before the temporal artery biopsy. If there is no visual loss, high-dose prednisolone is typically used, while IV methylprednisolone is usually given if there is evolving visual loss. Patients with visual symptoms should be seen by an ophthalmologist on the same day, as visual damage is often irreversible. Other treatments may include bone protection with bisphosphonates and low-dose aspirin, although the evidence supporting the latter is weak.

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.

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.

Clinical Manifestations of Henoch-Schönlein Purpura

Henoch-Schönlein Purpura (HSP) is a type of vasculitis that affects small blood vessels in the body. Its clinical manifestations include subcutaneous oedema of the feet, hands, scalp, and ears, as well as scrotal oedema. Pitting oedema up to the knees may indicate cardiac failure or nephrotic syndrome. Gastrointestinal bleeding may lead to bloody stools, while haematuria and proteinuria may occur. Abdominal pain, intussusception, and arthritis are also common features. Petechiae, purpura, and papules are commonly present in the thighs and buttocks. Notably, thrombocytopenia, haemolysis, and splenomegaly are absent, and clotting is normal. Understanding the clinical manifestations of HSP is crucial for its timely diagnosis and management.

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.

Understanding Multiple Myeloma: A Clonal Disorder of Plasma Cells

Multiple myeloma is a rare but serious type of cancer that affects plasma cells in the bone marrow. It is characterized by the presence of monoclonal immunoglobulin, which can be detected through serum electrophoresis. Patients with multiple myeloma often experience painful bone lesions, recurrent infections, weakness, renal failure, and hypercalcemia.

Plasma cells produce heavy and light chains separately, and an excess of free light chains can enter the bloodstream and be filtered by the kidneys. In cases of multiple myeloma, the amount of monoclonal free light chains can become too high for the kidneys to reabsorb, leading to the presence of Bence Jones protein in the urine.

While monoclonal gammopathy of undetermined significance can also cause a spike-like peak in the γ-globulin zone, the levels of antibody are lower and there are no other features of myeloma. Some cases of myeloma may secrete only light chains or no detectable immunoglobulin at all.

The amount of M protein present can be used to assess the amount of myeloma at diagnosis and track the disease throughout treatment. Understanding the characteristics and detection of multiple myeloma is crucial for effective management and care.

Haemoglobin Electrophoresis: Diagnosis of Thalassaemia Minor

Thalassaemia is an autosomal-recessive inherited disorder that affects globin chain production, resulting in decreased or absent α or β chains of the normal adult haemoglobin molecule. Homozygous states result in thalassaemia major, which can be fatal. Inheritance of only one affected gene results in a carrier state, called thalassaemia minor or a thalassaemia trait.

A patient’s ethnic origin and blood picture can help diagnose thalassaemia minor, which is characterized by mild or absent anaemia, hypochromic and microcytic red cells with low MCV and MCH, and normal serum ferritin. Haemoglobin electrophoresis is a useful diagnostic tool that reveals haemoglobin types and their amounts. In people with β-thalassemia, there is reduced or absent production of β-globin chains, resulting in reduced or absent HbA, elevated levels of HbA2, and increased HbF (fetal haemoglobin).

Other diagnostic tests, such as a barium enema, iron therapy, labelled red-cell scan, and upper and/or lower gastrointestinal endoscopy, are not indicated for thalassaemia minor unless there are coexisting conditions. Haemoglobin electrophoresis remains the gold standard for diagnosing thalassaemia minor.

Rhesus Negative Pregnancy and Anti-D Prophylaxis

A rhesus negative pregnant woman should receive anti-D prophylaxis after any sensitising event during pregnancy to prevent the production of antibodies that could cause rhesus haemolytic disease in the baby. Sensitisation can occur if RhD-positive blood cells enter the bloodstream of a RhD-negative woman, which can happen during an antepartum bleed, an invasive procedure, an abdominal injury, or at delivery. Rhesus disease can be avoided if sensitisation is prevented.

Rhesus disease affects the baby by causing haemolysis of red blood cells and anaemia. It occurs when a pregnant mother is RhD negative, the baby is RhD positive, and sensitisation has previously occurred. An injection of anti-D immunoglobulin can prevent sensitisation in a RhD-negative woman by neutralising any fetal RhD-positive antigens that have entered her blood.

A rhesus negative woman with a rhesus negative partner cannot have a rhesus positive baby and is not at risk. A rhesus negative baby will not introduce rhesus positive antigens into the mother’s blood, so anti-D is not required in this case.

Routine antenatal anti-D prophylaxis (RAADP) is administered during the third trimester of pregnancy to prevent sensitisation. This can be a single dose at 28-30 weeks or a two-dose treatment at 28 and 34 weeks. If RAADP is not given, the woman will be offered an injection of anti-D immunoglobulin within 72 hours of giving birth if the baby is RhD positive. This significantly decreases the risk of her next baby having rhesus disease.

Glimepiride, a medication used to treat type 2 diabetes and belonging to the sulphonylurea class, can trigger haemolysis in patients with G6PD deficiency. This can be indicated by mild anaemia, elevated bilirubin levels, and the presence of bite cells and blister cells on a blood film, suggesting haemolytic anaemia. Simvastatin, on the other hand, can induce hepatitis and cause jaundice, but this is unlikely if alanine transaminase and alkaline phosphatase levels are normal. Metformin, ramipril, and lansoprazole are not associated with haemolytic anaemia.

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.

Drugs to Avoid in G6PD Deficiency

G6PD deficiency is a genetic disorder that affects the red blood cells and can lead to haemolytic anaemia. Certain drugs and substances can trigger a haemolytic crisis in individuals with G6PD deficiency. Here are some drugs that should be avoided:

1. Quinolones: Ciprofloxacin, moxifloxacin, nalidixic acid, norfloxacin, and ofloxacin.

2. Sulphonamides: Co-trimoxazole (sulphamethoxazole and trimethoprim).

3. Nitrofurantoin.

4. Antimalarials: Chloroquine, primaquine, and quinine.

5. Chloramphenicol.

6. Isoniazid.

7. Dapsone.

8. Sulphonylureas such as glibenclamide.

9. Vitamin K analogues.

10. Aspirin (a dose up to 1 g daily is usually harmless) and paracetamol.

11. Probenecid.

12. Ascorbic acid.

13. Isosorbide dinitrate.

It is also important to avoid fava beans, severe infections, diabetic ketoacidosis, and acute kidney injury as they can also trigger a haemolytic crisis. However, co-amoxiclav is not known to precipitate haemolysis. G6PD deficiency was first discovered during an investigation of haemolytic anaemia occurring in some individuals treated for malaria with primaquine. It is important to consult with a healthcare provider before taking any medication if you have G6PD deficiency.