Management of patients with Hematologic disorders

Sickle cell disease (SCD) is a condition that requires extensive care, including infection prevention through vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication.

New gene therapies were approved in 2023 to cure a small percentage of people with sickle cell disease through bone marrow transplant.

The disease affects approximately 4.4 million people, with an additional 43 million having the sickle cell trait. The majority of cases occur in Sub-Saharan Africa.

The disease was first described by James B. Herrick in 1910, and the genetic transmission was determined by E. A. Beet and J. V. Neel in 1949. The protective effect against malaria of sickle cell trait was discovered in 1954.

Sickle cell disease leads to various acute and chronic complications, with sickle cell crises being one of the most common and potentially life-threatening.

There are several types of sickle cell crises, including vaso-occlusive, aplastic, splenic sequestration, and haemolytic crises, each with unique characteristics and treatments.

The vaso-occlusive crisis is caused by sickle-shaped red blood cells obstructing capillaries and restricting blood flow, leading to pain, ischemia, and potential organ damage. This crisis can be managed with hydration, analgesics, and blood transfusion for milder cases, and intravenous opioids for more severe cases.

Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells, which can result in a precipitous fall in haemoglobin levels and hypovolemic shock if left untreated. Management is supportive and may include blood transfusion.

Acute chest syndrome is the second most common complication and accounts for about 25% of deaths in patients with SCD. It is characterized by chest pain, fever, pulmonary infiltrate, respiratory symptoms, and hypoxemia. Most cases involve vaso-occlusive crises, but about 80% of people have vaso-occlusive crises during acute chest syndrome.

Aplastic crises are instances of an acute worsening of a patient's baseline anaemia, caused by parvovirus B19 infection, which directly affects red blood cell production and results in a precipitous drop in haemoglobin levels. This crisis can be managed supportively, but some patients may require a blood transfusion.

Haemolytic crises are acute accelerated drops in haemoglobin level, caused by an increased breakdown of red blood cells, particularly common in those with coexistent G6PD deficiency. The imbalance in oxygen reactive species in the cells leads to more production of red blood cells that are not properly oxygenated or formed. Management is supportive, with the use of antioxidants and blood transfusion if necessary.

Sickle cell disease (SCD) is a condition that requires extensive care, including infection prevention through vaccination and antibiotics, high fluid intake, folic acid supplementation, and pain medication.

New gene therapies were approved in 2023 to cure a small percentage of people with sickle cell disease through bone marrow transplant.

The disease affects approximately 4.4 million people, with an additional 43 million having the sickle cell trait. The majority of cases occur in Sub-Saharan Africa.

The disease was first described by James B. Herrick in 1910, and the genetic transmission was determined by E. A. Beet and J. V. Neel in 1949. The protective effect against malaria of sickle cell trait was discovered in 1954.

Sickle cell disease leads to various acute and chronic complications, with sickle cell crises being one of the most common and potentially life-threatening.

There are several types of sickle cell crises, including vaso-occlusive, aplastic, splenic sequestration, and haemolytic crises, each with unique characteristics and treatments.

The vaso-occlusive crisis is caused by sickle-shaped red blood cells obstructing capillaries and restricting blood flow, leading to pain, ischemia, and potential organ damage. This crisis can be managed with hydration, analgesics, and blood transfusion for milder cases, and intravenous opioids for more severe cases.

Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells, which can result in a precipitous fall in haemoglobin levels and hypovolemic shock if left untreated. Management is supportive and may include blood transfusion.

Acute chest syndrome is the second most common complication and accounts for about 25% of deaths in patients with SCD. It is characterized by chest pain, fever, pulmonary infiltrate, respiratory symptoms, and hypoxemia. Most cases involve vaso-occlusive crises, but about 80% of people have vaso-occlusive crises during acute chest syndrome.

Aplastic crises are instances of an acute worsening of a patient's baseline anaemia, caused by parvovirus B19 infection, which directly affects red blood cell production and results in a precipitous drop in haemoglobin levels. This crisis can be managed supportively, but some patients may require a blood transfusion.

Haemolytic crises are acute accelerated drops in haemoglobin level, caused by an increased breakdown of red blood cells, particularly common in those with coexistent G6PD deficiency. The imbalance in oxygen reactive species in the cells leads to more production of red blood cells that are not properly oxygenated or formed. Management is supportive, with the use of antioxidants and blood transfusion if necessary.