How Sickle Cell Disease Damages the Spleen, Liver, Kidneys and Eyes

Sickle cell disease can affect essentially every organ system, through the same underlying mechanism of vaso-occlusion and chronic haemolysis, but each organ has its own distinct emergency presentation and management.

Splenic Sequestration

Splenic sequestration is an acute emergency in which a vaso-occlusive crisis within the spleen causes red cells to pool there, producing a hypovolaemic shock state. It usually occurs in young children and presents with left upper quadrant pain and fullness, a sudden fall in haemoglobin of more than 2 g, reticulocytosis, and splenomegaly. Treatment is transfusion, but this has to be done carefully: transfusing too quickly can cause a sudden rise in haemoglobin that increases blood viscosity and worsens the situation, so both the rate and volume of transfusion need close attention. Over time, functional asplenia develops in most patients by two to three years of age as recurrent splenic infarcts destroy the spleen's tissue, which raises the risk of severe infection with encapsulated bacteria, especially Streptococcus pneumoniae, along with Salmonella, Haemophilus influenzae, Meningococcus and Mycoplasma. This is why the conjugate pneumococcal vaccine, routine vaccination, and penicillin prophylaxis started as early as possible are all part of standard care.

Liver Involvement

The liver can be affected directly by the sickling process itself, through acute sickle hepatic crisis, hepatic sequestration similar to the spleen, and sickle intrahepatic cholestasis, where sickling occurs within the hepatic sinusoids. It can also be affected indirectly, through the multiple blood transfusions many patients need, which carry a risk of hepatitis B and C, as well as through gallstones, biliary complications, hepatic abscess and cholelithiasis. Management depends on which of these is present: gallstones are treated with cholecystectomy, acute sickle hepatic crisis needs supportive care with red cell exchange considered, and worsening hepatic sequestration or intrahepatic cholestasis may need blood transfusion and supportive care, again with red cell exchange considered depending on the clinical picture.

Kidney Involvement

Renal papillary necrosis, caused by recurrent medullary infarctions, occurs in around 15 to 30% of people with sickle cell disease and can present as either painful or painless haematuria, sometimes complicated by a urinary tract infection or obstruction; treatment is hydration, alkalinising the urine, treating any infection, and relieving obstruction where present. Acute renal failure can also occur during an acute pain episode or chest crisis, or as part of multiorgan failure, and needs close monitoring of creatinine and fluid balance, avoidance of nephrotoxic drugs, and nephrology involvement.

Eye Involvement

Central retinal artery occlusion is an ocular emergency, more common in children and young adults, presenting with sudden vision loss; it is treated with either a simple or an exchange transfusion. Orbital infarction can occur during a vaso-occlusive crisis and presents as protrusion of the eye, eye pain, and sudden lid or orbital swelling, treated with IV fluids, pain control and steroids. Because sickle retinopathy can also develop chronically, annual retinal screening is recommended, with laser photocoagulation or vitrectomy used to treat it when it occurs.

This article is based on a Jivo Masterclass session conducted by Dr. Divya Doval, Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi. The article has been summarised with the assistance of an AI tool from the original masterclass recording. Watch the full Masterclass recording